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the indications for obtaining such scans have been controversial. Several behavioral presentations should generally prompt a neuroimaging scan, including acute confusional state or dementia of unknown cause, the initial episode of undiagnosed psychosis, and the first presentation of personality change after age 40. Other indications include focal neurologic findings, movement disorders, incontinence, or evidence of increased intracranial pressure such as headache, nausea, vomiting, and papilledema on funduscopic examination.

BIBLIOGRAPHY
1. Cummings JL: Organic delusions: Phenomenology, anatomical considerations, and review. Br J Psychiatry

146:184-197, 1985. 2. Cummings JL: Psychosis in neurologic disease: Neurobiology and pathogenesis. Neuropsychiatry Neuropsychol Behav Neurol5: 144-150, 1992. 3. Cummings JL, Miller BL: Visual hallucinations:Clinical occurrence and use in differential diagnosis. West J Med 146:46-51, 1987. 4. Gorman DG, Cummings JL: Organic delusional syndrome. Semin Neurol 10:229-238, 1990. 5. Gould R, Miller BL, Goldberg MA, Benson DF: The validity of hysterical signs and symptoms. J Nerv Ment Dis 174593-597, 1986. 6. Larson E W Organic causes of mania. Mayo Clin Proc 63:906-912, 1988. 7. Mackenzie TB, Popkin MK: Organic anxiety syndrome.Am J Psychiatry 140:342-344, 1983. 8. Skuster DZ, Digre KB, Corbett JJ: Neurologic conditions presenting as psychiatric disorders. Psychiatr Clin NorthAm 15:311-333, 1992. 9. Strub RL: Mental disorders in brain disease. In Frederiks JA (ed): Handbook of Clinical Neurology, Vol 2. Amsterdam, Elsevier, 1985, pp 413-441. 10. Taylor D, Lewis S: Delirium. 3 Neurol Neurosurg Psychiatry 56742-751, 1993. 11. Filley CM, Kleinschmidt-DeMasters BK: Neurobehavioral presentations of brain neoplasms. West J Med 163:19-25, 1995. 12. Lyoo IK, Seol HY, Byun HS, Renshaw PF: Unsuspected multiple sclerosis in patients with psychiatric disorders: A magnetic resonance imaging study. J Neuropsychiatry Clin Neurosci 83-59, 1996. 13. Yudofsky SC, Hales RE (eds): Neuropsychiatry, 2nd ed. Washington, DC, .4mencan Psychiatric Press, 1992.

34. DEMENTIA
Roberta M.Rkhardsovt, M.D.

1. Define dementia.
Dementia is an impairment in intellectual functioning in at least two spheres. One of the spheres is memory; the second may be any other area of cognition.

Cognitive Functions That May Be Impaired in Dementia

Language Visuospatial ability Personality Judgment Object recognition Ability to dress and do other semiautomatic tasks Abstraction Calculation Information synthesis Problem solving

In contrast to delirium, the deficits of dementia are relatively stable over at least a few months. In contrast to mental retardation, the deficits are acquired. Memory disturbance is an early feature. It may be evidenced by inability to learn new material or loss of ability to recall previously learned material.

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The degree of interference in the patients life should be taken into account, as should education and intelligence. For example, a highly educated man may pass a screening test but still have significant impairment in his usual complex occupation. Alternatively, a retired manual laborer may show some deficits on exam but have no problems in his daily life. Third-party informants are helpful. People who know the patient best can report changes in functioning from a previous level as well as notice signs or symptoms of which the patient is unaware. More extensive, formal neuropsychological testing may be helpful if the diagnosis is in doubt.

2. Why is dementia an increasingly important problem?

The population of the U.S. is aging. It is estimated that by the year 2010 about 15% of Americans will be 65 or older, and 25% will be 55 or older. Currently the fastest growing segment of the population is people over the age of 85. The incidence of dementia rises steadily with age. About 5% of people over 65 have severe dementia, and another 10-IS% show mild-to-moderate symptoms. Of people over 80, one-fourth have severe dementia. Dementia is presently the fourth leading cause of death in the U.S. Death occurs because so much of the nervous system has failed that the entire body is profoundly affected. Such patients are mute, unable to eat, incontinent, and immobile. The immediate cause of death may be pneumonia, dehydration, malnutrition, or sepsis.

3. What are the causes of dementia?

Alzheimers disease is the most common cause of dementia, accounting for about 50% of cases. Multi-infarct dementia accounts for about 25%, and the remaining 25% are caused by a wide variety of other conditions or agents.

Causes o f Dementiu
Cortical dementias Alzheimers disease Picks disease (frontal lobe degeneration) Lewy body disease Vascular dementias Multiinfarct dementia Lacunar state Binswangers disease Movement disorders Parkinsons disease Huntingtons disease Wilsons disease Progressive supranuclear palsy Spinocerebellar degeneration Normal pressure hydrocephalus Dementia syndrome of depression Chronic confusional states Infections Toxic-metabolic encephalopathies Trauma Neoplasms Demyelinating diseases

4. What are the differences between cortical and subcortical dementias? Subcortical dementias are caused by disorders that affect mainly the basal ganglia, thalamus, and brainstem. Clinical characteristics contrast with those of the cortical dementias, which, as the name implies, affect mainly the cerebral cortex. Recognizing this distinction assists in differential diagnosis. Movement disorders exemplify the subcortical dementias. In addition to effects on the motor system, cognition and comprehension are slowed. The ability to synthesize and manipulate information for problem solving and decision making is impaired. Memory deficits are characterized by retrieval deficits. Clinically, patients with subcortical dementias may be helped by clues, structure, and prompting. Information registers, but it is difficult to retrieve from storage. Such strategies do not help patients with Alzheimers disease, because the information was not learned. Cortical dementias are characterized by language disturbances, agnosia and apraxia, visuospatial deficits, and impairment of judgment, abstraction, and calculation. Many deficits are characterized by problems with registering new information and, as the dementia progresses, with remote

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recall. For example, the patient with Alzheimers disease has great difficulty recalling the three objects commonly requested during mental status screening. Clues do not help. The patient may not even remember that three objects were mentioned, because the information was not registered in the memory. The neurologic exam shows no abnormalities in motor function, gait, reflexes, or posture. Alzheimers disease is the prototypic cortical dementia. Mixed dementias have features of both cortical and subcortical type. Multi-infarct dementia and Lewy Body disease (LBD) are the most common examples. LBD differs from Alzheimers disease in that it shows psychotic symptoms and parkinsonian features early in the course. Also, the course is more fluctuating. A tell-tale sign is extreme sensitivity to extrapyramidal side effects of antipsychotic medications. Cognitive deficit is more prominent than movement disorder, distinguishing it from Parkinsons disease.

5. What conditions are commonly mistaken for dementia? Normal aging is associated with some alterations in mental functioning. Benign senescent forgetfulness refers to alterations in memory function with age; it is characterized by decreased retrieval of learned information rather than inability to learn. Word-finding difficulties, especially proper names, are common in the elderly. If cognitive decline does not interfere with social or occupational functioning, it is not considered to indicate dementia. Delirium is an acute or subacute decline in mental functioning associated with a specific organic cause. Because patients with dementia are predisposed to delirium, the two conditions may be superimposed. Delirium is temporary and should remit when the underlying organic cause is treated. Medication effects are the most common, and most commonly missed, cause of delirium mistaken for dementia. Focal brain syndromes, such as isolated amnesias or aphasias, may be mistakenly diagnosed as dementia. Dementia, however, involves disturbances of memory and at least one other area of cognition. The careful clinician is not fooled by the aphasic patient who at first glance seems to have memory deficits. Nor does the careful clinician assume that an amnestic patient has dementia. All areas of cognition must be considered and tested. Major depression may cause reversible dementia, especially in the elderly. It may also cause symptoms that may be misdiagnosed as dementia: apathy, withdrawal, decrease in attention to personal grooming, personality changes, and loss of interest in activities. Such symptoms are prominent features of major depression as well as common symptoms of senile dementia. Because major depression may cause memory loss and actual decrease in cognitive abilities, it is considered a reversible cause of dementia. Careful attention to full mental status testing, including subtleties of mood and thought, is essential for proper diagnosis and treatment. Like delirium, depression also may coexist with dementia.
6. How is delirium distinguished from dementia? It is important to distinguish delirium from dementia, but the distinction is especially difficult when delirium overlies preexisting dementia. The main distinguishing feature is level of attention, which is impaired in patients with delirium. When interviewed patients answer a question asked of the person in the next bed rather than the question just posed to them, impairment of attention is suggested. An easy and accurate bedside test of attention is the so-called A test. The examiner recites a series of random letters at a steady rate of about one per second and asks the patient to indicate by a gesture every time he or she hears the letter A. The examiner should sometimes say the letter A two or three times in a row and also allow a long list of letters to pass by without an A. The patient should be able to perform this task for about a minute without error. Errors indicating impairment of attention include omissions and gesturing for the wrong letter. Perseverating after a series of letter As suggests frontal lobe dysfunction. Other features that suggest delirium rather than dementia include: Acute or subacute onset. Family and friends of the demented patient will have noticed problems for at least a few months.

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Dementia
Fluctuating course. The delirious patient may be confused one hour, clear the next, and confused again a few hours later. Clouding of consciousness. The delirious patient may not be fully alert or aware of surroundings. Florid hallucinations, most often visual or tactile. Illusions, or misrepresentations of sensory stimuli, such as believing that the coat rack is a person or the stethoscope a snake.

7. How are depression and dementia related? Both depression and dementia are common in the elderly. They may coexist, either in a causative manner or coincidentally, and at times they are difficult to distinguish. Depressive pseudodementia describes what once were thought to be apparent but not real cognitive deficits associated with severe major depression. More recent research indicates that major depression is a cause of reversible dementia; that is, major depression may cause true cognitive deficits that resolve with successful treatment of depression. The term depressive pseudodementia should be replaced by the more precise term, dementia syndrome of depression. If the clinician is uncertain whether major depression is a part of a presentation that includes dementia, it is best to treat depression empirically, not only for the emotional well-being of the patient, but also because cognition may improve. The classic clue to the diagnosis of dementia syndrome of depression is the occurrence of many I dont know answers. Patients with pure dementia are likely to try their best or even to invent answers when memory is poor. But severely depressed patients have a motivational deficit and negative attitude. They may even emphasize and complain about thinking problems in a catastrophic way, whereas patients with Alzheimers disease are often poorly aware or in denial of cognitive difficulties. Rapid decline in functioning, inconsistency in the mental status exam, pervasively dysphoric mood, and suicidal ideation or expression of a wish to die are other important clues to major depression.

8. Describe the elements of the work-up for dementia.

There is no definitive work-up for dementia. The astute clinician takes a careful history, completes a careful examination, and orders further tests as indicated in each case. However, certain chronic confusional states are difficult or impossible to rule out by history and physical exam but common enough to warrant routine laboratory investigation. Others should be considered if the history, physical exam, or preliminary blood tests are suggestive.

Laborutory Tests fiw Work-up of Dementia

Routine Complete blood count Syphilis serology Chemistry panel Thyroid function tests Vitamin B 12 and folate Sedimentation rate Urinalysis

Supplementary Serum antibody for human immunodeficiency virus (HIV) Electroencephalogram Computed tomography scan of head Magnetic resonance imaging of head Heavy metals screen Lumbar puncture Antinuclear antibody test Pulse oximetry Serum amylase

9. Describe the most common cause of nontraumatic dementia in young people. HIV encephalopathy, also known as AIDS dementia complex (ADC), was first described in detail in 1986. Nearly all patients with AIDS develop HIV encephalopathy at some time during the course of the disease. In 20% of HIV-infected people, changes in mental status precede immunologic abnormalities, and in another 10% changes in mental status and immunologic abnormalities are recognized simultaneously. All persons with changes in mental status should be questioned about risk factors for HIV exposure; if any are present, serum testing should be performed.

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HIV encephalopathy is the most common but by no means the only neurologic manifestation of HIV infection. Although a wide variety of infections and neoplastic brain diseases may occur, the dementia syndrome progresses in a fairly predictable fashion. Forgetfulness, poor concentration, and slowing of thought are early symptoms. Apathy and social withdrawal often appear early and become progressively severe. Psychomotor retardation occurs. Memory impairment and disturbances of higher cortical functioning become apparent as the disease progresses. Delusions, hallucinations, and agitation may occur. The terminal state is usually characterized by quiet confusion.

10. Name the two behaviors of patients with dementia that most commonly lead to nursing home placement. The two behaviors of demented individuals most commonly leading to nursing home placement are insomnia and aggression. Caregivers often feel helpless to deal with these problems, which are distressingly frequent and extremely difficult to tolerate.

11. Discuss the management of insomnia in patients with dementia. Normal sleep patterns are disrupted in many patients with dementia. In addition to actual brain changes that can be demonstrated in sleep EEGs, changes in behavior and lifestyle may cause the demented patient to be awake and active at night. For example, disinterest and inability to participate in usual activities may lead to excessive napping during the day. Decreased sensory stimulation at night may foster hallucinations or confusion. Inability to recall structure and social norms may encourage arousal and activity at night. Increasing structure and activity during the day should be the first approach to insomnia. Senior centers and day programs for patients with dementia are excellent resources. Exercise, especially in the late afternoon, encourages appropriate desire for rest at night. Routine bedtime and rising, with structure and ritual as cues, also may be helpful. The caretaker should react calmly if the person arises at night. Offering guidance to the bathroom or a drink of water or milk and then leading the individual back to bed often allows a return to sleep. If such approaches are not enough, medication may be considered. Sedatives-hypnotics should be used sparingly in patients with dementia, because they may further impair memory, cause paradoxical excitement, and interfere with balance, leading to falls. All over-the-counter sleep aids, including Benadryl and Tylenol PM, should be avoided, because their anticholinergic properties frequently cause confusion in the demented patient. The sedating tricyclic antidepressants and antipsychotics are also highly anticholinergic and may cause significant orthostatic hypotension, increasing the risk of falls as well as cardiovascular complications. Sometimes a low dose of trazodone is effective. Trazodone is not sedating for all individuals but may be effective without the hazards of more traditional sleeping medications. A dose as low as 25 mg may be sufficient. Melatonin also has been found to be helpful for some.

12. How should aggression be approached in patients with dementia?

Aggression is a disturbing problem for caregivers and frequently brings the patient to the attention of a physician. The first response that comes to mind for many doctors and caregivers is sedating medication. However, medications involve significant risks and are usually only partially effective. It is best to attempt behavioral approaches first. Caregivers should be instructed to note the circumstances that commonly precede outbursts of aggression. Usually, there is a period of increasing agitation in response to something that the patient has difficulty in understanding or handling. Often the responses of caregivers increase rather than resolve the agitation. For example, caregivers may argue, raise their voice, or use physical force to make patients do something that they are resisting. It is much more effective to stop immediately whatever is happening when the patient begins to show warning signs of aggression and to initiate more soothing and relaxing activity. If possible, the patients underlying concern should be addressed. For example, if the patient is accusing someone of stealing, help the patient to find what has been lost. The Alzheimers Association, a national organization with chapters in many cities, is an invaluable asset for educating caregivers in how to deal with these and other difficult behaviors

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associated with dementia. The 36-hour Day, by Nancy Mace and Peter Rabins, is an excellent reference to recommend to caregivers.

13. If pharmacologictreatment of aggression or agitation becomes necessary, how should it be managed? First, consider the cause. Treating an underlying medical condition that is causing pain or discomfort may resolve the problem. The presence of depression, delirium, or psychosis will guide your choice of agent. Controlled studies have shown a modest improvement in overall agitation in dementia with the use of conventional antipsychotic agents. The high-potency agents are preferred to the more anticholinergic ones. For most, a dosage equivalent to haloperidol 2 mg/d is necessary. However, many elderly develop debilitating extrapyramidal symptoms, particularly parkinsonism, with this treatment. The second-generation antipsychotics risperidone and olanzapine are being used with success, and fewer side effects. Even so, it is wise to reserve the antipsychotic agents for those with psychotic symptoms. Benzodiazepines may be used carefully, in small doses, to treat agitation and aggression. Lorazepam or oxazepam are preferred; both are metabolized at the same rate regardless of patient age, and therefore do not accumulate in the aging body. Caution is needed because more than a small dose often causes postural instability, leading to falls. Consider starting a delayed-action agent such as buspirone or an antidepressant, with the intent to taper the benzodiazepine as soon as possible. Divalproex was the third most commonly used medication to treat these symptoms in the elderly in 1997, according to a medication usage study,9 following haloperidol and risperidone. Although controlled data on its effectiveness are lagging behind, the widespread use of this medication for this purpose most likely represents clinician experience, as well as its safety and relative lack of side effects. Effective dosages may be lower than those used to treat epilepsy or mania. Buspirone also is widely used, and is most recommended for the long-term treatment of anxiety. It generally is well tolerated and safe. Trazodone is helpful in many cases, as well. 14. Are there any medications that can treat Alzheimers disease directly, rather than just the behavioral symptoms? At present, there is no way to cure or stop the progression of Alzheimers disease. However, there are agents that can enhance cognition in some, in the early to middle stages of the disease, and slow the rate of decline. The anticholinesterase inhibitors were developed in response to the finding of cholinergic cell loss in the brains of Alzheimers disease patients. The first one available was tacrine. It has many side effects, including a fairly high rate of liver toxicity necessitating frequent blood monitoring, and a short half-life, requiring frequent dosing. Donepezil may be dosed once daily, and because it is more specific to brain tissue, the side effects are few. Use of these agents can produce measurable but quite modest improvement in cognition, and modest slowing of the decline. There is now sufficient data to recommend supplemental vitamin E for those with Alzheimers disease. Selegiline is about equally as effective but much more expensive. Many other substances are being studied but as yet data is insufficient to recommend them routinely.
BIBLIOGRAPHY
1. Alexopoulos GS, Silver JM, Kahn DA, et a1 (eds): Treatment of Agitation in Older Persons with Dementia. From The Expert Consensus Guideline Series. A Special Report of Postgraduate Medicine. April, 1998. 2. Cummings JL, Benson DF: Dementia: A Clinical Approach, 2nd ed. Boston, Butterworth-Heinemann, 1992. 3 . Eberling JL, Jagust WJ: Neuroimaging and the diagnosis of dementia. Psychiatr Ann 24:178-185, 1994. 4. Horowitz GR: What is a complete work-up for dementia? Clin Geriatr Med 4:163-180, 1988. 5. Kim E, Rovner BW: Depression in dementia. Psychiatr Ann 24:173-177, 1994. 6. Mace NL, Rabins PV: The 36-Hour Day, rev. ed. Baltimore, Johns Hopkins University Press, 1991. 7. Mortimer JA: The dementia of Parkinsons disease. Clin Geriatr Med 4:785-797, 1988. 8. Moss RJ, Miles SH: AIDS dementia. Clin Geriatr Med 4:889-895, 1988. 9. Schneider L: Clues to psychotropic prescribing practices in geriatric medicine. Primary Psychiatry 5:23-26, 1998. 10. Strub RL, Black Fw:Neurobehavioral Disorders: A Clinical Approach. Philadelphia, F.A. Davis, 1988, p 58. 11. Webster J: Recognition and treatment of dementing disorders in the elderly. Clinical Geriatrics 7 6 - 6 9 , 1999.