General description of organic (including symptomatic) psychical disorders.
Somatogenic psychical disorders.
Patients who have psychical violations at somatic and endocrine diseases.
Psychical violations at the damage of vessels of cerebrum.
Exogenic diseases.
Psychical violations at infectious violations.
Psychical violations at craniocerebral traumas.
Psychical violations at intoxications.
Psychical violations as a result of ecologically unfavorable influences.
Psychical violations in declining and senile years.
Every time a patient comes into your office, your emergency room or your hospital, there is a very real possibility that what seems to be a psychological problem is caused by some physical illness. The depressed patient may have an under active thyroid gland. The patient with panic attacks may have a pheochromocytoma, a tumor that secretes epinephrine. And the patient, whose personality change and increased irritability is thought to be caused by his marital problems, may actually have a brain tumor causing the personality changes and exacerbating longstanding marital issues.
How common is this problem? Very…and not very. Most of your clients will not have a medical disease masquerading as an emotional problem. In fact, one of the problems is that most really serious medical illnesses are rare enough that we all get sloppy and stop looking for them. Most of the time our medical workups are unnecessary-but most of the time is not the same as all of the time. It is not necessary to live in abject terror about missing all of the patients with unsuspected medical illnesses that come to you with symptoms of depression or anxiety. On the other hand, medical causes of psychiatric symptoms should always be considered. As a mental health professional, you need to know enough about these medical illnesses to make some basic assessment about whether a further medical assessment is necessary and how to focus that assessment so as to make it as productive as possible. Ex.-Johnson (1968) performed detailed physical exams on 250 patients admitted to an inpatient psychiatric unit. 12% of these patients were admitted to the psychiatric unit for problems that seemed to be caused by physical illness 80% of these had been missed by physician before admission 6.6% were initially missed even after the admission workup 60% had abnormal physical findings Ex.-Hall (1978) performed a detailed assessment on 658 consecutive psychiatric outpatients – 9.1% had a significant medical illness-Ex.-Slater (1965) studied 85 patients (32 men and 53 women) diagnosed as having “hysteria” – follow up 7-11 years. More than a third proved to have organic disease
Ex.-Sox et. al. (1989) did a thorough medical evaluation on 509 patients in community mental health programs in California.- 200 (or 39%) had at least one active, important, physical disease, Staff at the mental health program was aware of only 47% of these. Research program discovered previously undiagnosed, important diseases in 63 of these patients. 14% had medical illness that was causing or exacerbating their mental illness Ex.-Koran performed thorough medical assessments on 529 patients drawn from eight community mental health centers in California. 17% were found to have an organic condition that either caused or exacerbated the emotional symptoms for which the person was being treated. Ex.-Bartsch et. al. performed a comprehensive evaluation on 175 clients from two Colorado CMHCs. A previously undiagnosed physical health problem was found in 20% of the clients. 16% had conditions that could cause or exacerbate their mental disorder. 19 clients had a metabolic abnormality (elevated calcium, etc.).
7 clients had a neurological disorder (memory loss, post concussion syndrome, etc.) 7 clients had an adverse medication effect. 4 clients had some other disorder, including cancer
Conservative estimates suggest that 10% of persons initially seen in outpatient settings for psychological symptoms have an organic disease causing the symptoms. This figure is higher in the elderly, in persons with certain diagnosis such as hysteria, and much higher in inpatient settings.
What can one do about it?
Even internists and neurologists, working in academic centers and aware of the possibility of organic illness, miss medical illnesses with disturbing frequency. There is no set of tests that can definitively rule everything out. Some illnesses are hard to diagnose, especially at the beginning. Others are so rare that they are not thought of so that the specific tests that would allow the diagnosis are not considered. Still other times the illnesses present atypically. The patient’s symptoms seem different than those described in the medical textbooks, so that a medical illness is missed.
The most common problem, however, is that we do not think about the possibility of medical illness and, therefore, we do not specifically look for medical illness. IF YOU DO NOT LOOK FOR IT, YOU WILL NOT FIND IT. The purpose of this paper is not to get you to the point of being able to diagnose every possible disease. Rather, it is to give you a starting point-to know when to be particularly suspicious (or worried), to know something about the most common illnesses, and to learn enough to communicate with the consulting physician so that you can make sure that your patient gets the best possible evaluation.
There are at least three problems with trying to present this kind of brief review for non-medical mental health professionals.
The first is that there are a huge number of different possible illnesses to worry about. I am not about to try to list all possible illnesses or to give complete descriptions but, rather, to get you to think about some of the common illnesses that you are most likely to see in your practice.
The second problem is that it is almost impossible to talk about medical illnesses without lapsing into medical jargon. This is half a paper about medical illnesses, and half a paper on learning a new language that will hopefully help you when you need to communicate to other physicians.
The third problem is both more subtle and more serious. Non-medical mental health professionals organize the world according to psychological symptoms. The question is, what medical illnesses can cause depression, anxiety, etc.? The problem is that the depression caused by a brain tumor may be identical to the depression caused by marital discord or by an endogenous depression. What is likely to be different is the patient’s history and the associated signs and symptoms apart from the depression. Unfortunately, listing illnesses according to which ones can cause depression or which ones can cause anxiety does not produce a coherent organization. Many illnesses can cause many different psychological symptoms. More importantly, such a listing would not help to understand what other questions to ask to help separate physical from psychological illnesses.
Physicians organize the world much differently. The easiest way to remember all of the separate facts and to see patterns is to organize illnesses according to physiological systems. Throughout this paper I will keep talking about endocrine systems, neurological systems and cardiopulmonary systems. For someone who has been through medical school, this becomes the obvious way to organize things, but it is not always so obvious for the rest of the world. The problem with categorizing according to psychiatric symptoms will become obvious as you go through this paper. A huge number of illnesses can present as depression, and the vast majority of these illnesses can also present as anxiety or delirium. It does not do much good to think about the list of illnesses that can present as depression unless you begin to think about some of the other associated symptoms that those illnesses also have-and the best way to organize these associated symptoms is to understand what organ systems the illness effects.
Having said all of that, I will try to organize illnesses by their psychological effects, and, at the same time, try to introduce the way that physicians would organize their thinking about those illnesses.
Section I
General Approach
A. Always consider the possibility of organic disease- If you do not look for it you will not find it.1. Be suspicious of “medical clearance”.
B. Unfortunately, physicians tend to dismiss psychiatric patients for several reasons. There is a tendency to assume that all psych patients are just “nuts” without “real illness”. Physicians are often uncomfortable around patients who are obviously depressed or who are acting bizarrely, or who they are afraid might act bizarrely. At times these patients behave in ways that make evaluation more difficult, either by being unwilling to give a full history, unable to give an accurate description of symptoms, or too frightened to allow a full physical examination.
2. People with schizophrenia get sick too.
The fact that someone is actively psychotic does not mean that they do not also have a serious medical illness. One should always be concerned that a medical illness might, in fact, be the cause of the psychosis. But even in patients who clearly have schizophrenia or some other diagnosable mental illness and who have had an excellent medical workup in the past, it is important to consider whether their current complaints or recent change in behavior could be related to a medical illness. In fact, psychotic patients are more difficult to evaluate, and if they do happen to have a serious medical illness, it is more likely to get missed. Studies have demonstrated that disliked patients are more likely to have an undiagnosed organic brain syndrome than more likable patients, and it is just those disliked patients that will often get the most cursory and incomplete physical evaluation. My guess is that patients who are most different from their physicians are also more likely to have a medical illness missed, and this is especially true of psychiatric patients.
3. Be alert for presentations, which make medical illness more likely-but do not stop considering medical illness just because these are not present. o a patient over 40 with no previous psychiatric history no history of similar symptomso coexistence of chronic diseaseo a history of head injuryo a change in headache patterno a patient who gets worse when given antipsychotic or anxiolytic medications
4. Look for symptoms, which make medical illness more likely.o a change in headache pattern visual disturbances, either double vision or partial visual loss
o speech deficits, either dysarthrias (problems with the mechanical production of speech sounds) or aphasias (difficulty with word comprehension or word usage). abnormal autonomic signs (blood pressure, pulse, temperature) disorientation and/or memory impairment fluctuating or impaired level of consciousness abnormal body movements frequent urination, increased thirst (possible symptoms of diabetes) significant weight change, gain or loss
5. Do not assume that a certain symptom “must” be of psychological origin. For example, it used to be thought that male impotence was almost always a psychological problem. A recent study of 105 impotent men reported that 75% had impotency based either on a medical illness such as diabetes mellitus, or were using drugs that were likely to cause impotence. Of 34 men with hormonal problems who accepted medical treatment, 33 had return of sexual function. Fourteen of these men had previously undergone psychotherapy for this same problem.
B. Be Holistic
A psychiatric assessment should include the whole person, including the medical history and physiology of that person. This is needed to rule out a medical illness, but also so that you can understand the person’s current feelings and functioning within the context of what has happened to the person in the past and what is happening now.Much of the information that you need to suspect a medical illness is readily available as part of a psychiatric assessment. It is important to know how to organize this information so that it is useful, and to fill in gaps in your information so that important areas are not missed. (Note that a comprehensive psychiatric evaluation would include additional areas such as personal developmental history and current social support system, in addition to the assessment areas discussed below.)
1. Symptoms Start with a clear description of all of the patient’s symptoms. How did they begin? How long has he had them? What has the progression of symptoms been like? Include a careful review of other “extraneous” symptoms the patient may have-starting at the top with questions about headache and dizziness and ending at the bottom with questions about leg sores and trouble walking. This “review of systems” is an extremely important part of a medical assessment.
2. History Include history of similar problems in the past
o History of past medical problems including all medical hospitalizations and surgeries Family history, both medical and psychiatric
3. Current medical status
o Ask about all current medical illnesses
o Ask about all current medications (Include specific questions about vitamins, birth control, over the counter meds, etc.) Ask about past medical problems, past surgeries, past medical hospitalizations Ask about any head injury, coma, periods of unconsciousness, seizures. Obtaiame of person’s physician–date of last contact–for what purpose
4. Current habits Ask about drug use, starting with questions about tobacco, caffeine and alcohol and proceeding on to questions about other drugs Ask about exercise and activity patterns, sleep patterns
5. Observation.
The assessment starts when you first meet the patient, not when you first sit down to begin talking in your office. General appearance: How does the person look? How are they dressed? Do they appear ill? Then go to more specific observations. Skin: Is it very dry or abnormally colored? Extremely pale skin or lips may suggest anemia. A yellow skin may indicate jaundice and liver disease. Dry skin and hair may be a sign of hypothyroidism. Eyes: Are they focused? Are the pupils equal? Are they aligned with each other? Differences in pupil size may indicate brain masses such as tumors. Wildly dilated pupils may indicate a variety of drugs including hallucinogens, stimulants, and anticholinergics. Constricted pupils may indicate opiates. Bulging eyes can be a sign of hyperthyroidism. Observe body movement to rule out weakness, clumsiness, ataxia, facial asymmetry, asymmetry of movements, choreiform movements (“worm-like” or other involuntary movements, usually occurring less than 2 times/second), tremors. Observe for other neurological abnormalities such as motor stereotypy (repetitive stereotyped movements). Gait disturbance is a very common finding in a wide range of medical conditions.ubin (1983) studied 1140 patients cleared medically on a psychiatric service. 38 subsequently found to have a medical illness 14 of the 38 had either gait disturbance, weight loss, hypertension, abnormal vital signs or significant medical history
6. Mental status examination appearance degree of cooperation presence of perceptual distortions (hallucinations and illusions) mood (both appropriateness and quality) speech (both quality and content) motor activity general cognitive abilities
– attention memory judgment fund of knowledge Also consider evidence of specific neurological deficits: aphasias (difficulties with speech) can be broken down into word finding difficulties (nominal aphasias) difficulty understanding speech (receptive aphasias) or difficulty producing speech (expressive aphasia) agnosias (recognition of complex shapes) apraxias (execution of proper manipulation of objects) perseveration (inability to switch tasks or mental sets) ach of these can occur with varying degrees of severity.
7. Physical exam. full physical examination is obviously not possible if you are not a physician, and even psychiatrists rarely perform a physical examination themselves. Some parts of a physical examination are easy, even for non-physicians. Blood pressure, preferably lying and standing (or you can ask a patient about any recent blood pressure checks, or ask them to get their blood pressure taken at one of the blood pressure machines that seem to be in every bank and drugstore) Pulse for evaluation of rate and arrhythmias (irregularities of heart rhythm) Check eyes to see if they move equally and fully in all directions, equal and reactive pupils, and nystagmus (small “jerky” movements of eyes when client looks up or to the side) Assessment of the condition of the patient’s skin, looking for such things as dryness, dehydration, nutritional status, rashes, edema, petechiae
A useful screen for picking up physical disease in psychiatric patients includes:
o Laboratory tests: TSH (thyroid test), CBC (complete blood count), SGOT (liver function test), Fasting glucose [or random glucose if fasting not possible] (screen for diabetes), serum albumin, serum calcium, vitamin B12, and urinalysis History of epilepsy, emphysema, asthma, diabetes, thyroid disease, history of blood or pus in the urine, or history of high blood pressure HIV positive or history of high risk behavior for HIV Symptoms of chest pain while at rest, headaches associated with vomiting or loss of control of urine or stool Physical findings of high blood pressure (adapted from Sox et al 1989)
C. Develop a “differential diagnosis” that systematically considers possible medical illnesses. Consider all of the medical illness that could fit the set of symptoms. What further information would help distinguish between these various possibilities? As a way of organizing your information about the patient
focusing your attention and targeting what further information is needed.The goal is not to come up with a specific diagnosis. The goal is to organize the data that you collect about the patient so that you can decide what to do next, how worried you need to be, and when and how and what to say to your consulting physician if you decide further medical assessment is necessary.
The basic mental health assessment must, of course, be supplemented with appropriate outside consultation, which will include a physical examination and appropriate laboratory tests, but this should be focused by the differential diagnosis. BOTH YOU AND THE DOCTOR ARE MORE LIKELY TO FIND IT IF YOU ARE LOOKING FOR SOMETHING SPECIFIC THAN IF YOU ARE GROPING RANDOMLY. For Example: with “hysterical” symptoms, consider MS with mental status changes occurring over days to weeks, together with alcoholism or chronic headache, consider subdural hematoma (slow bleed inside the skull under the dura membrane that covers the brain) with depression along with weight gain, ask about cold intolerance and dry skin and consider hypothyroidismLaboratory and other diagnostic tests should be used to pursue specific parts of the differential diagnosis list. Diagnostic tests are much more likely to give useful results when you and the doctor are clear what question you have in mind and what specific test is needed to answer that specific question. For Example:
EEG detects abnormal brain function
CAT scan detects abnormal anatomy
If you are asking for a “drug screen” to find out if the client has recently used an illicit drug, find out if your laboratory can measure the drug or drugs that you expect this person might be using, and whether blood or urine tests are better depending on the particular drug and time since ingestion. Most labs can test for the presence of cocaine, but LSD is used in much smaller amounts and may not be detectable even if recently used. This kind of question can be answered by a call to the chemistry lab of the local hospital, but such a call requires that you step out of your typical “non-medical” role and interact with a strange and often forbidding medical system.
C. Work with and actively involve the consulting physician.
D. At different times with different doctors and different clinical situations this will mean different things. It always means making the consultation request as clear as possible. What kind of answer do you want back from the doctor? What are you most worried about? What information do you already have about the client? You might think that your job is just to get the client to see the doctor, and the rest of the job is up to the doctor. This is true-and not true. The doctor will typically spend less than 15 minutes with the patient to collect a history, do the physical, order the tests and write a note in the chart.
E. If the client is less than articulate, important information is likely to get lost. This is a particular problem with older clients, those who are hard of hearing or who have other communication problems, or those who are less organized or less clear in their thinking. It is also a problem when the symptoms you want evaluated are vague, or your concerns leading to the referral do not relate to a particular “medical” symptom. Your job must include organizing the information that you have collected and transmitting it to the doctor in such a way as to do your client the most good.
F. Telling the client to see his local doctor, or phoning the local internist with a request to “Please do a physical exam on this client.” is much less likely to lead to a reasonable consultation result than a request, “This client has a depression that seems very atypical. Could you please see if there could be a medical illness involved?” Or even better yet, “This patient is complaining of depression with decreased energy level, but he is also complaining of increased weight, cold intolerance, decreased libido and extremely dry skin. He was treated for hyperthyroidism 15 years ago. Could you see if any thyroid problems or any other medical problems might be increasing his depression?”
G. Most of the time you will not be able to frame a consult request with as much detail as this last example-but in all cases the more the better. Often, the referral to the physician is based on a pattern suggesting a higher probability of medical illness, rather than any particular symptom suggesting a particular illness. For example, any client who initially develops psychiatric symptoms over the age of 40 should have a medical workup. If this is the reason you are referring the client, then the physiciaeeds to have that information.
H. Finally, there are differences of communication styles between mental health professionals and physicians. The social worker or psychologist is likely to want to give the physician a complete description of the patient and the problem in a phone discussion that may go on for many minutes. The physician is likely to be in the middle of office hours, with a clinic full of patients waiting to be seen. A brief, succinct and very focused description and problem statement with a focused consultation request is likely to be better received by a physician than the more complete communication often expected between psychotherapists.
I. Common assumptions that lead to missed diagnosis: mistaking symptoms for their causes listening without fully considering all possibilities equating psychosis with schizophrenia relying on a single information source
Section II
Psychosis-Patients that Appear Out of Touch with Reality
A. Consider Organic Disease
B. If you do not look for it you will not find it. Be suspicious of “medical clearance”.
1. Other symptoms that suggest organic disease include: a patient over 40 with no previous psychiatric history hallucinations that are visual and vivid in color, that change rapidly olfactory (smell) hallucinations illusions: misinterpretations of stimuli large recent weight changes
2. A brief, minimal neurological exam can be easily and rapidly done, even on very agitated patients (even by someone who is not a physician).
o Observe gait and body movement to rule out weakness, paralysis, ataxia and other gait disturbances and choreoathetoid movements
Check eyes:
– Make sure pupils are equal and reactive to light.
– Check to see if eyes move fully in all directions.
– Check for vertical and horizontal nystagmus: refers to rapid movements of jerking of the eyes, and can be either up and down (vertical) or back and forth (horizontal). It is most easily seen if the client is asked to look up or over to the side as far as possible. Nystagmus is frequently present with drug intoxications, and vertical nystagmus is never a normal finding in functional psychosis.
Observe face for asymmetries.
Observe speech for slurring, aphasias, word finding difficulties, and perseveration.
The above observations are possible on a completely uncooperative patient. Summers et. al. have outlined a very rapid physical exam for screening purposes (see bibliography).
3. Consider medical emergencies that can present as psychiatric illness
a. Hypoglycemia (low blood sugar): symptoms can be variable and include delirium or coma. Can include palpitations, sweating, anxiety, tremor, vomiting. If in doubt, give candy or orange juice sweetened with sugar. In an emergency room, give 50 cc. of 50% dextrose for both treatment and diagnosis.
b. Diabetic Ketosis or non-ketotic hyperosmolarity (blood sugar so high that it upsets body chemistry): delirium with history of diabetes, increased breathing, sweet smell of acetone on breath (can be mistaken for smell of alcohol), dehydration, decreased blood pressure.
c. Wernickes-Korsakoff’s syndrome: acute thiamine (vitamin B6) deficiency so severe that it can cause rapid brain damage. Usually found in alcoholics. Symptoms include nystagmus (rapid small jerking movements of eyes), cerebellar ataxia (person moves as if drunk), evidence of peripheral neuropathy, ocular palsies (inability to move both eyes together in all directions) If in any doubt, give thiamine l00 mg. IM. This is not diagnostic but will prevent any further brain damage.
d. DT’s (delirium tremens): drug withdrawal from alcohol or other sedative hypnotics. Frequently missed and can be medically very serious. Symptoms include elevated autonomic signs, agitation, visual and tactile hallucinations and history of alcohol abuse. Onset is usually three to four days after reduction or discontinuation of alcohol.
e. Hypoxia (low blood oxygen): from pneumonia, heart attack, COPD (chronic obstructive pulmonary disease), arrhythmias (abnormal heart rhythm), etc.
f. Meningitis (infection of the covering of the brain): be alert for stiff neck and fever.
g. Subarachnoid hemorrhage (rapid arterial bleeding into the brain): stiff neck, fluctuating consciousness and headache. If there is a fluctuating consciousness along with stiff neck and headache, a spinal tap for diagnosis needs to be done immediately.
h. Subdural hematoma (bleeding from veins under the outside covering of the brain, which compresses the brain over hours to weeks or even longer): symptoms are variable but frequently (not invariably) there is a history of head trauma.
i. Anticholinergic (atropine) poisoning: from overdose of tricyclics or over-the-counter drugs, or from organophosphate insecticides. Classic symptoms include:
o Flushing “red as a beet”
o Mouth dry “dry as a bone”
o Dilated pupils “blind as a bat”
o Delirious “mad as a hatter”
These patients will also have increased pulse and sometimes elevated blood pressure. Most fatalities are from cardiac arrhythmias, although seizures are not uncommon.
Differentiate psychosis from delirium
Psychosis refers to an impairment in reality testing because of hallucinations, delusions or grossly disorganized thinking. Psychosis can be caused by organic diseases where we know the cause or by a variety of mental illnesses ranging from a brief reactive psychosis to schizophrenia.
Delirium refers to an acute organic brain syndrome causing a global cognitive impairment, with disorientation, memory impairment, and disturbance of consciousness. Illnesses causing deliriums are often life threatening, and a delirium should be considered to be a medical emergency.
Symptoms of delirium include:
disorientation or memory impairment
fluctuating or impaired level of consciousness, decreased awareness of environment
labile affect
impaired judgment or impaired insight
abnormal autonomic signs (changes in blood pressure, pulse, temperature, abnormal sweating, flushing, etc)
DSM IV Diagnostic criteria for Delirium
A. Disturbance of consciousness (i.e., reduced clarity of awareness of the environment) with reduced ability to focus, sustain, or shift attention.
B. A change in cognition (such as memory deficit, disorientation, language disturbance) or the development of a perceptual disturbance that is not better accounted for by a preexisting, established, or evolving dementia.
C. The disturbance develops over a short period of time (usually hours to days) and tends to fluctuate during the course of the day.
D. There is evidence from the history, physical examination, or laboratory findings that the disturbance is caused by the direct physiological consequences of a general medical condition.
Medical Illnesses that Can Present as Psychosis
1. Progressive neurological diseases
a. Multiple sclerosis: no typical signs or symptoms. It may begin very suddenly and affect any part of the neurological system. Early in its course, diagnosis may be extremely difficult.
b. Huntington’s chorea: hereditary illness that includes movement disorder but can present with psychosis initially.
c. Alzheimer’s disease and Pick’s disease: progressive diseases that cause dementia, but can initially present in a wide variety of ways. Alzheimer causes diffuse dementia, while Pick’s primarily affects the frontal lobes of the brain.
2. Central nervous system infections
a. Encephalitis (viral infection of the brain-usually Herpes Simplex): usually presents with fever and seizures, but various mental symptoms including catatonia or psychosis may present before any clear cut neurological symptoms. Usually has a fluctuating mental status.
b. Neurosyphilis (syphilis of the central nervous system).
c. HIV infections: HIV encepalopathy commonly includes apathy, decreased spontaneity and depression and may present before any other signs of AIDs are present. AIDS can also first present as delirium with paranoia and other prominent psychotic features.
3. Space occupying lesions within the skull
a. Brain tumors
b. Bleeding within the skull
c. Brain abscess
4. Metabolic disorders
a. Accumulation of toxins from severe liver or kidney disease.
b. Disturbances in electrolytes, either too low a serum level of sodium or too high a serum level of calcium.
c. Acute intermittent porphyria (disease of porphyrin metabolism): very rare, but may present as classical psychosis. Often has abdominal pain or other gastrointestinal symptoms such as vomiting.
d. Wilson’s disease: abnormality of copper metabolism that causes damage to brain and liver if untreated.
e. Systemic lupus erythematosis (autoimmune disease): usually a slowly progressive illness with joint and muscle pain, but it can present very suddenly. The nervous system is commonly involved and can present with depression, dyscontrol syndromes (unexpected impulsive or aggressive behavior), or psychosis.
5. Endocrine disorders
a. Myxedema (underactive thyroid gland-hypothyroidism)
b. Cushing’s syndrome (too much cortisol caused by overactive adrenal gland or overactive pituitary gland)
c. Hypoglycemia, either from insulin secreting tumor or administration of insulin
6. Deficiency states
a. Thiamine deficiency: Wernicke-Korsakoff amnestic syndrome
b. Pellegra (nicotinic acid deficiency) and other B complex deficiencies
c. Zinc deficiency
7. Temporal lobe epilepsy (or partial complex seizure disorder)
8. Drugs–prescription (L-DOPA, Amphetamine, cocaine, crack, methamphetamine, stimulants, hallucinogens)
Section III
Anxiety
A. Think About the Phenomenology of Anxiety
1. Psychological manifestations: Inner feelings of terror, tension, apprehension and dread, derealization, depersonalizations, fear of impending insanity
2. Intellectual disturbances: Decreased concentration, disorganized thinking, sensory flooding
3. Somatic manifestations: Autonomic or visceral symptoms, including palpitations, chest pain, tachycardia, fatigue, weakness, perspiration, flushing, numbness, tingling of extremities, vertigo, shortness of breath, headache, blurred vision, tinnitus, diarrhea, tremor, fainting
B. Differential Diagnosis of Anxiety
1. Primary anxiety disorders
Panic disorder with or without agoraphobia
Social phobia and other simple phobias
Obsessive-compulsive disorder
Post-traumatic stress disorder
Generalized anxiety disorder
Adjustment disorder with anxious mood
Depression may be a secondary feature
2. Other mental illness that can present as anxiety
Psychosis
Agitated Depression
Manic-depressive disorder (depressed phase)
3. Hyperventilation syndrome
C. Medical illness presenting with anxiety
Strongly suspect medical cause for anxiety in patients younger than 18 or older than 35 who suddenly develop anxiety which disrupts their normal activity and who have an otherwise negative psychiatric history (Hall 1980).
1. Anxiety secondary to organic brain syndromes
2. Apt to have a labile mood
3. Confusion which may be confused with psychosis
4. Mental status exam should demonstrate cognitive deficits, especially memory deficits
5. –delirium
6. –dementia
2. Other neurological illnesses (25% of medical causes of anxiety symptoms)
a. Cerebral vascular insufficiency: transient ischemi attacks lasting from 10-15 seconds up to an hour (brief blocks in the arteries to the brain causing temporary loss of brain blood supply)
b. Anxiety states and personality change following head injury
c. Infections of the central nervous system
d Meningitis: fever, stiff neck, and delirium
e Neurosyphilis: may present as almost anything
f. Degenerative disorders
g Alzheimer’s dementia
h Multiple sclerosis: may be marked early on by vague and changing medical complaints
i Huntington’s chorea: may present early as anxiety or other functional disorder before the movement disorder is evident-always has a positive family history
j. Toxic Disorders
k Lead Intoxication: loss of appetite, constipation and colicky abdominal pain followed by irritability and restlessness
l Mercury intoxication: from contaminated fish
m Manganese intoxication: from industrial exposure
n Organophosphate insecticides (similar to nerve gas): from chemical or insecticide exposure
o. Partial complex seizures
3. Endocrine disorders (25% of medical causes of anxiety symptoms)
a. Hyperthyroidism (increased thyroid hormone) commonly presents as anxiety, but may present as depression and is one of the most common endocrine abnormalities. Most common in 20- to 40-year-old women. The anxiety of hyperthyroidism may present with manic-like euphoria or agitation, along with weight loss, heat intolerance, rapid pulse, fine intention tremor and often exophthalmoses (bulging of the eyes caused by abnormal deposition of fat behind the eyeball).
b. Adrenal hyperfunction or Cushing’s syndrome: has a variety of causes, including tumors of the pituitary or adrenal glands or from steroids given to treat other illnesses. There is often a change in fat distribution with dorsal (back) hump, round face and thin arms and legs, hirsute (abnormal hairiness), acne, decreased menstruation in women and impotency in men.
c. Hypoglycemia (decreased blood glucose): usually associated with a history of diabetes and insulin or other hypoglycemic medications. Rarely from an insulin secreting tumor. Hypoglycemia as a response to dietary carbohydrate challenge is probably over diagnosed, and associated symptoms may not always be due to changes in blood glucose.
d. Hypoparathyroidism (decreased parathyroid hormone): almost always associated with a history of thyroid surgery. It often presents with overwhelming anxiety, either with or without personality change.
e. Menopausal and premenstrual syndromes.
4. Cardiopulmonary disorders: Often presents with shortness of breath, rapid breathing, complaints of chest pain, chest pain that are worse with exertion.
a. Angina
b. Pulmonary embolus
c. Arrhythmias (irregularities of heart beat)
d. Chronic obstructive pulmonary disease (COPD)
e. Mitral valve prolapse (generally harmless)
5. Pheochromocytoma (epinephrine secreting tumors)
Medications as a cause of anxiety
A. TAKE A CAREFUL AND DETAILED HISTORY.
B. Ask about all drugs that a patient is taking, licit and illicit, prescribed and over the counter
C. ask about all illnesses that a patient has had
D. asthmatics take combinations of sympathomimetics and xanthines (aminophylline, theophylline)
E. patients with allergies may take ephedrine
F. patients with diabetes may be hypoglycemic from their insulin
G. thyroid preparations may be prescribed for thyroid illness, following thyroid surgery (from years ago), or even for weight loss
1. Non-psychotropic medications
H. a. Sympathomimetics (often found ion-prescription cold and allergy medications): epinephrine, norephinephrine, isoproteronol, levodopa, dopamine hydrochloride, dobutamine, terbutaline sulfate, ephedrine, pseudo-ephedrine
I. b. Xanthene derivatives (asthma medications, coffee, colas, over-the-counter pain remedies): aminophylline, theophylline, caffeine
J. c. Anti-inflammatory agents: indomethacin
K. d. Thyroid preparations
L. e. Insulin (via hypoglycemic reaction)
M. f. Corticosteroids
N. g. Others: nicotine, ginseng root, monosodium glutamate
O. h. Drug withdrawal: caffeine, nicotine
2. Psychotropic medications
P. a. Antidepressants (including MAO-inhibitors), drugs for treatment of attention deficit disorders (on rare occasions cause anxiety-type syndromes)
Q. b. Tranquilizing drugs: benzodiazepines (paradoxical response most common in children and in elderly), antipsychotics (akathisia may present as anxiety)
R. c. Anticholinergic medications can cause a delirium which, in early stages, may easily be confused with anxiety: scopolamine and sedating antihistamines (found in over-the-counter sleep preparations) antiparkinsonian agents, tricyclic antidepressants, antipsychotics
3. Drugs–licit and illicit
S. a. Caffeine-intoxication or withdrawal
b. Nicotine-withdrawal even more than acute intoxication
c. Stimulants-cocaine, amphetamines, etc.
d. Alcohol or alcohol withdrawal
E. Drug withdrawal is a common cause of anxiety type syndromes
A large number of drugs can cause withdrawal states with symptoms of anxiety or even agitation. All sedative hypnotics, tricyclic anti-depressants and anti-cholinergics can cause withdrawal.
Section IV
Depression
A. Differential Diagnosis: Psychiatric Illness
1. Primary Affective Disorders
a. Major depression, either single episode or recurrent bipolar disorder
b. Dysthymia
c. Adjustment disorder with depressed mood
d. Bereavement
2. Depression Secondary to other Functional Disorders
B. Medical Illnesses that can present as Depression
1. Post viral depressive syndromes: especially influenza, infectious mononucleosis, viral hepatitis, viral pneumonia, and viral encephalitis
2. Cancer
a. Cancer of the pancreas commonly presents as depression
b. Lung Cancer, especially oat cell carcinoma
c. Brain tumors, either primary tumors or metastastic, may present with depression
3. Cardiopulmonary disease with hypoxia (decreased oxygen in the blood): acute hypoxia often leads to symptoms resembling anxiety or panic. Chronic hypoxia may present with lassitude, apathy, psychomotor retardation and other symptoms confused with depression.
4. Sleep apnea: should be suspected in a patient with sleep disturbance and daytime somnolence
5. Endocrine Disease
a. Hypothyroidism (under active thyroid): causes a general slowing of all body functions. Patient complains of fatigue, weight gain, constipation, and, when asked, will describe cold intolerance, dry skin and hair, and hoarseness or deepening of the voice. Often very insidious but easily diagnosed and treated ONCE SUSPECTED.
b. Hyperthyroidism or thyrotoxicosis (overactive thyroid): usually associated with anxiety but may present as depression, especially in the elderly who may have few classical signs of thyroid disease.
c. Adrenal hypofunction (Addison’s Disease): often presents with weakness and fatigue, along with low blood pressure and hyponatremia (low serum sodium) and hyperkalemia(increased serum potassium).
d. Adrenal hyperfunction (Cushing’s Disease): either from steroid medication, pituitary, adrenal or other ACTH secreting tumors. Various affective disturbances, either depression or mania, are common. Syndrome is marked by truncal obesity, hypertension, puffy face, and hirsutism.
e. Hyperparathyroidism: usually from small tumors of the parathyroid glands. Early symptoms develop insidiously and can include lassitude, anorexia, weakness, constipation and depressed mood. The classic symptoms of bone pain and renal colic often develop only years later.
f. Post-partum, post menopausal, and premenstrual syndromes.
6. Collagen-Vascular Diseases
This is a strange set of different diseases where the person essentially becomes allergic to parts of their own body. It can affect all parts of the body and can, at times, cause death.
Systemic lupus erythematosus (SLE) is most often seen in women 13-40 years old. It often presents initially with nonspecific symptoms such as fatigue, malaise, anorexia and weight loss, all of which can lead to the diagnosis of functional depression.
7. Central Nervous System Disease
a. Multiple Sclerosis
b. Brain tumors and other intracranial masses (masses inside of the skull) such as subdural hematomas (bleeding under the dural sack that surrounds the brain): masses, especially in the frontal and temporal areas, can grow for years and cause psychiatric symptoms before any focal neurological abnormality is apparent.
c. Complex partial seizures: ictal-repetitive behaviors during the seizure, interictal-personality changes between seizures, increased lability of emotions, quick to anger, increased preoccupation with religion, hypergraphia (increased writing).
d. Strokes, especially effecting left side of brain (right side of body)
Medications that can cause Depression
Ex.-Katerndahl found that 43% of patients diagnosed as depressed in a family practice clinic were taking medications that can cause depression.
1. Interferon (for treatment of hepatitis C infections)
2. Antihypertensive medications (drugs used to control high blood pressure): reserpine and alpha-methyldopa are probably the worst, but propranolol has been implicated and all antihypertensives are suspect
3. Digitalis preparations, along with a variety of other cardiac medications
4. Cimetidine: used for gastric ulcer disease
5. Indomethacin and other non-steroidal anti-inflammatory medications
6. Disulfuram (Antabuse): usually described by patients as more a sense of fatigue than true depression
7. Antipsychotic medications: can cause an akinesia or inhibition of spontaneity that can both feel and look like a true depression. This is much less common with the newer “atypical” antipsychotic medications
8. Anxiolytics: all sedative hypnotics from the barbiturates to the benzodiazepines have been implicated both in causing depression and making it worse in susceptible individuals
9. Steroids, including prednisone and cortisone
D. Drugs of abuse that can cause depression
1. Alcohol: very commonly a cause of depression, as well as a reaction to depression
2. Stimulant withdrawal
Dementia is a term that describes a collection of symptoms that include decreased intellectual functioning that interferes with normal life functions and is usually used to describe people who have two or more major life functions impaired or lost such as memory, language, perception, judgment or reasoning; they may lose emotional and behavioral control, develop personality changes and have problem solving abilities reduced or lost.
· There are different classification schemes for dementias roughly based (and with overlap) on observed problems; some frequently used are cortical (memory, language, thinking, social) , subcortical (emotions, movement, memory), progressive (cognitive abilities worsen over time), primary (results from a specific disease such as Alzheimer’s disease and secondary (occurs because of disease or injury).
·
· Alzheimer’s disease (AD): is the most common cause of dementia in people over age 65 with cause possibly related to amyloid plaques and neurofibrillary tangles; almost all brain functions, including memory, movement, language, judgment, behavior, and abstract thinking, are eventually affected.
· Vascular dementia: is the second most common cause of dementia caused by brain damage from cerebrovascular or cardiovascular problems (strokes) or other problems that inhibit vascular function; symptoms similar to AD but personality and emotions effected only late in the disease.
· Lewy body dementia: is common and progressive where cells in the brain’s cortex die and other contain abnormal structures (Lewy bodies); symptoms overlap with Alzheimer’s disease but also include hallucinations, shuffling gait, and flexed posture with symptoms that may vary daily.
· Frontotemporal dementia: is dementia linked to degeneration of nerve cells in the frontal and temporal brain lobes and some evidence for a genetic factor (many have a family history of the disease); symptoms in patients (usually ages 40 – 65) have judgment and social behavior problems such as stealing, neglecting responsibilities, increased appetite, compulsive behavior and eventual motor skill problems and memory loss.
· HIV-associated dementia: is due to infection of the brain with HIV virus; symptoms include impaired memory, apathy, social withdrawal, and concentration problems.
· Huntington’s disease: is a heredity disorder caused by a faulty gene and children of a person with the disorder have a 50% chance of getting the disease; symptoms begin in 30-40 year old people with personality changes such as anxiety, depression and progress to show psychotic behavior severe dementia and chorea – involuntary jerky, arrhythmic movements of the body.
· Dementia pugilistica: is also termed Boxer’s syndrome, is due to traumatic injury (often repeatedly) to the brain; symptoms commonly are dementia and parkinsonism (tremors, gait abnormalities) and other changes depending where brain injury has happened.
· Corticobasal degeneration: is a progressive nerve cell loss in multiple areas of the brain; symptoms begin at about age 60 on one side of the body and include poor coordination and rigidity with associated visual-spatial problems that can progress to memory loss, hesitant speech and dysphagia (difficulty swallowing).
· Creutzfeldt-Jakob disease: is a rare disease that seems related to a gene mutation that causes rapid (death about one year after symptoms begin to develop) degenerative and fatal brain disease in people usually over 60 years old; personality changes and reduced coordination develop, rapidly followed by impaired judgment and vision and many patients develop a coma before they die.
· Other rare hereditary dementias: – Most of these diseases develop in people between 50 – 60 years old and most have variable symptoms of poor reflexes, dementia, hallucinations, paralysis and most develop coma before death; some of the names of these diseases are Gerstmann-Straussler-Scheinker disease, familial British dementia, familial Danish dementia and fatal familial insomnia.
· Secondary dementias: These dementias occur in patients with other disorders of movement such as Parkinson’s disease or multiple sclerosis and may because by one or more problems listed above; these dementias may share symptoms with any of the above mentioned dementias but researchers are unsure if this is due to disease overlap or other causes.
· Dementias in children: While infections, trauma and poisoning can lead to dementia in both children and adults, there are some dementias that are unique to children but may result in mental problems, seizures, reduction or loss of motor skills, blindness, neurodegeneration and death; many are inherited disorders such as Niemann-Pick disease, Batten disease, Lafora disease and mitochondrial abnormalities.
· Other conditions that may cause dementia: Reactions to medications, endocrine and metabolic problems, nutritional deficiencies, infections, subdural hematomas, poisoning, brain tumors, anoxia (lack of oxygen), heart and lung problems.
· What conditions are not dementia: Although these conditions may resemble some aspects of dementia, they have different causes, usually are treatable and have better outcomes; examples are depression, delirium, mild cognitive impairment and age-related cognitive decline.
· Dementia causes: All causes of dementia result from death and damage of nerve cells in the brain; genetics and possibly the formation of different types of inclusions in the brain cells are likely the major causes, although some researchers suggest that certain inclusions may be only side effects of an underlying disorder.
· Risk factors for dementia include advancing age, genetics (family history), smoking, alcohol use, atherosclerosis, high cholesterol, diabetes, high plasma homocysteine levels, mild cognitive impairment, Down syndrome
· Dementia is diagnosed by using many methods such as patient’s medical and family history, physical exam, neurological evaluations, cognitive and neuropsychological testing, CT’s, MRI‘s and other brain scans, mental status exams, electroencephalograms, blood tests, psychiatric evaluations, and even some pre-symptomatic tests are available for some patients that may have a genetic link to dementia.
· Most treatments for dementia will neither reverse or stop the disease; however, there are treatments and medications that may reduce the symptoms and slow the disease progression; they are tight glucose control by persons with diabetes, intellectual stimulating activities, lowering cholesterol and homocysteine levels, regular exercise, education, controlling inflammation of body tissues, using NSAID’s and possibly other medications.
Introduction to Dementia
A woman in her early 50s was admitted to a hospital because of increasingly odd behavior. Her family reported that she had been showing memory problems and strong feelings of jealousy. She also had become disoriented at home and was hiding objects. During a doctor’s examination, the woman was unable to remember her husband’s name, the year, or how long she had been at the hospital. She could read but did not seem to understand what she read, and she stressed the words in an unusual way. She sometimes became agitated and seemed to have hallucinations and irrational fears.
· This woman, known as Auguste D., was the first person reported to have the disease now known as Alzheimer’s disease * (AD) after Alois Alzheimer, the German doctor who first described it. After Auguste D. died in 1906, doctors examined her brain and found that it appeared shrunken and contained several unusual features, including strange clumps of protein called plaques and tangled fibers inside the nerve cells. Memory impairments and other symptoms of dementia, which means “deprived of mind,” had been described in older adults since ancient times. However, because Auguste D. began to show symptoms at a relatively early age, doctors did not think her disease could be related to what was then called “senile dementia. “The word senile is derived from a Latin term that means, roughly, “old age.”
· It is now clear that AD is a major cause of dementia in elderly people as well as in relatively young adults. Furthermore, we know that it is only one of many disorders that can lead to dementia. The U. S. Congress Office of Technology Assessment estimates that as many as 6.8 million people in the United States have dementia, and at least 1.8 million of those are severely affected. Studies in some communities have found that almost half of all people age 85 and older have some form of dementia. Although it is common in very elderly individuals, dementia is not a normal part of the aging process. Many people live into their 90s and even 100s without any symptoms of dementia.
· Besides senile dementia, other terms often used to describe dementia include senility and organic brain syndrome. Senility and senile dementia are outdated terms that reflect the formerly widespread belief that dementia was a normal part of aging. Organic brain syndrome is a general term that refers to physical disorders (not psychiatric in origin) that impair mental functions.
· Research in the last 30 years has led to a greatly improved understanding of what dementia is, who gets it, and how it develops and affects the brain. This work is beginning to pay off with better diagnostic techniques, improved treatments, and even potential ways of preventing these diseases.
What Is Dementia?
Dementia is not a specific disease. It is a descriptive term for a collection of symptoms that can be caused by a number of disorders that affect the brain. People with dementia have significantly impaired intellectual functioning that interferes with normal activities and relationships. They also lose their ability to solve problems and maintain emotional control, and they may experience personality changes and behavioral problems such as agitation, delusions, and hallucinations. While memory loss is a common symptom of dementia, memory loss by itself does not mean that a person has dementia. Doctors diagnose dementia only if two or more brain functions – such as memory, language skills, perception, or cognitive skills including reasoning and judgment – are significantly impaired without loss of consciousness.
There are many disorders that can cause dementia. Some, such as AD, lead to a progressive loss of mental functions. But other types of dementia can be halted or reversed with appropriate treatment.
With AD and many other types of dementia, disease processes cause many nerve cells to stop functioning, lose connections with other neurons, and die. In contrast, normal aging does not result in the loss of large numbers of neurons in the brain.
What Are the Different Kinds of Dementia?
Dementing disorders can be classified many different ways. These classification schemes attempt to group disorders that have particular features in common, such as whether they are progressive or what parts of the brain are affected. Some frequently used classifications include the following:
· Cortical dementia: dementia where the brain damage primarily affects the brain’s cortex, or outer layer. Cortical dementias tend to cause problems with memory, language, thinking, and social behavior.
· Subcortical dementia: dementia that affects parts of the brain below the cortex. Subcortical dementia tends to cause changes in emotions and movement in addition to problems with memory.
· Progressive dementia: dementia that gets worse over time, gradually interfering with more and more cognitive abilities.
· Primary dementia: dementia such as AD that does not result from any other disease.
· Secondary dementia: dementia that occurs as a result of a physical disease or injury.
Some types of dementia fit into more than one of these classifications. For example, AD is considered both a progressive and a cortical dementia.
Alzheimer’s disease
Alzheimer’s disease is the most common cause of dementia in people aged 65 and older. Experts believe that up to 4 million people in the United States are currently living with the disease: one in ten people over the age of 65 and nearly half of those over 85 have AD. At least 360,000 Americans are diagnosed with AD each year and about 50,000 are reported to die from it.
In most people, symptoms of AD appear after age 60. However, there are some early-onset forms of the disease, usually linked to a specific gene defect, which may appear as early as age 30. AD usually causes a gradual decline in cognitive abilities, usually during a span of 7 to 10 years. Nearly all brain functions, including memory, movement, language, judgment, behavior, and abstract thinking, are eventually affected.
AD is characterized by two abnormalities in the brain: amyloid plaques and neurofibrillary tangles. Amyloid plaques, which are found in the tissue between the nerve cells, are unusual clumps of a protein called beta amyloid along with degenerating bits of neurons and other cells.
Neurofibrillary tangles are bundles of twisted filaments found withieurons. These tangles are largely made up of a protein called tau. In healthy neurons, the tau protein helps the functioning of microtubules, which are part of the cell’s structural support and deliver substances throughout the nerve cell. However, in AD, tau is changed in a way that causes it to twist into pairs of helical filaments that collect into tangles. When this happens, the microtubules cannot function correctly and they disintegrate. This collapse of the neuron’s transport system may impair communication betweeerve cells and cause them to die.
Researchers do not know if amyloid plaques and neurofibrillary tangles are harmful or if they are merely side effects of the disease process that damages neurons and leads to the symptoms of AD. They do know that plaques and tangles usually increase in the brain as AD progresses.
In the early stages of AD, patients may experience memory impairment, lapses of judgment, and subtle changes in personality. As the disorder progresses, memory and language problems worsen and patients begin to have difficulty performing activities of daily living, such as balancing a checkbook or remembering to take medications. They also may have visuospatial problems, such as difficulty navigating an unfamiliar route. They may become disoriented about places and times, may suffer delusions (such as the idea that someone is stealing from them or that their spouse is being unfaithful), and may become short-tempered and hostile. During the late stages of the disease, patients begin to lose the ability to control motor functions. They may have difficulty swallowing and lose bowel and bladder control. They eventually lose the ability to recognize family members and to speak. As AD progresses, it begins to affect the person’s emotions and behavior. Most people with AD eventually develop symptoms such as aggression, agitation, depression, sleeplessness, or delusions.
On average, patients with AD live for 8 to 10 years after they are diagnosed. However, some people live as long as 20 years. Patients with AD often die of aspiration pneumonia because they lose the ability to swallow late in the course of the disease.
Vascular dementia
Vascular dementia is the second most common cause of dementia, after AD. It accounts for up to 20 percent of all dementias and is caused by brain damage from cerebrovascular or cardiovascular problems – usually strokes. It also may result from genetic diseases, endocarditis (infection of a heart valve), or amyloid angiopathy (a process in which amyloid protein builds up in the brain’s blood vessels, sometimes causing hemorrhagic or “bleeding” strokes). In many cases, it may coexist with AD. The incidence of vascular dementia increases with advancing age and is similar in men and women.
Symptoms of vascular dementia often begin suddenly, frequently after a stroke. Patients may have a history of high blood pressure, vascular disease, or previous strokes or heart attacks. Vascular dementia may or may not get worse with time, depending on whether the person has additional strokes. In some cases, symptoms may get better with time. When the disease does get worse, it often progresses in a stepwise manner, with sudden changes in ability. Vascular dementia with brain damage to the mid-brain regions, however, may cause a gradual, progressive cognitive impairment that may look much like AD. Unlike people with AD, people with vascular dementia often maintain their personality and normal levels of emotional responsiveness until the later stages of the disease.
People with vascular dementia frequently wander at night and often have other problems commonly found in people who have had a stroke, including depression and incontinence.
There are several types of vascular dementia, which vary slightly in their causes and symptoms. One type, called multi-infarct dementia (MID), is caused by numerous small strokes in the brain. MID typically includes multiple damaged areas, called infarcts, along with extensive lesions in the white matter, or nerve fibers, of the brain.
Because the infarcts in MID affect isolated areas of the brain, the symptoms are often limited to one side of the body or they may affect just one or a few specific functions, such as language. Neurologists call these “local” or “focal” symptoms, as opposed to the “global” symptoms seen in AD, which affect many functions and are not restricted to one side of the body.
Although not all strokes cause dementia, in some cases a single stroke can damage the brain enough to cause dementia. This condition is called single-infarct dementia. Dementia is more common when the stroke takes place on the left side (hemisphere) of the brain and/or when it involves the hippocampus, a brain structure important for memory.
Another type of vascular dementia is called Binswanger’s disease. This rare form of dementia is characterized by damage to small blood vessels in the white matter of the brain (white matter is found in the inner layers of the brain and contains many nerve fibers coated with a whitish, fatty substance called myelin). Binswanger’s disease leads to brain lesions, loss of memory, disordered cognition, and mood changes. Patients with this disease often show signs of abnormal blood pressure, stroke, blood abnormalities, disease of the large blood vessels in the neck, and/or disease of the heart valves. Other prominent features include urinary incontinence, difficulty walking, clumsiness, slowness, lack of facial expression, and speech difficulty. These symptoms, which usually begin after the age of 60, are not always present in all patients and may sometimes appear only temporarily. Treatment of Binswanger’s disease is symptomatic, and may include the use of medications to control high blood pressure, depression, heart arrhythmias, and low blood pressure. The disorder often includes episodes of partial recovery.
Another type of vascular dementia is linked to a rare hereditary disorder called CADASIL, which stands for cerebral autosomal dominant arteriopathy with subcortical infarct and leukoencephalopathy. CADASIL is linked to abnormalities of a specific gene, Notch3, which is located on chromosome 19. This condition causes multi-infarct dementia as well as stroke, migraine with aura, and mood disorders. The first symptoms usually appear in people who are in their twenties, thirties, or forties and affected individuals often die by age 65. Researchers believe most people with CADASIL go undiagnosed, and the actual prevalence of the disease is not yet known.
Other causes of vascular dementia include vasculitis, an inflammation of the blood vessel system; profound hypotension (low blood pressure); and lesions caused by brain hemorrhage. The autoimmune disease lupus erythematosus and the inflammatory disease temporal arteritis can also damage blood vessels in a way that leads to vascular dementia.
Lewy body dementia (LBD)
Lewy body dementia (LBD) is one of the most common types of progressive dementia. LBD usually occurs sporadically, in people with no known family history of the disease. However, rare familial cases have occasionally been reported.
In LBD, cells die in the brain’s cortex, or outer layer, and in a part of the mid-brain called the substantia nigra. Many of the remaining nerve cells in the substantia nigra contain abnormal structures called Lewy bodies that are the hallmark of the disease. Lewy bodies may also appear in the brain’s cortex, or outer layer. Lewy bodies contain a protein called alpha-synuclein that has been linked to Parkinson’s disease and several other disorders. Researchers, who sometimes refer to these disorders collectively as “synucleinopathies,” do not yet know why this protein accumulates inside nerve cells in LBD.
The symptoms of LBD overlap with AD in many ways, and may include memory impairment, poor judgment, and confusion. However, LBD typically also includes visual hallucinations, parkinsonian symptoms such as a shuffling gait and flexed posture, and day-to-day fluctuations in the severity of symptoms. Patients with LBD live an average of 7 years after symptoms begin.
There is no cure for LBD, and treatments are aimed at controlling the parkinsonian and psychiatric symptoms of the disorder. Patients sometimes respond dramatically to treatment with antiparkinsonian drugs and/or cholinesterase inhibitors, such as those used for AD. Some studies indicate that neuroleptic drugs, such as clozapine and olanzapine, also can reduce the psychiatric symptoms of this disease. But neuroleptic drugs may cause severe adverse reactions, so other therapies should be tried first and patients using these drugs should be closely monitored.
Lewy bodies are often found in the brains of people with Parkinson’s and AD. These findings suggest that either LBD is related to these other causes of dementia or that the diseases sometimes coexist in the same person.
Frontotemporal dementia (FTD)
Frontotemporal dementia (FTD), sometimes called frontal lobe dementia, describes a group of diseases characterized by degeneration of nerve cells – especially those in the frontal and temporal lobes of the brain. Unlike AD, FTD usually does not include formation of amyloid plaques. In many people with FTD, there is an abnormal form of tau protein in the brain, which accumulates into neurofibrillary tangles. This disrupts normal cell activities and may cause the cells to die.
Experts believe FTD accounts for 2 to 10 percent of all cases of dementia. Symptoms of FTD usually appear between the ages of 40 and 65. In many cases, people with FTD have a family history of dementia, suggesting that there is a strong genetic factor in the disease. The duration of FTD varies, with some patients declining rapidly over 2 to 3 years and others showing only minimal changes for many years. People with FTD live with the disease for an average of 5 to 10 years after diagnosis.
Because structures found in the frontal and temporal lobes of the brain control judgment and social behavior, people with FTD often have problems maintaining normal interactions and following social conventions. They may steal or exhibit impolite and socially inappropriate behavior, and they may neglect their normal responsibilities. Other common symptoms include loss of speech and language, compulsive or repetitive behavior, increased appetite, and motor problems such as stiffness and balance problems. Memory loss also may occur, although it typically appears late in the disease.
In one type of FTD called Pick’s disease, certain nerve cells become abnormal and swollen before they die. These swollen, or ballooned, neurons are one hallmark of the disease. The brains of people with Pick’s disease also have abnormal structures called Pick bodies, composed largely of the protein tau, inside the neurons. The cause of Pick’s disease is unknown, but it runs in some families and thus it is probably due at least in part to a faulty gene or genes. The disease usually begins after age 50 and causes changes in personality and behavior that gradually worsen over time. The symptoms of Pick’s disease are very similar to those of AD, and may include inappropriate social behavior, loss of mental flexibility, language problems, and difficulty with thinking and concentration. There is currently no way to slow the progressive degeneration found in Pick’s disease. However, medication may be helpful in reducing aggression and other behavioral problems, and in treating depression.
In some cases, familial FTD is linked to a mutation in the tau gene. This disorder, called frontotemporal dementia with parkinsonism linked to chromosome 17 (FTDP-17), is much like other types of FTD but often includes psychiatric symptoms such as delusions and hallucinations.
Primary progressive aphasia (PPA) is a type of FTD that may begin in people as early as their forties. “Aphasia” is a general term used to refer to deficits in language functions, such as speaking, understanding what others are saying, and naming common objects. In PPA one or more of these functions can become impaired. Symptoms often begin gradually and progress slowly over a period of years. As the disease progresses, memory and attention may also be impaired and patients may show personality and behavior changes. Many, but not all, people with PPA eventually develop symptoms of dementia.
HIV-associated dementia (HAD)
HIV-associated dementia (HAD) results from infection with the human immunodeficiency virus (HIV) that causes AIDS. HAD can cause widespread destruction of the brain’s white matter. This leads to a type of dementia that generally includes impaired memory, apathy, social withdrawal, and difficulty concentrating. People with HAD often develop movement problems as well. There is no specific treatment for HAD, but AIDS drugs can delay onset of the disease and may help to reduce symptoms. `
Huntington’s disease (HD)
Huntington’s disease (HD) is a hereditary disorder caused by a faulty gene for a protein called huntingtin. The children of people with the disorder have a 50 percent chance of inheriting it. The disease causes degeneration in many regions of the brain and spinal cord. Symptoms of HD usually begin when patients are in their thirties or forties, and the average life expectancy after diagnosis is about 15 years.
Cognitive symptoms of HD typically begin with mild personality changes, such as irritability, anxiety, and depression, and progress to severe dementia. Many patients also show psychotic behavior. HD causes chorea – involuntary jerky, arrhythmic movements of the body – as well as muscle weakness, clumsiness, and gait disturbances.
Dementia pugilistica
Dementia pugilistica, also called chronic traumatic encephalopathy or Boxer’s syndrome, is caused by head trauma, such as that experienced by people who have been punched many times in the head during boxing. The most common symptoms of the condition are dementia and parkinsonism, which can appear many years after the trauma ends. Affected individuals may also develop poor coordination and slurred speech. A single traumatic brain injury may also lead to a disorder called post-traumatic dementia (PTD). PTD is much like dementia pugilistica but usually also includes long-term memory problems. Other symptoms vary depending on which part of the brain was damaged by the injury.
Corticobasal degeneration (CBD)
Corticobasal degeneration (CBD) is a progressive disorder characterized by nerve cell loss and atrophy of multiple areas of the brain. Brain cells from people with CBD often have abnormal accumulations of the protein tau. CBD usually progresses gradually over the course of 6 to 8 years. Initial symptoms, which typically begin at or around age 60, may first appear on one side of the body but eventually will affect both sides. Some of the symptoms, such as poor coordination and rigidity, are similar to those found in Parkinson’s disease. Other symptoms may include memory loss, dementia, visual-spatial problems, apraxia (loss of the ability to make familiar, purposeful movements), hesitant and halting speech, myoclonus (involuntary muscular jerks), and dysphagia (difficulty swallowing). Death is often caused by pneumonia or other secondary problems such as sepsis (severe infection of the blood) or pulmonary embolism (a blood clot in the lungs).
There are no specific treatments available for CBD. Drugs such as clonazepam may help with myoclonus, however, and occupational, physical, and speech therapy can help in managing the disabilities associated with this disease. The symptoms of the disease often do not respond to Parkinson’s medications or other drugs.
Creutzfeldt-Jakob disease (CJD)
Creutzfeldt-Jakob disease (CJD) is a rare, degenerative, fatal brain disorder that affects about one in every million people per year worldwide. Symptoms usually begin after age 60 and most patients die within 1 year. Many researchers believe CJD results from an abnormal form of a protein called a prion. Most cases of CJD occur sporadically – that is, in people who have no known risk factors for the disease. However, about 5 to 10 percent of cases of CJD in the United States are hereditary, caused by a mutation in the gene for the prion protein. In rare cases, CJD can also be acquired through exposure to diseased brain or nervous system tissue, usually through certain medical procedures. There is no evidence that CJD is contagious through the air or through casual contact with a CJD patient.
Patients with CJD may initially experience problems with muscular coordination; personality changes, including impaired memory, judgment, and thinking; and impaired vision. Other symptoms may include insomnia and depression. As the illness progresses, mental impairment becomes severe. Patients often develop myoclonus and they may go blind. They eventually lose the ability to move and speak, and go into a coma. Pneumonia and other infections often occur in these patients and can lead to death.
CJD belongs to a family of human and animal diseases known as the transmissible spongiform encephalopathies (TSEs). Spongiform refers to the characteristic appearance of infected brains, which become filled with holes until they resemble sponges when viewed under a microscope. CJD is the most common of the known human TSEs. Others include fatal familial insomnia and Gerstmann-Straussler-Scheinker disease (see below).
In recent years, a new type of CJD, called variant CJD (vCJD), has been found in Great Britain and several other European countries. The initial symptoms of vCJD are different from those of classic CJD and the disorder typically occurs in younger patients. Research suggests that vCJD may have resulted from human consumption of beef from cattle with a TSE disease called bovine spongiform encephalopathy (BSE), also known as “mad cow disease.”
Other rare hereditary dementias
Other rare hereditary dementias include Gerstmann-Straussler-Scheinker (GSS) disease, fatal familial insomnia, familial British dementia, and familial Danish dementia. Symptoms of GSS typically include ataxia and progressive dementia that begins when people are between 50 and 60 years old. The disease may last for several years before patients eventually die. Fatal familial insomnia causes degeneration of a brain region called the thalamus, which is partially responsible for controlling sleep. It causes a progressive insomnia that eventually leads to a complete inability to sleep. Other symptoms may include poor reflexes, dementia, hallucinations, and eventually coma. It can be fatal within 7 to 13 months after symptoms begin but may last longer. Familial British dementia and familial Danish dementia have been linked to two different defects in a gene found on chromosome 13. The symptoms of both diseases include progressive dementia, paralysis, and loss of balance.
Secondary Dementias
Dementia may occur in patients who have other disorders that primarily affect movement or other functions. These cases are often referred to as secondary dementias. The relationship between these disorders and the primary dementias is not always clear. For instance, people with advanced Parkinson’s disease, which is primarily a movement disorder, sometimes develop symptoms of dementia. Many Parkinson’s patients also have amyloid plaques and neurofibrillary tangles like those found in AD. The two diseases may be linked in a yet-unknown way, or they may simply coexist in some people. People with Parkinson’s and associated dementia sometimes show signs of Lewy body dementia or progressive supranuclear palsy at autopsy, suggesting that these diseases may also overlap with Parkinson’s or that Parkinson’s is sometimes misdiagnosed.
Other disorders that may include symptoms of dementia include multiple sclerosis; presenile dementia with motor neuron disease, also called ALS dementia; olivopontocerebellar atrophy (OPCA); Wilson’s disease; and normal pressure hydrocephalus (NPH)
Dementias in Children
While it is usually found in adults, dementia can also occur in children. For example, infections and poisoning can lead to dementia in people of any age. In addition, some disorders unique to children can cause dementia.
Niemann-Pick disease is a group of inherited disorders that affect metabolism and are caused by specific genetic mutations. Patients with Niemann-Pick disease cannot properly metabolize cholesterol and other lipids. Consequently, excessive amounts of cholesterol accumulate in the liver and spleen and excessive amounts of other lipids accumulate in the brain. Symptoms may include dementia, confusion, and problems with learning and memory. These diseases usually begin in young school-age children but may also appear during the teen years or early adulthood.
Batten disease is a fatal, hereditary disorder of the nervous system that begins in childhood. Symptoms are linked to a buildup of substances called lipopigments in the body’s tissues. The early symptoms include personality and behavior changes, slow learning, clumsiness, or stumbling. Over time, affected children suffer mental impairment, seizures, and progressive loss of sight and motor skills. Eventually, children with Batten disease develop dementia and become blind and bedridden. The disease is often fatal by the late teens or twenties.
Lafora body disease is a rare genetic disease that causes seizures, rapidly progressive dementia, and movement problems. These problems usually begin in late childhood or the early teens. Children with Lafora body disease have microscopic structures called Lafora bodies in the brain, skin, liver, and muscles. Most affected children die within 2 to 10 years after the onset of symptoms.
A number of other childhood-onset disorders can include symptoms of dementia. Among these are mitochondrial myopathies, Rasmussen’s encephalitis, mucopolysaccharidosis III (Sanfilippo syndrome), neurodegeneration with brain iron accumulation, and leukodystrophies such as Alexander disease, Schilder’s disease, and metachromatic leukodystrophy
What Other Conditions Can Cause Dementia?
Doctors have identified many other conditions that can cause dementia or dementia-like symptoms. Many of these conditions are reversible with appropriate treatment.
Reactions to medications. Medications can sometimes lead to reactions or side effects that mimic dementia. These dementia-like effects can occur in reaction to just one drug or they can result from drug interactions. They may have a rapid onset or they may develop slowly over time.
Metabolic problems and endocrine abnormalities. Thyroid problems can lead to apathy, depression, or dementia. Hypoglycemia, a condition in which there is not enough sugar in the bloodstream, can cause confusion or personality changes. Too little or too much sodium or calcium can also trigger mental changes. Some people have an impaired ability to absorb vitamin B12, which creates a condition called pernicious anemia that can cause personality changes, irritability, or depression. Tests can determine if any of these problems are present.
Nutritional deficiencies. Deficiencies of thiamine (vitamin B1) frequently result from chronic alcoholism and can seriously impair mental abilities, in particular memories of recent events. Severe deficiency of vitamin B3 can cause a neurological illness called pellagra that may include dementia. Deficiencies of vitamin B12 also have been linked to dementia in some cases. Dehydration can also cause mental impairment that can resemble dementia.
Infections. Many infections can cause neurological symptoms, including confusion or delirium, due to fever or other side effects of the body’s fight to overcome the infection. Meningitis and encephalitis, which are infections of the brain or the membrane that covers it, can cause confusion, sudden severe dementia, withdrawal from social interaction, impaired judgment, or memory loss. Untreated syphilis also can damage the nervous system and cause dementia. In rare cases, Lyme disease can cause memory or thinking difficulties. People in the advanced stages of AIDS also may develop a form of dementia (see HIV-associated dementia, page 14). People with compromised immune systems, such as those with leukemia and AIDS, may also develop an infection called progressive multifocal leukoencephalopathy (PML). PML is caused by a common human polyomavirus, JC virus, and leads to damage or destruction of the myelin sheath that covers nerve cells. PML can lead to confusion, difficulty with thinking or speaking, and other mental problems.
Subdural hematomas. Subdural hematomas, or bleeding between the brain’s surface and its outer covering (the dura), can cause dementia-like symptoms and changes in mental function.
Poisoning. Exposure to lead, other heavy metals, or other poisonous substances can lead to symptoms of dementia. These symptoms may or may not resolve after treatment, depending on how badly the brain is damaged. People who have abused substances such as alcohol and recreational drugs sometimes display signs of dementia even after the substance abuse has ended. This condition is known as substance-induced persisting dementia.
Brain tumors. In rare cases, people with brain tumors may develop dementia because of damage to their brains. Symptoms may include changes in personality, psychotic episodes, or problems with speech, language, thinking, and memory.
Anoxia. Anoxia and a related term, hypoxia, are often used interchangeably to describe a state in which there is a diminished supply of oxygen to an organ’s tissues. Anoxia may be caused by many different problems, including heart attack, heart surgery, severe asthma, smoke or carbon monoxide inhalation, high-altitude exposure, strangulation, or an overdose of anesthesia. In severe cases of anoxia the patient may be in a stupor or a coma for periods ranging from hours to days, weeks, or months. Recovery depends on the severity of the oxygen deprivation. As recovery proceeds, a variety of psychological and neurological abnormalities, such as dementia or psychosis, may occur. The person also may experience confusion, personality changes, hallucinations, or memory loss.
Heart and lung problems. The brain requires a high level of oxygen in order to carry out its normal functions. Therefore, problems such as chronic lung disease or heart problems that prevent the brain from receiving adequate oxygen can starve brain cells and lead to the symptoms of dementia.
What Conditions Are Not Dementia?
Age-related cognitive decline. As people age, they usually experience slower information processing and mild memory impairment. In addition, their brains frequently decrease in volume and some nerve cells, or neurons, are lost. These changes, called age-related cognitive decline, are normal and are not considered signs of dementia.
Mild cognitive impairment. Some people develop cognitive and memory problems that are not severe enough to be diagnosed as dementia but are more pronounced than the cognitive changes associated with normal aging. This condition is called mild cognitive impairment. Although many patients with this condition later develop dementia, some do not. Many researchers are studying mild cognitive impairment to find ways to treat it or prevent it from progressing to dementia.
Depression. People with depression are frequently passive or unresponsive, and they may appear slow, confused, or forgetful. Other emotional problems can also cause symptoms that sometimes mimic dementia.
Delirium. Delirium is characterized by confusion and rapidly altering mental states. The person may also be disoriented, drowsy, or incoherent, and may exhibit personality changes. Delirium is usually caused by a treatable physical or psychiatric illness, such as poisoning or infections. Patients with delirium often, though not always, make a full recovery after their underlying illness is treated.
Causes Dementia
All forms of dementia result from the death of nerve cells and/or the loss of communication among these cells. The human brain is a very complex and intricate machine and many factors can interfere with its functioning. Researchers have uncovered many of these factors, but they have not yet been able to fit these puzzle pieces together in order to form a complete picture of how dementias develop.
Many types of dementia, including AD, Lewy body dementia, Parkinson’s dementia, and Pick’s disease, are characterized by abnormal structures called inclusions in the brain. Because these inclusions, which contain abnormal proteins, are so common in people with dementia, researchers suspect that they play a role in the development of symptoms. However, that role is unknown, and in some cases the inclusions may simply be a side effect of the disease process that leads to the dementia.
Genes clearly play a role in the development of some kinds of dementia. However, in AD and many other disorders, the dementia usually cannot be tied to a single abnormal gene. Instead, these forms of dementia appear to result from a complex interaction of genes, lifestyle factors, and other environmental influences.
Researchers have identified several genes that influence susceptibility to AD. Mutations in three of the known genes for AD – genes that control the production of proteins such as amyloid precursor protein (APP), presenilin 1, and presenilin 2 – are linked to early-onset forms of the disease.
Variations in another gene, called apolipoprotein E (apoE), have been linked to an increased risk of late-onset AD. The apoE gene does not cause the disease by itself, but one version of the gene, called apoE epsilon4 (apoE E4), appears to increase the risk of AD. People with two copies of the apoE E4 gene have about ten times the risk of developing AD compared to people without apoE E4. This gene variant seems to encourage amyloid deposition in the brain. One study also found that this gene is associated with shorter survival in men with AD. In contrast, another version of the apoE gene, called apoE E2, appears to protect against AD.
Studies have suggested that mutations in another gene, called CYP46, may contribute to an increased risk of developing late-onset sporadic AD. This gene normally produces a protein that helps the brain metabolize cholesterol.
Scientists are trying to determine how beta amyloid influences the development of AD. A number of studies indicate that the buildup of this protein initiates a complex chain of events that culminates in dementia. One study found that beta amyloid buildup in the brain triggers cells called microglia, which act like janitors that mop up potentially harmful substances in the brain, to release a potent neurotoxin called peroxynitrite. This may contribute to nerve cell death in AD. Another study found that beta amyloid causes a protein called p35 to be split into two proteins. One of the resulting proteins triggers changes in the tau protein that lead to formation of neurofibrillary tangles. A third study found that beta amyloid activates cell-death enzymes called caspases that alter the tau protein in a way that causes it to form tangles. Researchers believe these tangles may contribute to the neuron death in AD.
Vascular dementia can be caused by cerebrovascular disease or any other condition that prevents normal blood flow to the brain. Without a normal supply of blood, brain cells cannot obtain the oxygen they need to work correctly, and they often become so deprived that they die.
The causes of other types of dementias vary. Some, such as CJD and GSS, have been tied to abnormal forms of specific proteins. Others, including Huntington’s disease and FTDP-17, have been linked to defects in a single gene. Post-traumatic dementia is directly related to brain cell death after injury. HIV-associated dementia is clearly tied to infection by the HIV virus, although the exact way the virus causes damage is not yet certain. For other dementias, such as corticobasal degeneration and most types of frontotemporal dementia, the underlying causes have not yet been identified.
Risk Factors for Dementia
Researchers have identified several risk factors that affect the likelihood of developing one or more kinds of dementia. Some of these factors are modifiable, while others are not.
Age. The risk of AD, vascular dementia, and several other dementias goes up significantly with advancing age.
Genetics/family history. As described in the section “What Causes Dementia?” researchers have discovered a number of genes that increase the risk of developing AD. Although people with a family history of AD are generally considered to be at heightened risk of developing the disease themselves, many people with a family history never develop the disease, and many without a family history of the disease do get it. In most cases, it is still impossible to predict a specific person’s risk of the disorder based on family history alone. Some families with CJD, GSS, or fatal familial insomnia have mutations in the prion protein gene, although these disorders can also occur in people without the gene mutation. Individuals with these mutations are at significantly higher risk of developing these forms of dementia. Abnormal genes are also clearly implicated as risk factors in Huntington’s disease, FTDP-17, and several other kinds of dementia. These dementias are described in the section “What are the different kinds of dementia?”
Smoking and alcohol use. Several recent studies have found that smoking significantly increases the risk of mental decline and dementia. People who smoke have a higher risk of atherosclerosis and other types of vascular disease, which may be the underlying causes for the increased dementia risk. Studies also have found that drinking large amounts of alcohol appears to increase the risk of dementia. However, other studies have suggested that people who drink moderately have a lower risk of dementia than either those who drink heavily or those who completely abstain from drinking.
Atherosclerosis. Atherosclerosis is the buildup of plaque – deposits of fatty substances, cholesterol, and other matter – in the inner lining of an artery. Atherosclerosis is a significant risk factor for vascular dementia, because it interferes with the delivery of blood to the brain and can lead to stroke. Studies have also found a possible link between atherosclerosis and AD.
Cholesterol.High levels of low-density lipoprotein (LDL), the so-called bad form of cholesterol, appear to significantly increase a person’s risk of developing vascular dementia. Some research has also linked high cholesterol to an increased risk of AD.
Plasma homocysteine.Research has shown that a higher-than-average blood level of homocysteine – a type of amino acid – is a strong risk factor for the development of AD and vascular dementia.
Diabetes. Diabetes is a risk factor for both AD and vascular dementia. It is also a known risk factor for atherosclerosis and stroke, both of which contribute to vascular dementia.
Mild cognitive impairment. While not all people with mild cognitive impairment develop dementia, people with this condition do have a significantly increased risk of dementia compared to the rest of the population. One study found that approximately 40 percent of people over age 65 who were diagnosed with mild cognitive impairment developed dementia within 3 years.
Down syndrome. Studies have found that most people with Down syndrome develop characteristic AD plaques and neurofibrillary tangles by the time they reach middle age. Many, but not all, of these individuals also develop symptoms of dementia.
How Is Dementia Diagnosed?
Doctors employ a number of strategies to diagnose dementia. It is important that they rule out any treatable conditions, such as depression, normal pressure hydrocephalus, or vitamin B12 deficiency, which can cause similar symptoms.
Early, accurate diagnosis of dementia is important for patients and their families because it allows early treatment of symptoms. For people with AD or other progressive dementias, early diagnosis may allow them to plan for the future while they can still help to make decisions. These people also may benefit from drug treatment.
The “gold standard” for diagnosing dementia, autopsy, does not help the patient or caregivers. Therefore, doctors have devised a number of techniques to help identify dementia with reasonable accuracy while the patient is still alive.
Patient history
Doctors often begin their examination of a patient suspected of having dementia by asking questions about the patient’s history. For example, they may ask how and when symptoms developed and about the patient’s overall medical condition. They also may try to evaluate the patient’s emotional state, although patients with dementia often may be unaware of or in denial about how their disease is affecting them. Family members also may deny the existence of the disease because they do not want to accept the diagnosis and because, at least in the beginning, AD and other forms of dementia can resemble normal aging. Therefore additional steps are necessary to confirm or rule out a diagnosis of dementia.
Physical examination
A physical examination can help rule out treatable causes of dementia and identify signs of stroke or other disorders that can contribute to dementia. It can also identify signs of other illnesses, such as heart disease or kidney failure, that can overlap with dementia. If a patient is taking medications that may be causing or contributing to his or her symptoms, the doctor may suggest stopping or replacing some medications to see if the symptoms go away.
Neurological evaluations
Doctors will perform a neurological examination, looking at balance, sensory function, reflexes, and other functions, to identify signs of conditions – for example movement disorders or stroke – that may affect the patient’s diagnosis or are treatable with drugs.
Cognitive and neuropsychological tests
Doctors use tests that measure memory, language skills, math skills, and other abilities related to mental functioning to help them diagnose a patient’s condition accurately. For example, people with AD often show changes in so-called executive functions (such as problem-solving), memory, and the ability to perform once-automatic tasks.
Doctors often use a test called the Mini-Mental State Examination (MMSE) to assess cognitive skills in people with suspected dementia. This test examines orientation, memory, and attention, as well as the ability to name objects, follow verbal and written commands, write a sentence spontaneously, and copy a complex shape. Doctors also use a variety of other tests and rating scales to identify specific types of cognitive problems and abilities.
Brain scans
Doctors may use brain scans to identify strokes, tumors, or other problems that can cause dementia. Also, cortical atrophy -degeneration of the brain’s cortex (outer layer) – is common in many forms of dementia and may be visible on a brain scan. The brain’s cortex normally appears very wrinkled, with ridges of tissue (called gyri) separated by “valleys” called sulci. In individuals with cortical atrophy, the progressive loss of neurons causes the ridges to become thinner and the sulci to grow wider. As brain cells die, the ventricles (or fluid-filled cavities in the middle of the brain) expand to fill the available space, becoming much larger than normal. Brain scans also can identify changes in the brain’s structure and function that suggest AD.
The most common types of brain scans are computed tomographic (CT) scans and magnetic resonance imaging (MRI). Doctors frequently request a CT scan of the brain when they are examining a patient with suspected dementia. These scans, which use X-rays to detect brain structures, can show evidence of brain atrophy, strokes and transient ischemic attacks (TIAs), changes to the blood vessels, and other problems such as hydrocephalus and subdural hematomas. MRI scans use magnetic fields and focused radio waves to detect hydrogen atoms in tissues within the body. They can detect the same problems as CT scans but they are better for identifying certain conditions, such as brain atrophy and damage from small TIAs.
Doctors also may use electroencephalograms (EEGs) in people with suspected dementia. In an EEG, electrodes are placed on the scalp over several parts of the brain in order to detect and record patterns of electrical activity and check for abnormalities. This electrical activity can indicate cognitive dysfunction in part or all of the brain. Many patients with moderately severe to severe AD have abnormal EEGs. An EEG may also be used to detect seizures, which occur in about 10 percent of AD patients as well as in many other disorders. EEGs also can help diagnose CJD.
Several other types of brain scans allow researchers to watch the brain as it functions. These scans, called functional brain imaging, are not often used as diagnostic tools, but they are important in research and they may ultimately help identify people with dementia earlier than is currently possible. Functional brain scans include functional MRI (fMRI), single photon-emission computed tomography (SPECT), positron emission tomography (PET), and magnetoencephalography (MEG). fMRI uses radio waves and a strong magnetic field to measure the metabolic changes that take place in active parts of the brain. SPECT shows the distribution of blood in the brain, which generally increases with brain activity. PET scans can detect changes in glucose metabolism, oxygen metabolism, and blood flow, all of which can reveal abnormalities of brain function. MEG shows the electromagnetic fields produced by the brain’s neuronal activity.
Laboratory tests
Doctors may use a variety of laboratory tests to help diagnose dementia and/or rule out other conditions, such as kidney failure, that can contribute to symptoms. A partial list of these tests includes a complete blood count, blood glucose test, urinalysis, drug and alcohol tests (toxicology screen), cerebrospinal fluid analysis (to rule out specific infections that can affect the brain), and analysis of thyroid and thyroid-stimulating hormone levels. A doctor will order only the tests that he or she feels are necessary and/or likely to improve the accuracy of a diagnosis.
Psychiatric evaluation
A psychiatric evaluation may be obtained to determine if depression or another psychiatric disorder may be causing or contributing to a person’s symptoms.
Presymptomatic testing
Testing people before symptoms begin to determine if they will develop dementia is not possible in most cases. However, in disorders such as Huntington’s where a known gene defect is clearly linked to the risk of the disease, a genetic test can help identify people who are likely to develop the disease. Since this type of genetic information can be devastating, people should carefully consider whether they want to undergo such testing.
Researchers are examining whether a series of simple cognitive tests, such as matching words with pictures, can predict who will develop dementia. One study suggested that a combination of a verbal learning test and an odor-identification test can help identify AD before symptoms become obvious. Other studies are looking at whether memory tests and brain scans can be useful indicators of future dementia.
Is There Any Treatment for Dementia?
While treatments to reverse or halt disease progression are not available for most of the dementias, patients can benefit to some extent from treatment with available medications and other measures, such as cognitive training.
Drugs to specifically treat AD and some other progressive dementias are now available and are prescribed for many patients. Although these drugs do not halt the disease or reverse existing brain damage, they can improve symptoms and slow the progression of the disease. This may improve the patient’s quality of life, ease the burden on caregivers, and/or delay admission to a nursing home. Many researchers are also examining whether these drugs may be useful for treating other types of dementia.
Many people with dementia, particularly those in the early stages, may benefit from practicing tasks designed to improve performance in specific aspects of cognitive functioning. For example, people can sometimes be taught to use memory aids, such as mnemonics, computerized recall devices, or note taking.
Behavior modification – rewarding appropriate or positive behavior and ignoring inappropriate behavior – also may help control unacceptable or dangerous behaviors.
Alzheimer’s disease
Most of the drugs currently approved by the U. S. Food and Drug Administration (FDA) for AD fall into a category called cholinesterase inhibitors. These drugs slow the breakdown of the neurotransmitter acetylcholine, which is reduced in the brains of people with AD. Acetylcholine is important for the formation of memories and it is used in the hippocampus and the cerebral cortex, two brain regions that are affected by AD. There are currently four cholinesterase inhibitors approved for use in the United States: tacrine (Cognex), donepezil (Aricept), rivastigmine (Exelon), and galantamine (Reminyl). These drugs temporarily improve or stabilize memory and thinking skills in some individuals. Many studies have shown that cholinesterase inhibitors help to slow the decline in mental functions associated with AD, and that they can help reduce behavioral problems and improve the ability to perform everyday tasks. However, none of these drugs can stop or reverse the course of AD. A fifth drug, memantine (Namenda), is also approved for use in the United States. Unlike other drugs for AD, which affect acetylcholine levels, memantine works by regulating the activity of a neurotransmitter called glutamate that plays a role in learning and memory. Glutamate activity is often disrupted in AD. Because this drug works differently from cholinesterase inhibitors, combining memantine with other AD drugs may be more effective than any single therapy. One controlled clinical trial found that patients receiving donepezil plus memantine had better cognition and other functions than patients receiving donepezil alone. Doctors may also prescribe other drugs, such as anticonvulsants, sedatives, and antidepressants, to treat seizures, depression, agitation, sleep disorders, and other specific problems that can be associated with dementia. In 2005, research showed that use of “atypical” antipsychotic drugs such as olanzapine and risperdone to treat behavioral problems in elderly people with dementia was associated with an elevated risk of death in these patients. Most of the deaths were caused by heart problems or infections. The FDA has issued a public health advisory to alert patients and their caregivers to this safety issue.
Vascular dementia
There is no standard drug treatment for vascular dementia, although some of the symptoms, such as depression, can be treated. Most other treatments aim to reduce the risk factors for further brain damage. However, some studies have found that cholinesterase inhibitors, such as galantamine and other AD drugs, can improve cognitive function and behavioral symptoms in patients with early vascular dementia. The progression of vascular dementia can often be slowed significantly or halted if the underlying vascular risk factors for the disease are treated. To prevent strokes and TIAs, doctors may prescribe medicines to control high blood pressure, high cholesterol, heart disease, and diabetes. Doctors also sometimes prescribe aspirin, warfarin, or other drugs to prevent clots from forming in small blood vessels. When patients have blockages in blood vessels, doctors may recommend surgical procedures, such as carotid endarterectomy, stenting, or angioplasty, to restore the normal blood supply. Medications to relieve restlessness or depression or to help patients sleep better may also be prescribed.
Other dementias
Some studies have suggested that cholinesterase inhibitors, such as donepezil (Aricept), can reduce behavioral symptoms in some patients with Parkinson’s dementia. At present, no medications are approved specifically to treat or prevent FTD and most other types of progressive dementia. However, sedatives, antidepressants, and other medications may be useful in treating specific symptoms and behavioral problems associated with these diseases. Scientists continue to search for specific treatments to help people with Lewy body dementia. Current treatment is symptomatic, often involving the use of medication to control the parkinsonian and psychiatric symptoms. Although antiparkinsonian medication may help reduce tremor and loss of muscle movement, it may worsen symptoms such as hallucinations and delusions. Also, drugs prescribed for psychiatric symptoms may make the movement problems worse. Several studies have suggested that cholinesterase inhibitors may be able to improve cognitive function and behavioral symptoms in patients with Lewy body disease. There is no known treatment that can cure or control CJD. Current treatment is aimed at alleviating symptoms and making the patient as comfortable as possible. Opiate drugs can help relieve pain, and the drugs clonazepam and sodium valproate may help relieve myoclonus. During later stages of the disease, treatment focuses on supportive care, such as administering intravenous fluids and changing the person’s position frequently to prevent bedsores.
Prevention
Research has revealed a number of factors that may be able to prevent or delay the onset of dementia in some people. For example, studies have shown that people who maintain tight control over their glucose levels tend to score better on tests of cognitive function than those with poorly controlled diabetes. Several studies also have suggested that people who engage in intellectually stimulating activities, such as social interactions, chess, crossword puzzles, and playing a musical instrument, significantly lower their risk of developing AD and other forms of dementia. Scientists believe mental activities may stimulate the brain in a way that increases the person’s “cognitive reserve” – the ability to cope with or compensate for the pathologic changes associated with dementia.
Researchers are studying other steps people can take that may help prevent AD in some cases. So far, none of these factors has been definitively proven to make a difference in the risk of developing the disease. Moreover, most of the studies addressed only AD, and the results may or may not apply to other forms of dementia. Nevertheless, scientists are encouraged by the results of these early studies and many believe it will eventually become possible to prevent some forms of dementia. Possible preventive actions include:
· Lowering homocysteine. In one study, elevated blood levels of the amino acid homocysteine were associated with a 2.9 times greater risk of AD and a 4.9 times greater risk of vascular dementia. A preliminary study has shown that high doses of three B vitamins that help lower homocysteine levels – folic acid, B12, and B6 – appear to slow the progression of AD. Researchers are conducting a multi-center clinical trial to test this effect in a larger group of patients.
· Lowering cholesterol levels. Research has suggested that people with high cholesterol levels have an increased risk of developing AD. Cholesterol is involved in formation of amyloid plaques in the brain. Mutations in a gene called CYP46 and the apoE E4 gene variant, both of which have been linked to an increased risk of AD, are also involved in cholesterol metabolism. Several studies have also found that the use of drugs called statins, which lower cholesterol levels, is associated with a lower likelihood of cognitive impairment.
· Lowering blood pressure.Several studies have shown that antihypertensive medicine reduces the odds of cognitive impairment in elderly people with high blood pressure. One large European study found a 55 percent lower risk of dementia in people over 60 who received drug treatment for hypertension. These people had a reduced risk of both AD and vascular dementia.
· Exercise. Regular exercise stimulates production of chemicals called growth factors that help neurons survive and adapt to new situations. These gains may help to delay the onset of dementia symptoms. Exercise also may reduce the risk of brain damage from atherosclerosis.
· Education. Researchers have found evidence that formal education may help protect people against the effects of Alzheimer’s disease. In one study, researchers found that people with more years of formal education had relatively less mental decline than people with less schooling, regardless of the number of amyloid plaques and neurofibrillary tangles each person had in his or her brain. The researchers think education may cause the brain to develop robust nerve cell networks that can help compensate for the cell damage caused by Alzheimer’s disease.
· Controlling inflammation. Many studies have suggested that inflammation may contribute to AD. Moreover, autopsies of people who died with AD have shown widespread inflammation in the brain that appeared to be caused by the accumulation of beta amyloid. Another study found that men with high levels of C-reactive protein, a general marker of inflammation, had a significantly increased risk of AD and other kinds of dementia.
· Nonsteroidal anti-inflammatory drugs (NSAIDs). Research indicates that long-term use of NSAIDs – ibuprofen, naproxen, and similar drugs – may prevent or delay the onset of AD. Researchers are not sure how these drugs may protect against the disease, but some or all of the effect may be due to reduced inflammation. A 2003 study showed that these drugs also bind to amyloid plaques and may help to dissolve them and prevent formation of new plaques.
The risk of vascular dementia is strongly correlated with risk factors for stroke, including high blood pressure, diabetes, elevated cholesterol levels, and smoking. This type of dementia may be prevented in many cases by changing lifestyle factors, such as excessive weight and high blood pressure, which are associated with an increased risk of cerebrovascular disease. One European study found that treating isolated systolic hypertension (high blood pressure in which only the systolic or top number is high) in people age 60 and older reduced the risk of dementia by 50 percent. These studies strongly suggest that effective use of current treatments can prevent many future cases of vascular dementia.
A study published in 2005 found that people with mild cognitive impairment who took 10 mg/day of the drug donepezil had a significantly reduced risk of developing AD during the first two years of treatment, compared to people who received vitamin E or a placebo. By the end of the third year, however, the rate of AD was just as high in the people treated with donepezil as it was in the other two groups.
What Kind of Care Does a Person with Dementia Need?
People with moderate and advanced dementia typically need round-the-clock care and supervision to prevent them from harming themselves or others. They also may need assistance with daily activities such as eating, bathing, and dressing. Meeting these needs takes patience, understanding, and careful thought by the person’s caregivers.
A typical home environment can present many dangers and obstacles to a person with dementia, but simple changes can overcome many of these problems. For example, sharp knives, dangerous chemicals, tools, and other hazards should be removed or locked away. Other safety measures include installing bed and bathroom safety rails, removing locks from bedroom and bathroom doors, and lowering the hot water temperature to 120°F (48. 9°C) or less to reduce the risk of accidental scalding. People with dementia also should wear some form of identification at all times in case they wander away or become lost. Caregivers can help prevent unsupervised wandering by adding locks or alarms to outside doors.
People with dementia often develop behavior problems because of frustration with specific situations. Understanding and modifying or preventing the situations that trigger these behaviors may help to make life more pleasant for the person with dementia as well as his or her caregivers. For instance, the person may be confused or frustrated by the level of activity or noise in the surrounding environment. Reducing unnecessary activity and noise (such as limiting the number of visitors and turning off the television when it’s not in use) may make it easier for the person to understand requests and perform simple tasks. Confusion also may be reduced by simplifying home decorations, removing clutter, keeping familiar objects nearby, and following a predictable routine throughout the day. Calendars and clocks also may help patients orient themselves.
People with dementia should be encouraged to continue their normal leisure activities as long as they are safe and do not cause frustration. Activities such as crafts, games, and music can provide important mental stimulation and improve mood. Some studies have suggested that participating in exercise and intellectually stimulating activities may slow the decline of cognitive function in some people.
Many studies have found that driving is unsafe for people with dementia. They often get lost and they may have problems remembering or following rules of the road. They also may have difficulty processing information quickly and dealing with unexpected circumstances. Even a second of confusion while driving can lead to an accident. Driving with impaired cognitive functions can also endanger others. Some experts have suggested that regular screening for changes in cognition might help to reduce the number of driving accidents among elderly people, and some states now require that doctors report people with AD to their state motor vehicle department. However, in many cases, it is up to the person’s family and friends to ensure that the person does not drive.
The emotional and physical burden of caring for someone with dementia can be overwhelming. Support groups can often help caregivers deal with these demands and they can also offer helpful information about the disease and its treatment. It is important that caregivers occasionally have time off from round-the-clock nursing demands. Some communities provide respite facilities or adult day care centers that will care for dementia patients for a period of time, giving the primary caregivers a break. Eventually, many patients with dementia require the services of a full-time nursing home.