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Query: UMLS:C0036572 (seizures)
 80,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The clinical and neuropathologic findings of 55 adults and 30 children who received liver transplants were reviewed. Encephalopathy was the most common clinical neurologic syndrome and was usually caused by metabolic or anoxic causes. (Alzheimer type astrocytes were present in 73 per cent of patients, and evidence of diffuse hypoxic damage was present in 40 per cent of children and 25 per cent of adults.) Cerebrovascular lesions were a common finding with infarcts or hemorrhages present in 30 per cent of patients. CNS infections were documented in 34 per cent of patients. Seizures were present in a third of patients. Central pontine myelinolysis was present in 12 per cent of patients and was more common in adults than in children. Antemortem diagnosis of neurologic complications was more often based on clinical presentation rather than specific radiologic or laboratory tests.
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PMID:Neurologic complications of liver transplantation. 304 44

We reviewed 81 patients with dementia and autopsy findings of Alzheimer's disease (AD) to identify patients with seizures or myoclonus after onset of dementia. Eight (10%) had seizures, and eight others (10%) had myoclonus. The incidence of seizures was 10 times more than expected in a reference population. Seizures occurred in any stage of AD, but myoclonus was often a late manifestation. Both seizures and myoclonus, individually or together, are manifestations of AD and may be seen at any time in the course of the illness.
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PMID:Seizures and myoclonus in patients with Alzheimer's disease. 309 31

Clinical and neuropathologic evidence points to the development of Alzheimer's disease (AD) in seven Down's syndrome patients above age 40. Dementia was observed in these patients over periods of 2.5 to 9.2 years. The first clinical sign of AD, visual memory loss, was succeeded by impaired learning capacity and decreased occupational and social functioning, and culminated in seizures and urinary incontinence. The morphometric observations of the brains of these seven patients with AD showed that the numbers of plaques and tangles exceeded 20 per 1.5 X 10(6) microns2 area, in both the prefrontal and hippocampal cortices. Plaques and tangles were also evident in the basal ganglia, thalamus, hypothalamus, and midbrain. In addition, we found that four of the seven brains showed small strokes, and five of the seven amyloid angiopathy. This study also indicates that by longitudinal neuropsychological evaluations and lab tests, which exclude other causes of dementia, the diagnosis of AD can be made even in severely and profoundly retarded patients.
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PMID:Alzheimer's disease in Down's syndrome: clinicopathologic studies. 315 74

Although interest in possible structural substrates of epilepsy has been high since anatomical abnormalities in autopsy material were first noted, a clear pathognomic picture has yet to emerge for subtypes other than temporal lobe epilepsy. While identification of anatomical features unique to the diseased brain does not ensure that a cure will be found (viz. Alzheimer's disease), such knowledge can certainly guide researchers who must search out both the necessary and sufficient etiologic agent. Such investigation is hampered by the difficulty of separating cause from effect in clinical material, where the patient may have a history of drug use or of poorly controlled seizures. For instance, the cell-density depletion noted in temporal-lobe epileptics could follow from, or be a cause of, seizures. An experimental approach to isolating the structural substrates of epilepsy requires establishing several "levels" of epilepsy of varying duration, and observing the evolution of anatomical change. Such manipulation is, of course, possible only when an animal model is used, and it is for this reason, among others, that researchers have developed several of these. In general, there are two types of animal model: one where an exogenous agent (for example, a neurotoxin) or treatment (kindling) is applied, and another that relies on a strain of animal bred to exhibit seizures. Anatomical findings in this latter case have been reported infrequently, although the approach, which is not confounded by central nervous system (CNS) damage, seems promising as a model for certain types of epilepsy. Rodents have been among the best-researched genetic preparations, and several such reports appear in this volume. We have investigated one such strain, the Mongolian gerbil, which has been bred in UCLA's animal colony to exhibit seizures (the SS strain). Another strain (SR) has been bred to be seizure-free. Our initial study compared adult SS and SR animals with respect to two morphometric parameters in hippocampal region CA3. We found that the SS adult gerbil had fewer dendritic spines than its nonseizuring counterpart. Dendritic spines are considered the locus of excitatory synapses, and their density has been correlated with a variety of functional states in other paradigms. Our next set of questions concerned the development of these strain differences, and we accordingly set up four different age groups, bracketing the age at which seizures first appear in the gerbil. Again, spine-density differences appeared between SS and SR animals, but in the opposite direction from the adult case.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Structural substrates of epilepsy. 370 25

Forty-three patients affected with Alzheimer's disease were identified in a kindred of Italian origin, emigrated in part to the U.S.A. and France. Thirteen were known by history, 21 by medical record, and 9 by personal examination, of whom 5 were confirmed histopathologically. The clinical picture was fairly uniform: the first symptom was memory loss beginning around age 40. Psychotic-like symptoms often followed, with rapid evolution into profound dementia, and death around age 50. Akinesia was prominent at a late stage, often with myoclonus. Grand mal seizures sometimes occurred, with occasional interictal spike and wave discharge; repetitive paroxystic periodic discharges were never recorded. A genealogical study, as far as possible free from line bias, has been conducted mainly by analysis of municipal records. 1 435 subjects in 10 generations, linked to affected subjects through ascent/descent or marriage, were listed in a computer file; the corresponding genealogical tree or selected part thereof are generated by computer. Application of Bayesian techniques to demographic data makes possible an estimation of disease probability in subjects for which no clinical data were available: such an estimate was confirmed by the later discovery of a living patient in descent of a subject with 0.7 estimated disease probability. No patient was found in descent from an inbred union known as such. Patients are the only transmitters. The sex ratio is not significantly different from 1. There is no detectable maternal effect. The segregation ratio, as calculated from extensively known sibships, lies in the range 0.65 to 0.89; the lower value itself is significatively higher than the 0.5 value expected in an autosomal dominant monogenic Mendelian transmission. An environment factor is ruled out by the diversity of locations and circumstances in kindred members. Such a kindred may represent an useful model for fundamental studies in Alzheimer's disease and senile dementia of the Alzheimer type.
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PMID:[Alzheimer's presenile dementia transmitted in an extended kindred]. 400 7

Clinical manifestations of dementia were reviewed in 15 Down's syndrome (DS) patients referred to a neurological clinic over a 24-month period for mental deterioration. The ages ranged from 32-64 years. One hundred percent showed personality changes and loss of independent daily living skills, the presenting symptoms in two-thirds of the cases. Other manifestations included seizures (53%), gait deterioration (73%), sphincteric incontinence (40%), and pathological release reflexes (67%). All 7 patients with CT-scans showed moderate or severe central and peripheral cortical atrophy. Detailed clinical information is presented for two patients, one of whom showed a temporary remission with imipramine. A characteristic dementia syndrome appears to be present in a subpopulation of aging DA patients with radiographic findings of Alzheimer's disease.
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PMID:Dementia in Down's syndrome: observations from a neurology clinic. 621 49

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.
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PMID:Morphological features of Pick's and atypical Alzheimer's disease in Down's syndrome. 629 25

The authors compared with regard clinical and the radionuclide cisternography examination 27 patients with Hakim-Syndrome and 11 with Alzheimer-Disease. In all cases, dementia was a stable symptom. Patients with Hakim-Syndrome have an early gait disturbance, ataxia and epileptic seizures. Only in case of patients with Alzheimer-Disease we found aphasia, apraxia and psychotic disorders. 24 hours after the injection radionuclide cisternography showed ventricular retention and absence of parasagital accumulation only in patients with Hakim-Syndrome. Lighter changes (mixed cisternographic pattern) were found in patients with Alzheimer-Disease as well.
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PMID:[Clinical and cisternographic differential diagnosis between presenile dementia and Hakim syndrome]. 652 22

Positron emission tomography (PET) using 18F-fluorodeoxyglucose (FDG) was used to map local cerebral glucose utilization in the study of local cerebral function. This information differs fundamentally from structural assessment by means of computed tomography (CT). In normal human volunteers, the FDG scan was used to determine the cerebral metabolic response to controlled sensory stimulation and the effects of aging. After stroke, regional brain dysfunction is more extensive than had been suspected on the basis of CT scans. Cerebral metabolic patterns are distinctive among depressed and demented elderly patients. The FDG scan appears normal in the depressed patient, studded with multiple metabolic defects in patients with multiple infarct dementia, and in the patients with Alzheimer disease, metabolism is particularly reduced in the parietal cortex, but only slightly reduced in the caudate and thalamus. The caudate is markedly hypometabolic in Huntington disease, even in the absence of caudate atrophy, and possibly may be mildly hypometabolic even before the appearance of symptoms. The interictal FDG scan effectively detects hypometabolic brain zones that are sites of onset for seizures in patients with partial epilepsy, even though these zones usually appear normal on CT scans. The future prospects of PET are discussed.
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PMID:Imaging local brain function with emission computed tomography. 660 81

Progressive presenile dementia with lipomembranous polycystic osteodysplasia was first described by Jarvi and Hakola in an isolated region of Finland. We report the occurrence of this disorder in 4 of 10 siblings in an American family of Czechoslovakian ancestry. Characteristics of the disease include multiple bone cysts with pathologic fractures, progressive dementia with seizures and abnormal EEG, calcification of basal ganglia, and death in the fourth to six decades. Autosomal-recessive inheritance is likely. Electronmicroscopy of fat cells reveals peculiar membrane convolutions. Limited neuropathologic material has shown gliosis and demyelination of white matter, senile plaques, and neurofibrillary tangles. Leukemia and a disorder of intestinal motility may be associated findings. Prevalence of the disorder is unknown, partly because it may be confused with Alzheimer disease and fibrous dysplasia of bone. Radiographs of hands and feet should be part of the evaluation of patients with unexplained presenile dementia.
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PMID:Lipomembranous polycystic osteodysplasia (brain, bone, and fat disease): a genetic cause of presenile dementia. 668 64


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