Evaluation and Treatment of Patients with Dementia Frank R. Freemon, MD Nashville, Tennessee
Dementia is a common problem in middle-aged and elderly adults. Thorough diagnostic evaluation uncovers a significant minority who are suffering from an underlying neuropsychiatric disease amenable to specific medical or surgical therapy. Even in those patients with brain atrophy of unknown cause the physician can offer counseling and environmental manipulation to maximize remaining intellectual capacity.
Introduction The syndrome of dementia is characterized by a progressive, chronic deterioration of intellectual function. The term dementia implies organic dysfunction of brain cells to produce poor memory, impaired judgment, decreased attention, and loss of arithmetic, reasoning, and problem-solving abilities. Some workers use the term presenile dementia to refer to any dementing process beginning before age 65; others restrict the term to a specific illness, Alzheimer's disease. Since the term presenile has different meanings for different people, and because the 65th birthday has no biological significance, this article does not use it.
Presented at the 81st meeting of the National Medical Association, Nashville, Tennessee, August 12, 1976. Supported by Veterans Administration research funds. From Neurology Service, Veterans' Administration Hospital, and Department of Neurology, Vanderbilt University, Nashville, Tennessee. Requests for reprints should be addressed to Frank R. Freemon, Neurology Service, Veterans' Administration Hospital, Nashville, Tenn 37203.
Clinical Presentation As in all organic mental diseases, the early clinical picture varies greatly from one patient to another depending partly on pre-morbid personality and partly upon life-style. Individuals with a highly-demanding technical career often begin to make minor errors more often than they did before. They may notice this themselves, or their colleagues may be the first to observe this tendency. In individuals doing more routine work, the initial symptomatology may involve home activities. Often a relative who visits only occasionally sees a change in the patient-tobe, a change of such insidious onset it has escaped notice by closer relatives. Sometimes the type of change cannot be specified in detail, even in retrospect; the patient just is "not himself." Sometimes the initial symptom involves memory. The patient may have the same memory foibles he has always had, but now they are more frequent and more noticeable. He must look up telephone numbers twice or ask his spouse where things are. Several male patients have told me stories similar to the following: The
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 69, NO. 5, 1977
first time anything serious was suspected was when his wife sent him on an errand. The patient had to call home to confess he had forgotten where he was supposed to go or what he was to buy. As the disease progresses most patients develop some degree of depression, reacting to their own recognition of their decreasing intellectual powers. As the mental deterioration worsens, neurological abnormalities sometimes appear. These findings, generally referred to as "frontal lobe signs," include the palmomental and snout reflexes. Not all patients develop these signs even with advanced dementia and they are sometimes present in normal subjects so these findings must be interpreted in the context of the full clinical picture. Some physicians have attempted to differentiate two presentations of the dementia syndrome.' In one of these dementia subtypes the major clinical symptom is apathy and withdrawal. The patient appears to be ill, or at least "not himself," to a superficial observer. He does not speak unless spoken to. He often shows a drab and lifeless facial expression, punctuated by episodes of uncontrollable sobbing. The frontal lobe signs may be present on neurological examination. In the other type the major symptom is memory loss. The patient appears normal to a nonmedical observer. He has what has been termed a "cocktail party" personality; superficial discussion of general topics is normal but deeper investigation reveals enormous memory holes. The patient may engage in a lively discussion of the lack of morals or intelligence of governmental leaders but, when he is questioned on specifics, it becomes apparent he does not even know the name of the President or his predecessor. Some experts have attempted to 307
Table 1. Treatable Causes of Dementia
Normal pressure hydrocephalus
(Hakim-Adams syndrome) Chronic drug intoxication
Mass lesions frontal lobe tumors subdural hematomas Fungal meningitis
Hypothyroidism Vitamin deficiency vitamin B12 malabsorption folic acid deficiency pel lagra
Teritary syphilis Liver or renal failure
Temporal lobe or psychomotor seizure status
Table 2. Protocol for Work-up of a Dementia Patient
Site: Nashville Veterans Hospital Dates: July 1, 1973 to June 30, 1975 Patients: 60 males, average age 66.2
History: From relatives as well as the patient, with special attention to alcohol and drug use. Examination: Physical, neurological, and mental status.
Blood tests: CBC, urinalysis, SMA-12 and 6, B 1 2, folate, barbiturate and bromide levels, VDRL and FTA tests, column T4.
Laboratory examinations: Skull x-ray, EEG, radioisotope brain scan, and
(general health permitting) pneumoencephalogram. Either with the PEG or a separate LP, CSF examination with india ink and CSF analysis for cells, protein, glucose, and VDRL.
308
differentiate the latter memory disorder, referred to as limbic or subcortical dementia, from the more global deficit of the previously described "frontal lobe" or cortical dementia. While extremes are easily differentiated, most patient's symptoms fall between the two. Some degenerative disorders, especially Alzheimer's disease, show a progression; the early case is more "limbic," the deteriorated patient more "cortical."
Syndrome Diagnosis One must distinguish the symptomatic differences in the patient suffering from dementia from those in patients suffering other conditions that masquerade as a progressive decline in intellectual function. Many to most dementia patients suffer a reactive depression usually early in the course of the development of the dementia. Because of this, one cannot really separate depression from dementia but rather one evaluates depressed patients to see whether there is any underlying component of dementia. Midlife depression is a common problem and these patients commonly complain of memory difficulty.2 Often there is extensive psychomotor retardation, and examination of these patients can be very tiring. Usually, however, great patience on the part of the examiner will disclose slow answers normal in content. The patient with depression due to dementia does not usually have semi-hallucinatory symptomatology such as feeling that his internal organs are rotten. Neuropsychological testing of the depressed patient is often valuable in determining if any underlying dementia is present. In the final analysis it is sometimes necessary to treat depression by pharmacological means and await a clinical response. Often the depression will clear completely and the patient will return to normal; in other patients the depression will lift and dementia will be clearly seen. While most patients with aphasia have associated symptoms of left hemispheral dysfunction such as right hemiparesis or right homonymous hemianopsia, there are some patients who have parietal lesions, usually on the left, which produce a predominately sensory or Wernicke aphasia with few other signs. These individuals have very great difficulty with reading and in understanding speech. They
have difficulty following the examiner's questions and superficial evaluation may lead the physician to suspect dementia. Actually a more detailed mental status examination usually quickly differentiates a patient who has clear aphasic symptomatology (such as inability to name common objects) from a demented patient. In addition, the aphasia is usually due to a vascular problem, often an embolus, and is of sudden onset - while the dementia syndrome is of insidious progression.
Etiologic Diagnosis Once it is determined that a patient suffers from the dementia syndrome the physician looks for the underlying cause. Many degenerative brain diseases produce dementia. These disorders, diagnosed only by histological examination of brain tissue, are designated by a series of eponyms: Alzheimer, Binswanger, Pick, Kraeplin, Be hc e t, Hallevorden-Spatz. Other, poorly-defined histopathologic changes have been associated with dementia.3 Academic neurologists often argue about the best eponym to apply to a given clinical picture but this diagnosis is often of little practical value. In my opinion, the diagnostic effort in etiologic diagnosis should be di-, rected toward the identification of treatable diseases. A large number of medical and neurological illnesses at least partially reversible by appropriate specific therapy can present as dementia as tabulated in Table 1. Several other disorders amenable to specific therapy include mental changes as part of their symptomatology but the clinical picture of dementia does not predominate. Wilson's disease can present as personality change or even psychosis; Kayser-Fleischer rings will be present at that point in the development of the disease. Similarly porphyria can produce striking mental changes such as vivid hallucinations but is never really associated with the full dementia syndrome. Cushing's disease and syndrome can produce mental changes which might, in some cases, resemble dementia; these patients are always hypertensive at this point in disease development and usually have the typical Cushingoid body habitus. Though most patients with Parkinson's disease are mentally normal, some have an associated dementia; for all
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION,
VOL. 69, NO. 5, 1977
these patients, treatment is aimed at countering the motor manifestations of Parkinsonism and no specific therapy counters the cognitive deficits. Let us consider the entities of Table 1 individually. Normal pressure hydrocephalus (NPH) is an incompletely described syndrome related to abnormal cerebrospinal fluid (CSF) absorption.4 The clinical picture is apathetic dementia, wide-based gait, and, in extreme cases, urinary incontinence without embarrassment. Most patients have associated frontal lobe signs such as masked facies, emotional blunting, and grasp, snout, and palmomental reflexes. The pneumographic picture is enlarged ventricles - especially the frontal horns of the lateral ventricles with failure of the injected air to flow over the cerebral convexities. Computed tomography has largely replaced pneumoencephalography for the evaluation of NPH, since air injected into the subarachnoid space causes such severe headache.5 Most of the entities of Table 1 are self-explanatory. Chronic drug intoxication causing dementia includes specific single entities such as bromides or phenytoin6 but more common are mixtures of many depressant psychoactive agents. The heavy prolonged use of marihuana or hashish as a cause of dementia remains controversial. A comment about tertiary syphilis may be in order. While there is little doubt that significant permanent structural damage has occurred in every case of general paresis, evidence exists that living treponemal organisms continue to multiply within the human brain, and it seems reasonable to expect some degree of mental improvement after high dosage penicillin therapy. The last diagnosis on Table 1 represents a somewhat rare problem but one worth considering in every patient whose dementia shows some degree of fluctuation. In some patients momentary confusion is the major clinical manifestation of a temporal lobe or psychomotor seizure (newly renamed complex partial seizure). In rare cases these seizures come so frequently as to be almost continuous and masquerade as chronic intellectual deterioration.
Prolonged
electroencephalogram
(EEG) recordings reveal continuous seizure discharge, better anti-convulsant management decreases seizure frequency, and mental status improves.
TREATABLE CAUSES OF DEMENTIA SERIES:
CASES:
TREATABLE/TOTAL NASHVILLE LONDON
7
NUMBER OF CASES
18/6
6_ 5 4
3-
2-
DRUG N. P H. MASS OTHER TOXICITY LESION Figure 1. A comparison of the case findings from two consecutive patient series.
DEPRESSION
Consecutive Patient Series There are two reports in the literature concerning evaluation of dementia. A neurological specialty hospital in London analyzed the records from 106 consecutive cases referred by area neurologists and psychiatrists with the prospective diagnosis of dementia.7 Twelve of these patients were determined upon admission to have a completely different problem which did not really represent the dementia syndrome. Nineteen of the remainder were determined to have a treatable medical illness which produced progressive intellectual deterioration or a treatable condition which simulated dementia. The most frequently encountered condition was depression. A veterans' hospital in Nashville, Tennessee, evaluated consecutive dementia patients brought to the hospital by relatives or referred by local general practitioners.8 Sixty patients, all male military veterans, were encountered in a two-year period. They underwent the diagnostic protocol of Table 2. Seven patients had the clinical and pneumoencephalographic criteria for normal pressure hydrocephalus, but only one of these showed a marked response to shunting. Five patients had mental deterioration associated with the use of multiple analgesics, hypnotics, and anticonvulsants. In all cases the multiple drugs were prescribed by different practitioners
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 69, NO. 5, 1977
unaware of the treatment of the other practitioners. Mental function returned completely to normal in every case with drug discontinuance or dosage adjustment. Two cases of subdural hematoma were seen and one case each of tertiary syphilis, hepatic encephalopathy, and hypothyroidism. Figure 1 compares these series (from two different referral groups in two different nations). In the British series, 20 percent of the patients had an underlying disease potentially responsive to specific therapy. In the American series 30 percent had a treatable illness, if one allows the diagnosis of NPH by clinical and radiographic criteria alone, or 20 percent if one requires response to shunting for the diagnosis. In either case, both these series indicate that many patients with progressive intellectual deterioration could benefit from thorough diagnostic analysis.
Treatment Treatment of the dementia syndrome should exemplify the ancient principle of medicine, "to heal sometimes but to comfort always." A certain percentage, probably about a fifth to a fourth of cases will be suffering from an underlying medical or neuro309
logical illness and will show at least a partial response to specific treatment. One must not downgrade the nonspecific or "comforting" treatment which includes counseling to the patient and his family, providing the best possible environment for the patient and treating complications.9 Counseling to the patient should probably be more optimistic than that given to the family. Certainly the patient needs his spouse or another trusted family member to take over financial affairs, although the family should try to avoid placing the patient in the "sick role," sitting in a wheelchair while all his needs are met. Rather, he should continue to be as useful and busy as his mental capacity allows. Most demented patients do best in the environment most familiar to them. Just as their memory of people and events from years gone by are better than of more recent things, so also the strongest recollections are for objects and rooms with which the patient became familiar many years
310
ago. If the patient has lived in one home for many years he will probably do best remaining there. Some patients deteriorate to such a degree that admission to a professional nursing facility becomes absolutely necessary. One can usually predict, however, that further deterioration will follow immediately upon that admission as the patient copes with a new environment. An exception to the rule of nursing home deterioration is the demented housewife who cannot give up the exhausting demands of running a home to her relatives as long as she remains there but improves in the more regulated environment of the nursing facility. Of course, the physician treats complications as they arise. Depression is frequent. Some people become particularly disoriented at night, but night lights and familiar environmental cues help more than sleeping pills. Many clinicians feel that the brain of the demented patient is particularly sensitive to the mildest metabolic derangement and that the dementia worsens
with urinary or respiratory infection and improves following eradication of such problems.
Literature Cited 1. Albert ML, Feldman RG, Willis AL: The subcortical dementia of progressive supranuclear palsy. J Neurol Neurosurg Psychiatry 37:121-130, 1974 2. Kahn RL, Zarit SH, Hilbert NM, et al: Memory complaint and impairment in the aged. Arch Gen Psychiatry 32:1569-1573,
1975 3. Hughes CP, Myers FK, Smith K, et al: Nosologic problems in dementia. Neurology 23:344-351, 1973 4. Messert B, Wannamaker BB: Reappraisal of the adult occult hydrocephalus syndrome. Neurology 24:224-231, 1974 5. Fox JH, Topel JL, Huckman MS: Use of computerized tomography in senile dementia. J. Neurol Neurosurg Psychiatry 38:948-953, 1975 6. Vallarta JM, Bell DB, Reichert A: Progressive encephalopathy due to chronic hydantoin intoxication. Am J Dis Child 128:27-34, 1974 7. Marsden CD, Harrison MJG: Outcome of investigation of patients with presenile dementia. Br Med J 2:249-252, 1972 8. Freemon FR: Evaluation of patients with progressive intellectual deterioration. Arch Neurol 33:658-659, 1976 9. Snyder BD, Harris S: Treatable aspects of the dementia syndrome. J Am Geriatr Soc 24:179-184, 1976
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL.
69,
NO. 5, 1977
Dementia is a common problem in middle-aged and elderly adults. Thorough diagnostic evaluation uncovers a significant minority who are suffering from an underlying neuropsychiatric disease amenable to specific medical or surgical therapy. Even in those patients with brain atrophy of unknown cause the physician can offer counseling and environmental manipulation to maximize remaining intellectual capacity.
Introduction The syndrome of dementia is characterized by a progressive, chronic deterioration of intellectual function. The term dementia implies organic dysfunction of brain cells to produce poor memory, impaired judgment, decreased attention, and loss of arithmetic, reasoning, and problem-solving abilities. Some workers use the term presenile dementia to refer to any dementing process beginning before age 65; others restrict the term to a specific illness, Alzheimer's disease. Since the term presenile has different meanings for different people, and because the 65th birthday has no biological significance, this article does not use it.
Presented at the 81st meeting of the National Medical Association, Nashville, Tennessee, August 12, 1976. Supported by Veterans Administration research funds. From Neurology Service, Veterans' Administration Hospital, and Department of Neurology, Vanderbilt University, Nashville, Tennessee. Requests for reprints should be addressed to Frank R. Freemon, Neurology Service, Veterans' Administration Hospital, Nashville, Tenn 37203.
Clinical Presentation As in all organic mental diseases, the early clinical picture varies greatly from one patient to another depending partly on pre-morbid personality and partly upon life-style. Individuals with a highly-demanding technical career often begin to make minor errors more often than they did before. They may notice this themselves, or their colleagues may be the first to observe this tendency. In individuals doing more routine work, the initial symptomatology may involve home activities. Often a relative who visits only occasionally sees a change in the patient-tobe, a change of such insidious onset it has escaped notice by closer relatives. Sometimes the type of change cannot be specified in detail, even in retrospect; the patient just is "not himself." Sometimes the initial symptom involves memory. The patient may have the same memory foibles he has always had, but now they are more frequent and more noticeable. He must look up telephone numbers twice or ask his spouse where things are. Several male patients have told me stories similar to the following: The
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 69, NO. 5, 1977
first time anything serious was suspected was when his wife sent him on an errand. The patient had to call home to confess he had forgotten where he was supposed to go or what he was to buy. As the disease progresses most patients develop some degree of depression, reacting to their own recognition of their decreasing intellectual powers. As the mental deterioration worsens, neurological abnormalities sometimes appear. These findings, generally referred to as "frontal lobe signs," include the palmomental and snout reflexes. Not all patients develop these signs even with advanced dementia and they are sometimes present in normal subjects so these findings must be interpreted in the context of the full clinical picture. Some physicians have attempted to differentiate two presentations of the dementia syndrome.' In one of these dementia subtypes the major clinical symptom is apathy and withdrawal. The patient appears to be ill, or at least "not himself," to a superficial observer. He does not speak unless spoken to. He often shows a drab and lifeless facial expression, punctuated by episodes of uncontrollable sobbing. The frontal lobe signs may be present on neurological examination. In the other type the major symptom is memory loss. The patient appears normal to a nonmedical observer. He has what has been termed a "cocktail party" personality; superficial discussion of general topics is normal but deeper investigation reveals enormous memory holes. The patient may engage in a lively discussion of the lack of morals or intelligence of governmental leaders but, when he is questioned on specifics, it becomes apparent he does not even know the name of the President or his predecessor. Some experts have attempted to 307
Table 1. Treatable Causes of Dementia
Normal pressure hydrocephalus
(Hakim-Adams syndrome) Chronic drug intoxication
Mass lesions frontal lobe tumors subdural hematomas Fungal meningitis
Hypothyroidism Vitamin deficiency vitamin B12 malabsorption folic acid deficiency pel lagra
Teritary syphilis Liver or renal failure
Temporal lobe or psychomotor seizure status
Table 2. Protocol for Work-up of a Dementia Patient
Site: Nashville Veterans Hospital Dates: July 1, 1973 to June 30, 1975 Patients: 60 males, average age 66.2
History: From relatives as well as the patient, with special attention to alcohol and drug use. Examination: Physical, neurological, and mental status.
Blood tests: CBC, urinalysis, SMA-12 and 6, B 1 2, folate, barbiturate and bromide levels, VDRL and FTA tests, column T4.
Laboratory examinations: Skull x-ray, EEG, radioisotope brain scan, and
(general health permitting) pneumoencephalogram. Either with the PEG or a separate LP, CSF examination with india ink and CSF analysis for cells, protein, glucose, and VDRL.
308
differentiate the latter memory disorder, referred to as limbic or subcortical dementia, from the more global deficit of the previously described "frontal lobe" or cortical dementia. While extremes are easily differentiated, most patient's symptoms fall between the two. Some degenerative disorders, especially Alzheimer's disease, show a progression; the early case is more "limbic," the deteriorated patient more "cortical."
Syndrome Diagnosis One must distinguish the symptomatic differences in the patient suffering from dementia from those in patients suffering other conditions that masquerade as a progressive decline in intellectual function. Many to most dementia patients suffer a reactive depression usually early in the course of the development of the dementia. Because of this, one cannot really separate depression from dementia but rather one evaluates depressed patients to see whether there is any underlying component of dementia. Midlife depression is a common problem and these patients commonly complain of memory difficulty.2 Often there is extensive psychomotor retardation, and examination of these patients can be very tiring. Usually, however, great patience on the part of the examiner will disclose slow answers normal in content. The patient with depression due to dementia does not usually have semi-hallucinatory symptomatology such as feeling that his internal organs are rotten. Neuropsychological testing of the depressed patient is often valuable in determining if any underlying dementia is present. In the final analysis it is sometimes necessary to treat depression by pharmacological means and await a clinical response. Often the depression will clear completely and the patient will return to normal; in other patients the depression will lift and dementia will be clearly seen. While most patients with aphasia have associated symptoms of left hemispheral dysfunction such as right hemiparesis or right homonymous hemianopsia, there are some patients who have parietal lesions, usually on the left, which produce a predominately sensory or Wernicke aphasia with few other signs. These individuals have very great difficulty with reading and in understanding speech. They
have difficulty following the examiner's questions and superficial evaluation may lead the physician to suspect dementia. Actually a more detailed mental status examination usually quickly differentiates a patient who has clear aphasic symptomatology (such as inability to name common objects) from a demented patient. In addition, the aphasia is usually due to a vascular problem, often an embolus, and is of sudden onset - while the dementia syndrome is of insidious progression.
Etiologic Diagnosis Once it is determined that a patient suffers from the dementia syndrome the physician looks for the underlying cause. Many degenerative brain diseases produce dementia. These disorders, diagnosed only by histological examination of brain tissue, are designated by a series of eponyms: Alzheimer, Binswanger, Pick, Kraeplin, Be hc e t, Hallevorden-Spatz. Other, poorly-defined histopathologic changes have been associated with dementia.3 Academic neurologists often argue about the best eponym to apply to a given clinical picture but this diagnosis is often of little practical value. In my opinion, the diagnostic effort in etiologic diagnosis should be di-, rected toward the identification of treatable diseases. A large number of medical and neurological illnesses at least partially reversible by appropriate specific therapy can present as dementia as tabulated in Table 1. Several other disorders amenable to specific therapy include mental changes as part of their symptomatology but the clinical picture of dementia does not predominate. Wilson's disease can present as personality change or even psychosis; Kayser-Fleischer rings will be present at that point in the development of the disease. Similarly porphyria can produce striking mental changes such as vivid hallucinations but is never really associated with the full dementia syndrome. Cushing's disease and syndrome can produce mental changes which might, in some cases, resemble dementia; these patients are always hypertensive at this point in disease development and usually have the typical Cushingoid body habitus. Though most patients with Parkinson's disease are mentally normal, some have an associated dementia; for all
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION,
VOL. 69, NO. 5, 1977
these patients, treatment is aimed at countering the motor manifestations of Parkinsonism and no specific therapy counters the cognitive deficits. Let us consider the entities of Table 1 individually. Normal pressure hydrocephalus (NPH) is an incompletely described syndrome related to abnormal cerebrospinal fluid (CSF) absorption.4 The clinical picture is apathetic dementia, wide-based gait, and, in extreme cases, urinary incontinence without embarrassment. Most patients have associated frontal lobe signs such as masked facies, emotional blunting, and grasp, snout, and palmomental reflexes. The pneumographic picture is enlarged ventricles - especially the frontal horns of the lateral ventricles with failure of the injected air to flow over the cerebral convexities. Computed tomography has largely replaced pneumoencephalography for the evaluation of NPH, since air injected into the subarachnoid space causes such severe headache.5 Most of the entities of Table 1 are self-explanatory. Chronic drug intoxication causing dementia includes specific single entities such as bromides or phenytoin6 but more common are mixtures of many depressant psychoactive agents. The heavy prolonged use of marihuana or hashish as a cause of dementia remains controversial. A comment about tertiary syphilis may be in order. While there is little doubt that significant permanent structural damage has occurred in every case of general paresis, evidence exists that living treponemal organisms continue to multiply within the human brain, and it seems reasonable to expect some degree of mental improvement after high dosage penicillin therapy. The last diagnosis on Table 1 represents a somewhat rare problem but one worth considering in every patient whose dementia shows some degree of fluctuation. In some patients momentary confusion is the major clinical manifestation of a temporal lobe or psychomotor seizure (newly renamed complex partial seizure). In rare cases these seizures come so frequently as to be almost continuous and masquerade as chronic intellectual deterioration.
Prolonged
electroencephalogram
(EEG) recordings reveal continuous seizure discharge, better anti-convulsant management decreases seizure frequency, and mental status improves.
TREATABLE CAUSES OF DEMENTIA SERIES:
CASES:
TREATABLE/TOTAL NASHVILLE LONDON
7
NUMBER OF CASES
18/6
6_ 5 4
3-
2-
DRUG N. P H. MASS OTHER TOXICITY LESION Figure 1. A comparison of the case findings from two consecutive patient series.
DEPRESSION
Consecutive Patient Series There are two reports in the literature concerning evaluation of dementia. A neurological specialty hospital in London analyzed the records from 106 consecutive cases referred by area neurologists and psychiatrists with the prospective diagnosis of dementia.7 Twelve of these patients were determined upon admission to have a completely different problem which did not really represent the dementia syndrome. Nineteen of the remainder were determined to have a treatable medical illness which produced progressive intellectual deterioration or a treatable condition which simulated dementia. The most frequently encountered condition was depression. A veterans' hospital in Nashville, Tennessee, evaluated consecutive dementia patients brought to the hospital by relatives or referred by local general practitioners.8 Sixty patients, all male military veterans, were encountered in a two-year period. They underwent the diagnostic protocol of Table 2. Seven patients had the clinical and pneumoencephalographic criteria for normal pressure hydrocephalus, but only one of these showed a marked response to shunting. Five patients had mental deterioration associated with the use of multiple analgesics, hypnotics, and anticonvulsants. In all cases the multiple drugs were prescribed by different practitioners
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL. 69, NO. 5, 1977
unaware of the treatment of the other practitioners. Mental function returned completely to normal in every case with drug discontinuance or dosage adjustment. Two cases of subdural hematoma were seen and one case each of tertiary syphilis, hepatic encephalopathy, and hypothyroidism. Figure 1 compares these series (from two different referral groups in two different nations). In the British series, 20 percent of the patients had an underlying disease potentially responsive to specific therapy. In the American series 30 percent had a treatable illness, if one allows the diagnosis of NPH by clinical and radiographic criteria alone, or 20 percent if one requires response to shunting for the diagnosis. In either case, both these series indicate that many patients with progressive intellectual deterioration could benefit from thorough diagnostic analysis.
Treatment Treatment of the dementia syndrome should exemplify the ancient principle of medicine, "to heal sometimes but to comfort always." A certain percentage, probably about a fifth to a fourth of cases will be suffering from an underlying medical or neuro309
logical illness and will show at least a partial response to specific treatment. One must not downgrade the nonspecific or "comforting" treatment which includes counseling to the patient and his family, providing the best possible environment for the patient and treating complications.9 Counseling to the patient should probably be more optimistic than that given to the family. Certainly the patient needs his spouse or another trusted family member to take over financial affairs, although the family should try to avoid placing the patient in the "sick role," sitting in a wheelchair while all his needs are met. Rather, he should continue to be as useful and busy as his mental capacity allows. Most demented patients do best in the environment most familiar to them. Just as their memory of people and events from years gone by are better than of more recent things, so also the strongest recollections are for objects and rooms with which the patient became familiar many years
310
ago. If the patient has lived in one home for many years he will probably do best remaining there. Some patients deteriorate to such a degree that admission to a professional nursing facility becomes absolutely necessary. One can usually predict, however, that further deterioration will follow immediately upon that admission as the patient copes with a new environment. An exception to the rule of nursing home deterioration is the demented housewife who cannot give up the exhausting demands of running a home to her relatives as long as she remains there but improves in the more regulated environment of the nursing facility. Of course, the physician treats complications as they arise. Depression is frequent. Some people become particularly disoriented at night, but night lights and familiar environmental cues help more than sleeping pills. Many clinicians feel that the brain of the demented patient is particularly sensitive to the mildest metabolic derangement and that the dementia worsens
with urinary or respiratory infection and improves following eradication of such problems.
Literature Cited 1. Albert ML, Feldman RG, Willis AL: The subcortical dementia of progressive supranuclear palsy. J Neurol Neurosurg Psychiatry 37:121-130, 1974 2. Kahn RL, Zarit SH, Hilbert NM, et al: Memory complaint and impairment in the aged. Arch Gen Psychiatry 32:1569-1573,
1975 3. Hughes CP, Myers FK, Smith K, et al: Nosologic problems in dementia. Neurology 23:344-351, 1973 4. Messert B, Wannamaker BB: Reappraisal of the adult occult hydrocephalus syndrome. Neurology 24:224-231, 1974 5. Fox JH, Topel JL, Huckman MS: Use of computerized tomography in senile dementia. J. Neurol Neurosurg Psychiatry 38:948-953, 1975 6. Vallarta JM, Bell DB, Reichert A: Progressive encephalopathy due to chronic hydantoin intoxication. Am J Dis Child 128:27-34, 1974 7. Marsden CD, Harrison MJG: Outcome of investigation of patients with presenile dementia. Br Med J 2:249-252, 1972 8. Freemon FR: Evaluation of patients with progressive intellectual deterioration. Arch Neurol 33:658-659, 1976 9. Snyder BD, Harris S: Treatable aspects of the dementia syndrome. J Am Geriatr Soc 24:179-184, 1976
JOURNAL OF THE NATIONAL MEDICAL ASSOCIATION, VOL.
69,
NO. 5, 1977
