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Query: UMLS:C0002395 (Alzheimer's disease)
 110,584 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Serial EEG studies and full neuropathological investigations (optic and electronic microscopy of biopsy and necropsy material) were carried out on two patients: 1. A 68-year-old man: development in two and a half months of Creutzfeldt-Jakob's disease signalled by early clinical and EEG changes and confirmed by associated spongiosis of the triad characteristic of Alzheimer's disease, which was unexpected in this case. 2. A 43-year-old man: first phase of four years of progressive deterioration, followed by an encephalopathic syndrome with myoclonus developing in twelve months. The serial EEG studies showed discontinuous periodic paroxystic activity from the start of the second phase of the disease, although the first biopsy still showed nothing but the signs of Alzheimer's disease. A fortnight later, a second biopsy revealed ultrastructural microspongiosis. Examination of necropsy material confirmed the extensive association of the characteristic images of the two processes. On the basis of these two case studies and some similar cases published in the literature, the authors discuss the possible etiological links between these two diseases and stress the importance of the EEG and cerebral biopsy for the purposes of differential diagnosis. (Acta neurol. belg., 1977, 77, 202-212).
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PMID:[Association of Alzheimer's disease and Creutzfeldt-Jakob's disease (author's transl)]. 33 54

A few autopsy proved cases of Alzheimer's disease with myoclonus have been hitherto reported. We think that myoclonus is a frequent clinical feature in advanced cases of Alzheimer's disease. Our second case is such an example with a chronic evolution. In cases like our first one, with a short history, myoclonus, and atypical, diphasic, periodic complexes in the EEG, clinical differential diagnosis with Creutzfeldt-Jakob disease can be very difficult. Alzheimer disease has been considered an unitary clinico-pathological entity. However, transmission to the non human primates has been successfully achieved only in familiar cases but no in the sporadic ones. On the other hand some of his neuropathological features have been found in two cases of Creutzfeldt-Jakob disease, one of them successfully transmitted to the chimpanzee. All these points could eventually modify our present unitary concept on Alzheimer's disease.
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PMID:[Myoclonus in Alzheimer's disease. 2 anatomoclinical cases]. 39 58

Myoclonus in association with dementia of later adult life has been considered almost pathognomonic of Creutzfeldt-Jakob disease. However, myoclonus may also be seen with Alzheimer disease, and when the myoclonus occurs as an early manifestation of Alzheimer disease, distinction from Creutzfeldt-Jakob disease may prove difficult.
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PMID:Myoclonus in Alzheimer disease. A confusing sign. 76 61

Neurologic signs and their neuropathologic correlates were examined in a sample of 56 patients with autopsy-proved Alzheimer's disease (13 men, 43 women; mean age at death, 83.1 years; range, 67 to 96 years) from a prospective longitudinal study. Full-range regular rigidity with cog-wheeling was found in 20 patients and was significantly associated with lower neuron counts in the substantia nigra and with the presence of Lewy bodies in the brain stem and neocortex. Twelve patients with myoclonus had a younger age at onset, a lower age at death (mean, 78.6 years), and lower neuron counts in the serotoninergic dorsal raphe nucleus and in the noradrenergic locus coeruleus than did the patients without myoclonus. Generalized motor seizures were reported in six patients, and they had significantly lower counts of pyramidal cells in cortical layers III through IV of the parietal cortex (area 7) and slightly decreased pyramidal cell numbers in the parahippocampal gyrus (area 28). The 19 patients with a positive grasp reflex had an earlier onset of illness and a significantly inferior performance on the Mini-Mental State examination and Cambridge Cognitive Examination tests. They, and 25 patients with a positive snout reflex, had significantly lower counts of large pyramidal cells in layers III through V of the frontal cortex (area 32). These results indicate that different neurologic symptoms in Alzheimer's disease can be related to disproportionate neuronal degeneration in functionally different brain areas.
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PMID:Neurologic signs in Alzheimer's disease. Results of a prospective clinical and neuropathologic study. 141 11

Atypical dementias confront the adequacy of current diagnostic concepts. The two patients with atypical dementia syndromes described here shared common postmortem features of numerous neocortical neuritic (senile) plaques and microvascular amyloid, sparing of hippocampus and substantia nigra, and the virtual absence of neurofibrillary tangles. Microscopically, the two differed only by the presence of a few subcortical Lewy bodies in case 1. These similar morphologic features were associated with dramatically different clinical presentations. In the first patient, visual hallucinations, Capgras' syndrome, cognitive slowing, myoclonus, parkinsonism, and primitive reflexes evolved over 3 years. Memory and language were relatively spared. In the second, dysphagia, nonfluent aphasia, hypophonia, motor perseveration, and a severe disorder of attention developed during this 18-month illness. At autopsy, an unrecognized colon malignancy was found. Despite high neuritic plaque counts in cortex, neither the clinical nor the pathologic criteria for Alzheimer's disease adequately describe either case. The cases will be examined first as clinical, then as neuropathologic, entities. From this approach, we conclude that a specific clinical dementia syndrome may be expressed by several neuropathologic "diseases" and that a variety of clinical syndromes may represent a single neuropathologic diagnosis. This strategy identifies a conceptual dichotomy between Alzheimer's syndrome and postmortem Alzheimer's disease. Meticulous clinical and neuropathologic observation is essential in advancing an understanding of the relationship between the two.
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PMID:Alzheimer's disease or plaque disease? Two cases at the frontier of a definition. 141 64

A 56-year-old woman initially noticed dizziness in October, 1988, and later dementia and gait disturbance developed, associated with myoclonus and periodic synchronous discharge in the electroencephalogram. On the basis of these clinical findings we made a diagnosis of Creutzfeldt-Jakob disease (CJD). Using RIA for ubiquitin (signal peptide of the ATP dependent proteolytic system), we measured the cerebrospinal fluid (CSF) ubiquitin levels. The CSF level of ubiquitin was markedly elevated in this case five months after the initial symptoms (230.0 ng/ml) compared with normal values (14.3 +/- 1.1 ng/ml) and values in patients with senile dementia of Alzheimer type (21.3 +/- 6.1 ng/ml) and vascular dementia (16.6 +/- 6.4 ng/ml). With progression of brain atrophy in this case, CSF levels of ubiquitin rapidly decreased to near the normal values. These findings suggest that CSF ubiquitin concentration reflects the activity of the disease process in CJD, and it may be useful in the diagnosis of CJD.
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PMID:[A case of Creutzfeldt-Jakob disease with markedly elevated ubiquitin concentration in the cerebrospinal fluid]. 165 80

Cumulative risks of developing extrapyramidal signs, psychosis, and myoclonus in the course of Alzheimer's disease (AD) were estimated in 72 patients with probable AD by the Kaplan-Meier survival method. The cumulative risk functions were found to increase at different rates for different signs as AD progressed. Comparisons of the cumulative risk functions revealed that in the early stages of AD, extrapyramidal signs and psychosis were more likely to develop than myoclonus. As AD progressed, the risk of developing myoclonus became as great as that of developing the other two signs. This study suggests that extrapyramidal signs, psychosis, and myoclonus represent developmental features that mark the progression of AD, rather than indicators of disease subtypes. The estimated cumulative risk functions set a reasonable expectation for the timing and likelihood of the emergence of the clinical signs. This, in turn, might aid in disease prognosis because the biological bases of these signs have been established and they have been shown to be predictive of other markers of disease course.
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PMID:Cumulative risks of developing extrapyramidal signs, psychosis, or myoclonus in the course of Alzheimer's disease. 195 98

Familial Alzheimer's disease (FAD) is a dominantly inherited condition that may present with an early onset, and myoclonus occurs frequently in the course of the disease. We report clinical and neuropathologic data on 2 large Belgian families with FAD in which we obtained 17 autopsies of the CNS. In family A, each of 11 autopsies had the typical neuropathologic features of Alzheimer's disease (AD), and there were a few cerebellar plaques in the molecular layer. In family B, in addition to the typical characteristics of AD in 6 autopsies, there were numerous amyloid plaques in the cortical cerebellar layers. In both families, we immunostained the amyloid deposits for the A4 protein, and they were negative for prion-associated protein immunoreactivity.
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PMID:Early-onset Alzheimer's disease in 2 large Belgian families. 198 97

A German family with 21 members affected by Alzheimer disease (AD) was studied clinically and genetically. The diagnosis was histologically verified in three affected family members. Ancestors were traced through seven generations to a couple residing in East-Westfalia during the middle of the 19th century. Dementia was often accompanied by extrapyramidal features and myoclonus. No cases of Down syndrome or hematologic malignancy occurred in this family. Clinical manifestations, temporal progression, neurological testing, and neuropathological features do not differ from the more common sporadic form of AD. The inheritance pattern is most consistent with autosomal-dominant transmission.
Alzheimer Dis Assoc Disord 1991
PMID:Familial Alzheimer disease: a large, multigeneration German kindred. 202 23

Neurological signs were assessed in 178 patients satisfying NINCDS/ADRDA criteria for Alzheimer's disease. A snout reflex was present in 41%, extrapyramidal signs in 12%, drug-induced extrapyramidal signs in 3%, myoclonus in 5%, and a history of epileptic fits in 3%. A grasp reflex, extrapyramidal signs and symptoms were associated with severe cognitive impairment. On CT scanning, extrapyramidal signs were associated with increased 3rd ventricular size and basal ganglia calcification, a grasp reflex with frontal lobe atrophy and a history of epilepsy with left temporal lobe atrophy. Lateral ventricular size was greater in patients developing a grasp reflex during a 12 month follow-up. Extrapyramidal signs and primitive reflexes were associated with a higher mortality.
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PMID:Neurological signs in Alzheimer's disease. 202 51


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