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Elderly, community residing subjects (N = 106; mean age = 70.6 +/- 6.02 years) with cognitive functioning consistent with normal aging or dementia of the Alzheimer's type (DAT), were followed over a 3.6 year mean interval (range = 2.78 to 5.12 years). All subjects were assessed at baseline on the Global Deterioration Scale (GDS), a global clinical instrument reflecting the continuum of cognitive dysfunction from normal aging to severe DAT. At follow-up subjects were reassessed with respect to mortality, institutionalization and clinical change, defined as at least a two-point change on the 7-point GDS. Our results suggest that patients at deterioration levels GDS greater than or equal to 4, are more likely to show negative outcomes, specifically, institutionalization (Ps less than .001), death (Ps less than .01), or, for the community residing remainder, clinical deterioration (Ps less than .05), than subjects from less impaired (GDS = 2 or GDS = 3) subject groups. Seventy-six per cent of subjects at deterioration levels four or greater (N = 34) had negative outcomes at follow-up, whereas ninety percent of subjects with deterioration levels less than four (N = 72) did not.

A 40-year-old male patient with progressive dementia presented adversive seizures, and CT scan showed an enlarging focal mass lesion in the right cerebral hemisphere. Cerebrospinal fluid examination and brain biopsy confirmed the diagnosis of neurosyphilis. After a course of penicillin therapy there was disappearance of the cerebral mass lesion and the CT scan showed focal atrophy in the right cerebral hemisphere. This case suggests that Lissauer form of paretic neurosyphilis may present as a focal mass lesion. Registro de caso de paciente do sexo masculino com 40 anos de idade em que a tomografia computadorizada revelou a presença de processo expansivo no hemisfério cerebral direito. Os exames do líqüido cefalorraqueano e biópsia cerebral evidenciaram as características de neurossífilis. Após penicilinoterapia foi constatada atrofia focal no hemisfério cerebral direito. Este caso sugere que a forma de Lissauer da paralisia geral progressiva pode manifestar-se como processo expansivo cerebral.

7 Creutzfeldt-Jakob disease patients from western India are described. Of these, 5 patients came from greater Bombay, all within a radius of 20 km. These patients, however, came from different social backgrounds and there was no possibility of social contact. 2 of the 7 patients were vegetarians. There was no clue in any of these patients as to how the disease was acquired. All 7 patients had classic clinical manifestations with progressive dementia and myoclonic jerks. Electroencephalographic studies were abnormal in all 7 patients and, in 4 of these, classic sharp-wave complexes, recurring at intervals of about 1/s, were recorded. In 4 patients, autopsy confirmation was available with pathologic findings showing neuronal loss, spongiform change, astrocytic proliferation and absence of inflammatory changes.

Female patient, aged 63 died after 5 years of progressive dementia. Autopsy revealed multiple sclerosis, diffuse lymphoplasmocytic encephalitis, and Alzheimer's disease. Possible pathogenetic connections between these disease entities are discussed.

Presentation of Case A 15-year-old boy was admitted to the hospital because of progressive dementia. The patient was the first child of a 25-year-old mother, who had an uneventful pregnancy, labor, and delivery. His developmental milestones were normal. He earned As and Bs in school until the age of 11 years, when his performance began to deteriorate, and he complained of not feeling well enough to participate in sports. He barely passed the eighth grade. The ninth grade had to be repeated, and a tutor was obtained for him. He became progressively withdrawn, discontinued most activities, lost all his friends, . . .

Renal failure may be complicated by the development of encephalopathy, epileptic seizures, or peripheral neuropathy. Treatment of renal disease by dialysis may produce a disequilibrium syndrome or, rarely, progressive dementia due to cerebral aluminum toxicity. Transplantation may be followed by stroke, infections characteristic of immunocompromized hosts, or tumors, either primary lymphomas or metastatic tumors from remote primaries. Early diagnosis and appropriate treatment have reduced the frequency and severity of these complications of renal failure and its treatment.

Since neuronal lesions produced by aluminum (Al) are morphologically similar to lesions produced by microtubule inhibitors, the effect of aluminum on microtubule integrity was investigated. Aluminum inhibited microtubule formation at concentrations as low as 0.1 mM. This approaches the aluminum concentrations found in brains of patients with several disorders which result in progressive dementia.

The following report presents an additional example of subactue diencephalic angioencephalopathy. The patient, a 68-year-old man, unexplicably withdrawn and asocial throughout his life, presented with an altered mental status of relatively recent onset. His illness was marked by progressive dementia and ended in death within 7 weeks. The neuropathologic findings were essentially identical to those described in the previous case in both their histology and topographic localization within the thalamus. The etiology, pathogenesis, and reason for the localization of this entity are unknown. Nevertheless, the lesion underscores the major role of the thalamus in cognitive function, emotional behavior, and awareness.

Clinical concepts of age‐associated memory impairment (AAMI) have inevitably been linked to distinctions between this entity and progressive dementia, particularly Alzheimer's disease (AD). Kral was perhaps the first modern clinician to attempt to delineate these distinctions. More recently, Folstein has suggested mental status boundaries between AAMI and progressive dementia, including AD. We have delineated clinically distinguishable stages of progressive cognitive impairment in AAMI and in AD. Present longitudinal investigations suggest clinical and mental status boundaries between these conditions. Cumulatively, these studies indicate that relatively precise clinical criteria for a diagnosis of AAMI and for a definition of boundaries between AAMI and AD can presently be formulated for the selection of appropriate study populations.

• We describe a 16-year-old boy who has a progressive dementia and seizures. On investigation, he was found to have a mitochondrial myopathy and elevated lactate levels in the blood and cerebrospinal fluid. His sister died at 18 years of age of a similar condition.

Presentation of Case A 72-year-old woman was admitted to the hospital because of progressive dementia and a gait disorder. She was well and of normal mental status until six months previously, when she fainted. On admission to another hospital examination disclosed severe facial contusions. The results of routine laboratory studies, an electroencephalogram (EEG) and a radionuclide brain scan were normal. Monitoring of the electrocardiogram for 24 hours disclosed no important cardiac arrhythmias. Diazepam was prescribed, and she was discharged. Three months before entry she complained of anosmia, which appeared to improve. Her husband observed that she had difficulty in ambulation, . . .

The role of physicians in the long-term management of patients with the primary diagnosis of progressive dementia was investigated by surveying the opinions of 57 physicians and 47 family members. Respondents were asked to evaluate the difficulty and helpfulness of various activities physicians perform in the diagnosis, treatment, and management of patients with dementia. Both physicians and families rated the diagnostic services provided by physicians very highly. Although families found physicians' explanations of the diagnosis and prognosis extremely helpful, physicians reported this as an area of difficulty. Physicians and families expressed considerable frustration with the limited medical treatments and interventions for the relief of symptoms associated with dementia. Despite the acknowledged sensitivity of physicians to the social-psychological consequences of dementia, physicians were found to be least helpful in addressing these issues, either directly or through referral to allied health and social services. This study outlines areas of physician education that need improvement and calls for development of an interdisciplinary network of services for the biopsychosocial management of dementia. J Am Geriatr Soc 33:383, 1985

The case is described of a 35-year-old housewife diagnosed as having membranous lipodystrophy (as described by Nasu et al. in 1970 and called lipomembranous polycystic osteodysplasia by Hakola in 1972). The main symptom of this patient was a slowly progressive dementia. Skeletal symptoms were not seen. The computerized tomogram of the brain showed calcification of bilateral basal ganglia and the plain roentgenograms of the bones revealed cystic radiolucent areas at the distal end of the bones of the patient's extremities. Histological examination of the curretted material from the right talus revealed fa 'membranocystic' pattern. The fatty tissue curetted from the cyst of the talus and the lysosomal enzymes of the white blood cells were biochemically normal. A possible relationship between this disease entity and connective disorders is considered.

A retrospective clinical analysis of 70 demented patients who were admitted with disturbed behaviour to an acute psychogeriatric assessment and treatment centre revealed that 34.4% of patients had a significant untreated physical illness, 8.5% of patients were suffering from drug-induced toxicity and 11.4% of patients had a superimposed psychiatric illness. In only 39% of patients was progressive dementia alone considered an adequate explanation for their behavioural disturbance. The condition of the majority of patients improved significantly and they returned to the community. This study suggests that all patients with dementia with disturbed behaviour should be assessed carefully. In the majority, a clear treatable cause can be found and a good response can be attained with appropriate management.

To evaluate a standardized mental status exam's ability to predict activities of daily living (ADLs), Mini Mental Status Exam (MMSE) scores and ADL scores were obtained from 59 patients with progressive dementias of widely varying severity but with no other psychiatric disorders or major medical problems. The MMSE scores explained only about one-third of the variance in both instrumental ADLs and physical ADLs in the whole sample, and the MMSE and ADLs were independent of one another in the less demented half of the sample. This suggests that cognitive losses and functional impairments are two distinct aspects of dementia severity, which must be assessed separately.

A 46-year-old female is described with prolonged, progressive dementia and a brain biopsy consistent with Creutzfeldt-Jakob disease (CJD). She had neither myoclonic jerks nor an electroencephalogram with periodic spikes and suppression. Five of her close relatives were also demented. The nosology of CJD was discussed in the light of this case in which histopathology was characteristic of spongiform encephalopathy but the clinical features were atypical. We concluded that it would be premature to expand the traditional diagnostic criteria to include such cases as having CJD but, at the same time, it would be prudent to handle tissue, linens and surgical instruments as if they were contaminated by the resistant agent of CJD.

Normal pressure hydrocephalus (NPH) is a surgically correctable syndrome of progressive dementia, gait abnormalities, and urinary incontinence resulting from an occult hydrocephalus in association with normal cerebrospinal fluid pressure. Occurring most frequently in midlife and often idiopathic in origin, the early course of the illness may be characterized by symptoms of apathy, inattentiveness, agitation, and poverty of thought which mimic a depressive illness and may delay the recognition and treatment of the underlying structural defect. A review of the literature reveals that this association of depressive symptomatology and NPH has received little attention in the psychiatric literature, and the authors describe a case of NPH which presented as a severe, agitated depression. Clinical findings which suggest the presence of NPH are discussed, and the need to include NPH in the differential diagnosis of depression in the presenium is emphasized. The authors believe such diagnostic vigilance is necessary if the needless deterioration of potentially salvageable individuals is to be prevented.

SINCE the original descriptions of A Pick (1892) and Alzheimer (1907), there have been a number of clinical and pathologic studies of the pre-senile dementias (1,2). Although the diagnostic value of pneumo-encephalography in these conditions is accepted without question, the published radiographs are few in number and their interpretations are somewhat confusing. As these clinical syndromes are relatively unfamiliar to American radiologists, we will preface the presentation of a case history with a brief summary of the pertinent clinical and pathologic characteristics of these diseases. In this patient the diagnosis of a pre-senile dementia was made on clinical data and was supported by pneumo-encephalographic study. However, autopsy was not obtained and pathologic confirmation of the diagnosis was not possible. Pick's Disease.—Pick's disease is a progressive dementia, occurring in women twice as often as in men, with its highest incidence in the sixth decade of life. Its cause is unknown. Heredodegenerat...

This report deals with the clinical and neuropathological findings in a case of Creutzfeldt-Jakob disease (subacute spongioform encephalopathy). A 60-year-old woman died after a 4 month illness characterized by a progressive dementia with pyramidal and extrapyramidal signs, myoclonic jerks and focal seizures in the left arm.

Summary Clinico-pathological descriptions were made on a case of Binswanger's disease, in whom the illness began with Parkinsonism and followed by progressive dementia. The authors compared the clinico-pathological findings with those of the cases reported in the literature.

A review of the literature will reveal the fact that post-apoplectic psychoses have received less consideration than the subject deserves, in fact not a great deal has been written about this symptom-complex. That a perverted mental state occurs after an apoplexy in a certain number of cases has always been recognized, but the textbooks on the whole have described this condition in a more or less perfunctory manner. The usual and best recognized form of mental change after an apoplexy consists of a simple moderate intellectual defect characterized by failing memory, emotionalism and defective will. Beside this, however, there may develop a progressive dementia, best described as dementia post-apoplectica. But more rarely there occurs a certain mental state which has features which are more or less characteristic of a post-apoplectic psychosis. It is to illustrate the last, that I venture to report two cases which recently have come under my observation in private practice and which I have had the opportunity of studying from the very onset of the mental symptoms, an event which is not often afforded the alienist who is more apt to be called to the care of such cases later in the course of the disease. The following cases are practically identical in their clinical features and seem to present a phase of post-apoplectic psychoses which is infrequently observed.

THE ASSOCIATION of retinal degeneration and neurologic disorders is well known. Prominent among these is cerebromacular degeneration, or amaurotic familial idiocy. The association of pigmentary degeneration of the retina with neurologic disorders is best known in the Laurence-Moon-Biedl syndrome, which includes mental retardation. Still other heredodegenerative diseases of the nervous system reported to be associated with pigmentary degeneration of the retina are Friedreich's ataxia, 1 progressive dementia, 2 the cerebellopyramidal syndrome 3 and spastic 4 and flaccid 5 paraplegias. In 1944, Barnard and Scholz 6 reported 4 cases of ophthalmoplegia with pigmentary degeneration of the retina and concluded that they all represented a single syndrome with a common etiologic factor. Walsh 7 concurred in this opinion and described 4 cases of a similar disorder. His case 224 appears to be the same as case 4 of Barnard and Scholz; thus a total of 7 such cases have been reported. In

THE MORPHOLOGICAL AND FUNCTIONAL changes which follow the removal of the thyroid glands have been well studied both in man and in animal experiments. But severe though the mental and nervous changes are following thyroid abla-tion in man, the histopathologic changes are indefinite and do not explain at all the progressive dementia in cretinism and myxedema (1). Pighini (2) and later Parhon and Werner (3) tried to find the chemical changes of the brain which might be correlated with the functional changes. Pighini analyzed the different lipid fractions in two pup-pies and two adult dogs which survived atotal ablation of both thyroids and parathy roids for 50 days. He found a mild decrease of3 and 2‰ respectively in the amount of total lipoids. Parhon and Werner investigated the phosphorus content of thyroidec-tomised dogs which survived from 2 to 14 days.

A pair of sibs is reported with severe growth retardation since infancy, lack of subcutaneous fat, aged appearance, severe mental retardation with possibly a progressive dementia and evidence of peripheral polyneuropathy. The correct labelling of this disorder is discussed in relation to cases previously reported under the heading of Donohue’s syndrome or leprechaunism or under the heading of Cockayne’s syndrome.

✓ Adult patients suffering from progressive dementia and showing enlargement of cerebral ventricles without elevation of cerebrospinal fluid (CSF) pressure received intrathecal injections of radioisotopes. By plotting subsequent blood radioactivity at intervals, two types of curves were obtained. Group A showed a rapid rise of activity followed by a high plateau; Group B showed a slow but continuous rise of activity. In patients in whom intrathecal radioisotope scans were also performed, the concentration of radioactivity was mainly sagittal in Group A, whereas ventricular radioactivity persisted in Group B. It is proposed that the diminished rate of transfer in Group B is due to either diminished rate of absorption or of formation of CSF, or both. The estimation of the rate of transfer of isotope from CSF to plasma provides a supplementary aid in the diagnosis of normal-pressure hydrocephalus.

Intraganglionic inclusion bodies of presumably non-viral ongm have been associated with a range of defined neurologic disturbances, including myoclonus epilepsy (1), Pick's disease (2), and Parkinson's disease (3). In each of these entities the inclusions present a pattern of characteristics (histochemical and histologic properties, topographical distribution and size) which permits a degree of diagnostic differentiation. In the Parkinsonian states, the inclusion is typically intracytoplasmic, and most frequently encountered in the pigmented neurons of the mesencephalic and rhombencephalic nuclei (4). Two elderly patients with progressive dementia associated with quadriparesis in flexion, and without any Parkinsonian stigmata, have been studied, demonstrating at autopsy widely disseminated intracytoplasmic ganglionic inclusions morphologically and histochemically indistinguishable from those initially described by Lewy in Parkinson's disease (paralysis agitans).

✓ The authors describe a patient who presented with progressive dementia and hemiparesis. A neurofibroma was demonstrated arising from the intracavernous portion of the contralateral oculomotor nerve and the patient was treated successfully by subtotal excision.

We have recently encountered a patient with progressive dementia and parkinsonism beginning at the age of 68 years and developing for 2 years. Pathological examination revealed severe degeneration exclusively in the substantia nigra with absence of Lewy bodies and neurofibrillary changes. The features of this case are reported and discussed.

• Documented cases of classic amyotrophic lateral sclerosis (ALS) combined with severe dementia are few and come from the isolated populations of the Western Pacific. In this report, three women had a clinically and neuropathologically documented combination of ALS and dementia. In all cases the symptoms and signs of ALS were mainly bulbar. In two of them dementia appeared first, followed by ALS. At autopsy, there was a marked discrepancy between the severe degree of dementia of Alzheimer's type and the slight nonspecific neuropathologic findings without Alzheimer's changes. Our cases bear a close resemblance to recently published Japanese cases. It may be concluded that the combination of sporadic ALS and progressive dementia seems to be a clinical entity without consistent neuropathologic changes and that it also occurs in the western countries.

A rare hereditary syndrome is presented with pathognomonic talar cysts shown on plain radiography and diffuse presenile brain atrophy demonstrated on computed tomography (CT). The main value of CT is related to prognosis and follow-up, as well as to possible genetic counseling.

ALZHEIMER'S disease, a common progressive dementia of later life, is associated with several histopathological changes. Although one of these pathological changes, neuritic plaques with amyloid cores, has been reproduced in brain of mice following inoculation with scrapie agent1,2, Alzheimer-type neurofibrillary degeneration has not yet been reproduced in the laboratory. This unique form of human neurofibrillary degeneration is composed of arrays of paired 100-A diameter filaments twisted into a helical configuration with a period of approximately 800 A (refs 3, 4). We present here evidence that paired helical filaments morphologically closely resembling those found in the human disease are induced in cultured human foetal cerebral cortical neurones after exposure to an extract prepared from Alzheimer-affected brain.

Progressive dementia, vertical ophthalmoplegia, and prominent hypothalamic dysfunction developed in one patient with documented intestinal Whipple's disease despite ongoing antibiotic therapy with intestinal remission. A clinical diagnosis of central nervous Whipple's disease was made on the basis of the patient's presentation. High-dose parenteral penicillin and chloramphenicol were administered for 4 1/2 weeks. There was no improvement in results of daily mental status examination or neuropsychologic testing. Deterioration was noted in the electroencephalographic findings after therapy. Vigorous antibiotic therapy with agents that cross the blood-brain barrier had no immediate beneficial effect. Irreversible neurologic damage or a slow, delayed response may account for this observation.

The involvement of pathology in mental disease was confirmed by Wilks in 1864: The more marked changes which have been described as occurring in the insane, especially in those long demented, are those of an atrophic kind; the whole brain, when weighed, is found to be much lighter than an organ would be which had quite filled the skull case. Thus on removing the dura mater, the surface of the brain is often quite hidden by the layer of serum which lies beneath the arachnoid, and filling up the spaces between the convolutions; instead of the sulci meeting they are widely separated, and their intervals filled with serum, and which, on being removed with the pia mater, the full depth of the sulci can be seen, At the same time the ventricles are distended by a large amount of serum; their walls are granular and the choroid plexus is full of cysts. Such conditions are indicative of a wasted brain and are those which have been regarded as characteristic of a chronic form of insanity.

We studied 33 consecutive patients with computed tomographic findings of decreased density in the periventricular white matter (leukoaraiosis). Computed tomograms in five (aged 56-75 years) of the 33 demonstrated intracerebral hematoma. The hemorrhages were situated in the thalamic area in four and in the parietotemporal area in one patient. These five patients were hypertensive and had signs characteristic of parenchymal hemorrhage. Three of the five patients had progressive dementia prior to the ictus, and two of the three also had a history of single or multiple strokes. There was no significant difference in the clinical findings of hematoma patients with or without leukoaraiosis.

Atrial myxoma can appear with a broad array of systemic and/or neurologic symptoms. This article describes a previously unrecognized initial appearance: progressive multipleinfarct dementia and seizure disorder. This case is reported because of its rarity and because of the potential for therapy if clinically recognized. REPORT OF A CASE An 82-year-old man was examined at the Minneapolis Veterans Administration Medical Center for a one-year history of progressive mental decline. His symptoms began with progressive loss of recent memory, irritability, and narrowing of his range of interests. When driving his car in familiar surroundings, he became lost. His speech output diminished, and he finally was noticed to be disoriented. Eight months after his initial symptoms appeared, he began experiencing brief seizures characterized by stereotyped lapses in contact with his surroundings. On one occasion such an episode progressed to a major motor seizure for which phenytoin sodium was prescribed. He also

First described in 1907, Alzheimer’s disease (AD) is a neuropathological disorder that causes progressive dementia with deterioration of memory and all cognitive functions in advanced cases. The brain pathology of AD consists of the presence of senile plaques and neurofibrillary tangles (Wisniewsky and Terry, 1976; Constantinidis et al., 1978; Constantinidis and Tissot, 1979). Many studies on post-mortem brains of AD patients have consistently shown evidence of neurotransmitter impairment.

A 35-year-old homosexual man with acquired immune deficiency syndrome developed progressive dementia and spastic quadriparesis over a two-month period. Severe symmetrical, bilateral degeneration of the corticospinal and frontopontine fibers and cerebellar white matter were found postmortem. Several glial nodules in the brainstem and cerebellum suggested "smoldering" brainstem encephalitis. The possible association of the adenovirus that was isolated from the patient's cerebrospinal fluid to these obscure changes in the central nervous system is discussed.

Current prevalence rates for alcoholism, and associated depression and dementia, were determined on random samples of approximately 200 patients admitted to the Medicine Service, and a similar sample to the Surgery Service, of the Harlem Hospital Center. The Medicine patients averaged 51 years of age, significantly older than the Surgery sample's average age of 44 years. Surgery patients also had a significantly greater proportion of patients (46.8 percent) who had achieved at least a high school education compared to Medicine (32.1 percent). The alcohol prevalence rate of 30.2 percent for Medicine was significantly greater than the 18.3 percent Surgery prevalence. Both Medicine and Surgery patients showed that a progressively serious pattern of drinking was associated with progressively serious depression. Progressive dementia was associated with progressive severity of drinking in the Medicine sample, but this finding was not demonstrated in the Surgery patients. Medicine and Surgery patients demonstrated dissimilar profiles of principal admitting diagnoses. Patient management is seriously handicapped by problems of alcoholism and associated problems of depression and dementia.

The available clinical and pathological data of 5 cases with solitary midbrain metastasis including 2 of the present study are reviewed. Progressive dementia occurred in one case and mild dementia in another who also developed ocular symptoms. Ocular symptoms with sensory and coordination disturbances were seen in one, and only ocular symptoms in another case. Right-sided hemiplegia of 5 years duration occurred in the remaining case. Survival in tegmentum lesions is short.

Colloid cysts of the third ventricle represent 0.3%–2.0% of all brain tumors [10]. Despite their rarity, these lesions have fascinated neurosurgeons since the first was removed successfully through a pineal approach by Dandy in 1921 [3]. The interest in colloid cysts developed because of their surgical accessibility, their benign histology, and their occasionally disastrous sequelae when left untreated [4, 16]. However, their clinical behavior frequently is not benign. Although most patients (85%–96%) present with headaches, fluctuating or progressive dementia, seizures also are describe [18]. Even recently, sudden unconsciousness followed by death, possibly secondary to acute hydrocephalus leading to herniation, has been associated with colloid cysts [16].

This is a pathological analysis of a 50-year-old severely mentally retarded female with trisomy 21 who five years prior to her demise developed progressive dementia, epileptic seizures and choreiform movements. The necropsy revealed gross and microscopic features of Alzheimer's and Pick's disease. Ultrastructurally the majority of neurofibrillary changes and all studied Pick bodies consisted of 15 nm straight tubules. A few neurofibrillary changes were composed of 22 nm paired helical filaments, which were also found in the enlarged neurites of neuritic plaques. A few paired helical filaments were interspersed between straight tubules of Pick bodies. These findings in a patient with Down's syndrome and previous reports of atypical features of Alzheimer's disease indicate that very probably our case is a variant of Alzheimer's disease, thus broadening the spectrum of pathological changes observed in Alzheimer's disease.

This is a case report of a thirty-seven-year-old man with systemic noninflam matory vasculopathy who developed a slowly progressive dementia with multi ple sites of stroke. Cerebral angiography revealed abnormal terminal vascula ture with beading and occlusions. Brain biopsy confirmed a noninflammatory vascular pathology. We have also reviewed the world literature relevant to this clinicopathologic entity and have discussed its relationship to thromboangiitis obliterans cerebri.

Some very relevant information about Alzheimer’s disease and other progressive dementias has surfaced through the endeavors of health care professionals who painstakenly compiled case histories and noted observations over time of patients who presented with cognitive disorders. For example, during the first decade of this century, Alois Alzheimer, a neuropathologist, used a case study approach with historical dimensions to describe behavioral and mental changes in a patient with a progressive and irreversible form of dementia (Wolstenholme & O’Conner, 1970). Today, this condition is referred to as Alzheimer’s disease (AD or senile dementia of the Alzheimer’s type (SDAT). The information obtained by using the case history approach and clinical observations served as the impetus for Dr. Alzheimer’s post-mortem exploration of brain cells to identify changes associated with the dementia (Reisberg, 1981). This report is divided into two sections, the first section focuses upon the use of case histories to describe the onset and progression of AD, and the second portion describes a program of research which has developed a health history profile to discriminate between AD and other disorders.

Since our earlier CT examinations of 31 AIDS patients with central nervous disease (Hacker and Merdes 1986), we have extended our experience to a total of 127 cases. All patients presented with the full clinical picture of AIDS with manifestation of neurological disturbances, beginning with headache and meningitic reactions and extending to severe AIDS encephalopathy with development of progressive dementia. The patients, who suffered from opportunistic infections and lymphomas, often had supplementary focal neurological deficits.

A 73-year-old white woman presented with a six-week history of weight loss, joint pain, and weakness in the lower extremities. The family had noted episodes of anxiety and confusion.

Lymphocytes from 3 children with a form of juvenile amaurotic idiocy (characterized by retinal blindness, progressive dementia, and extrapyramidal motor disturbance) were studied by electron microscopy. Numerous fingerprint profiles (FP) were found in vacuolated lymphocytes from all 3 patients and an asymptomatic younger sibling of 1 patient who subsequently became symptomatic. We propose that the combination of this clinical picture and vacuolated lymphocytes with FP is sufficiently distinctive for clinical and research purposes until the biochemical defect is discovered. Wider utilization of ultrastructural study of lymphocytes should increase the number of children diagnosed and allow detection of asymptomatic patients.