Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P50583 (asymmetrical)
12,197 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A combination of medial temporal lobe atrophy, shown by computed tomography, and reduced blood flow in the parietotemporal cortex, shown by single photon emission tomography, was found in 86% (44/51) of patients with a clinical diagnosis of senile dementia of the Alzheimer type (SDAT). The same combination of changes was found in four out of 10 patients with other clinical types of dementia and in two out of 18 with no evidence of cognitive deficit. Of the 12 patients who died, 10 fulfilled histopathological criteria for Alzheimer's disease, nine of them having a clinical diagnosis of SDAT, and one a clinical diagnosis of multi-infarct dementia. All 10 patients with histopathologically diagnosed Alzheimer's disease had shown a combination of hippocampal atrophy and reduced parietotemporal blood flow in life. In 10 patients (nine with SDAT) out of 12 in whom the hippocampal atrophy was more noticeable on one side of the brain than on the other the parietotemporal perfusion deficit was also asymmetrical, being greater on the side showing more hippocampal atrophy. These results suggest that the combination of atrophy of the hippocampal formation and reduced blood flow in the parietotemporal region is a feature of dementia of the Alzheimer type and that the functional change in the parietotemporal region might be related to the loss of the projection neurons in the parahippocampal gyrus that innervate this region of the neocortex.
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PMID:Association of atrophy of the medial temporal lobe with reduced blood flow in the posterior parietotemporal cortex in patients with a clinical and pathological diagnosis of Alzheimer's disease. 156 78

A progressive disorder of relatively focal but asymmetric biposterior dysfunction is described in a 54 year old right handed male. Initial clinical features included letter-by-letter alexia, visual anomia, acalculia, mild agraphia, constructional apraxia, and visuospatial compromise. Serial testing demonstrated relentless deterioration with additional development of transcortical sensory aphasia, Gerstmann's tetrad, and severe visuoperceptual impairment. Amnesia was not an early clinical feature. Judgment, personality, insight, and awareness remained preserved throughout most of the clinical course. Extinction in the right visual field to bilateral stimulation was the sole neurological abnormality. Early CT was normal and late MRI showed asymmetrical bioccipitoparietal atrophy with greater involvement of the left hemisphere. Results from positron emission tomography (PET) showed bilaterally asymmetric (left greater than right) occipitotemporoparietal hypometabolism. The metabolic decrement was strikingly asymmetric with a 50% reduction in glucose consumption confined to the left occipital cortex. The picture of occipitotemporoparietal compromise verified by MRI, PET, and neurobehavioural testing would be unusual for such degenerative dementias as Alzheimer's (AD) and Pick's disease, although atypical AD with predominant occipital lobe involvement cannot be excluded. This case supports the concepts of posterior cortical dementia (PCD) as a clinically distinct entity and for the first time documents its corresponding metabolic deficit using PET.
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PMID:Posterior cortical dementia with alexia: neurobehavioural, MRI, and PET findings. 186 9

A case of intraventricular cyst associated with normal pressure hydrocephalic condition (NPHC) is reported. A 72 year-old female, with 2-year-history of slowly progressing dementia and gait disturbance, was admitted to our hospital on September 19, 1989. On admission, she had mild dementia, unsteadiness of gait, and at times urinary incontinence. Cerebrospinal fluid (CSF) pressure was found to be 90mmH2O by lumbar tap. Plain computed tomographic (CT) scan and T1-weighted magnetic resonance image (MRI) showed asymmetrical enlargement of the trigon and posterior horn of the right lateral ventricle. CT cisternography showed a cyst in the trigon and in the posterior horn of the right lateral ventricle. T1-weighted MRI with Gd-DTPA demonstrated no enhancement of the cyst wall, and there was superior and posteromedial displacement of the choroid plexus at the trigon of the right lateral ventricle. The patient was diagnosed as having an intraventricular cyst in the right trigon with NPHC. Ventriculo-peritoneal shunt and partial removal of the cyst were performed. Histological examination of the cyst wall revealed collagenous strands and no epithelial cells. Developmental intracranial cysts, especially arachnoid or ependymal cysts, occasionally lack an epithelial layer, so their histological diagnosis is difficult. This case was considered to be an arachnoid cyst because there was adhesion between the cyst and the choroid plexus in the right trigon, and superior, posteromedial displacement of the choroid plexus, which indicated extension of the cyst from the extracerebral to the intracerebral region. NPHC was considered to be due to disturbance of CSF circulation caused by gradual expansion of the cyst.
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PMID:[A case of intraventricular cyst associated with normal pressure hydrocephalic condition]. 194 2

Clinical records and EEGs of 114 consecutive patients with clinical diagnosis of Alzheimer's disease have been reviewed. The EEGs of 15 patients (13%) contained triphasic waves. Triphasic waves were always atypical as they occurred singularly or in short bursts, and/or they had occipital predominance, and/or they were bilateral but asymmetrical. Triphasic waves, in all but one patient were recorded in a state of complete alertness. No statistically significant differences were found between the triphasic waves group and the other one, with respect to age, age at onset, illness duration, presence of seizures. Myoclonus was more frequent in patients with triphasic waves (p less than 0.01). Triphasic waves patients had a higher degree of dementia (p less than 0.01), both in cases with rapidly evolving disease and in subjects with relatively slow clinical course.
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PMID:Triphasic waves in Alzheimer's disease. 208 90

Lafora's disease is included among the progressive myoclonic epilepsies. Despite the fact that dementia is a constant finding in this disease only a few papers have studied the timing of mental deterioration. We have performed wide neuropsychological testing in two cases early diagnosed as Lafora disease. The initial neuropsychological testing was carried out by the time there were no complaints of mental deterioration in both cases. In the first case consecutive neuropsychological testing demonstrated the rapidly progressive dementia. All neuropsychological testings in these cases showed severe impairment of right parietal lobe functions. Higher cortical functions related to language and intellectual processes were best preserved in both cases. The functions related to constructional praxis, memory and abstract concepts and processes were severely impaired. Our data suggest that mental deterioration is an early manifestation in Lafora disease, even by the time normal social life is not yet disturbed. Dominant hemisphere cognitive functions have been less impaired than the non-dominant ones. How a diffuse illness such as Lafora disease can cause such an asymmetrical higher cortical function deficit is not yet clear.
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PMID:Mental deterioration in Lafora's disease. 212 83

A patient with supranuclear upward gaze palsy, pseudobulbar palsy, dementia, asymmetrical pyramidal signs, and atypical parkinsonism that developed after recurrent strokes was reported. The clinical features closely resembled idiopathic progressive supranuclear palsy (PSP). Computerised tomography of the brain showed, in addition to dilated third ventricle and prominent quadrigeminal plate and ambient cisterns, multiple infarcts at the thalamus, striatum, frontal subcortex and corona radiata. Multi-infarct PSP (MI-PSP) was thought to be the most likely diagnosis rather than coincidental idiopathic PSP with recurrent cerebral infarcts.
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PMID:Multi-infarct progressive supranuclear palsy--case report. 217 61

Seventy-one EEGs (0.49%) of 53 patients, out of 14,458 recordings, contained triphasic waves: twenty-nine were patients with metabolic encephalopathies and 24 were demented patients (16 of these had a presumptive diagnosis of Alzheimer's disease and the other eight of mult-infarct dementia). Demented subjects with metabolic disorders are included in the metabolic encephalopathies group. In all of the cases of Alzheimer's disease, triphasic waves were atypical: in 14 they occurred singly or in short bursts, in 10 they had occipital predominance and in 2 they were bilateral but asymmetrical. In 5 cases, triphasic waves were associated with myoclonus and in 2 of them they occurred in long runs with a pseudo-periodic pattern. In these cases the distinction from Creutzfeldt-Jakob disease was based on neuropathologic findings.
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PMID:[Triphasic waves in dementia syndromes]. 261 26

Because of its binding to specific cerebral amine receptor sites 123I-N-isopropyl-amphetamine (IMP) tracer activity can be used as a measure for the regional cerebral blood flow (rCBF) in the human brain. In 21 psychiatric in-patients an IMP perfusion study was performed using a rotating gamma camera system. The findings at single photon emission computed tomography (SPECT) were compared with different clinical parameters including conventional transmission computed tomography. In SPECT, two different patterns of decrease in IMP uptake could be identified. In patients with a history of cerebrovascular disease SPECT showed asymmetrical, multifocal microcirculatory defects without preference for either hemisphere. In patients with suggested Alzheimer type dementia the perfusion deficits involved the gray and white matter of the parieto-occipital lobes in a bilaterally symmetrical mode with variable extension. Thus, by using IMP-SPECT, it was possible to define the underlying pathologic condition in dementia.
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PMID:123I-N-isopropyl-amphetamine single photon emission computed tomography as a brain imaging technique in dementia. 298 May 27

Six hundred and sixty-one patients with stroke, confirmed by CT scan or at autopsy, were reviewed in order to evaluate the frequency of presentation with altered mental state. Nineteen patients (3%) had presented with delirium, an organic delusional state, the acute onset of dementia, or mania, mimicking psychiatric illness. All had focal cerebrovascular lesions which were usually, but not invariably, right sided. None had a previous history of cognitive impairment, psychiatric disease, drug abuse, or alcohol excess. Neurological signs were absent or mild and transient, and therefore easily missed. Post-mortem examinations in four patients showed localised cerebral infarctions with no evidence of multiple lesions, Alzheimer's disease, or metabolic encephalopathies. The possible causative factors are discussed and the evidence of asymmetrical cerebral representation of emotion, and for a relationship with epilepsy, is reviewed.
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PMID:Inobvious stroke: a cause of delirium and dementia. 347 Nov 94

A clinico-pathological entity of progressive neuronal degeneration of childhood with liver disease has now been recognised. Onset is in early childhood with intractable fits and progressive dementia. EEG/ERG/VEP studies have been carried out in 12 children with this condition. In most patients the EEG showed strikingly similar and unusual abnormal patterns (high amplitude slow activity together with smaller polyspikes). The flash VEP was usually abnormal and often asymmetrical. In the appropriate clinical setting the neurophysiological features are sufficiently characteristic to aid the clinician in early diagnosis of this autosomal recessive disorder.
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PMID:Progressive neuronal degeneration of childhood with liver disease ("Alpers' disease"): characteristic neurophysiological features. 372 91


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