Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0032285 (pneumonia)
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An autopsy case of a 65-year-old female with dentatorubropallidoluysian atrophy (DRPLA) is reported. Her mother had gait disturbance and died at the age of 63. Her mother's brother developed psychotic symptoms. A daughter of her older sister was observed to have involuntary movement when she admitted to a mental hospital due to post-delivery psychotic state. Her younger brother has developed gait disturbance from about 56-year-old. Her older son has suffered from schizophrenia for long years. Since 58-year-old, she developed cerebellar ataxic gait and three years later, choreic involuntary movement developed in her extremities and face and progressively became prominent. Since 63-year-old, abnormal behavior brought about by the visual hallucination was occasionally observed. At the age of 63, she admitted to a mental hospital because of persistent persecutive delusion for her husband and was clinically diagnosed as Huntington's chorea for her remarkable choreic movement and psychotic state with dementia. Hypertension was also noticed. At the age of 65, she died of acute pneumonia. The duration of her illness was about 6 years. Histopathological findings of the CNS: the brain weighed 1,014 g. Brainstem and spinal cord were noticed to be relatively small in size. The cerebral cortex was well preserved. The cerebral white matter was diffusely demyelinated in the central semiovale where arteriosclerotic change of the small vessels was remarkable. Significant pathological changes consisted of marked symmetrical atrophy of the following two systems, i. e., dentatofugal pallidoluysian systems.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[An autopsy case of dentatorubropallidoluysian atrophy (DRPLA) clinically diagnosed as Huntington's chorea]. 293 81

A 76-year-old man with parkinsonism and dementia was reported. He developed resting tremor at age 69 followed by hypokinesia, rigidity and small step gait. L-dopa ameliorated his symptoms with no hallucinations for the initial 5 years. His mental level did not decrease during that period. He was admitted to our hospital because of dehydration and fever at age 74. Subsequently, his cognitive function deteriorated, with visual hallucination. Serial brain CT studies displayed a progressive cerebral cortical atrophy without focal lesions. He died of respiratory distress syndrome and disseminated coagulopathy resulting from pneumonia, dehydration and syndrome malin. Postmortem examination revealed a marked bilateral loss of melanin-containing neurons with Lewy bodies in the substantia nigra and locus ceruleus. Lewy bodies were also in the basal nucleus of Meynert, with moderate neuronal cell loss. The distribution of Lewy bodies was widespread in the cerebral cortical areas, corresponding to the neocortical subtype according to the consensus guideline for the pathologic diagnosis of dementia with Lewy bodies. According to the criteria of the Consortium to Establish a Registry for Alzheimer's Disease, the age-related plaque score in the present case suggested Alzheimer's disease, although cortical neurofibrillary changes corresponded to stage II by the criteria of Braak and Braak. These pathological findings established the diagnosis of dementia with Lewy bodies from the quantitative and distributional viewpoints. Based on recent neuropathological evidence, a spectral theory, which presents idiopathic Alzheimer's disease and Parkinson's disease as the two extremes of a spectrum of neurodegeneration, has been proposed. Dementia with Lewy bodies is located in the middle of this spectrum. Pathological evaluation based on quantitative consensus guidelines is important to establish the diagnosis in patients with parkinsonism and dementia, since neuropathological changes of Alzheimer's disease, Parkinson's disease and dementia with Lewy bodies are often observed in a mixed manner in these patients.
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PMID:[An autopsy case of dementia with Lewy bodies who showed the typical parkinsonism in the initial five years]. 1120 Nov 92

We report an autopsy case of Parkinson's disease mimicking senile dementia of the Alzheimer type. A Japanese man developed memory disturbance and visual hallucination at age 70. Although he died from pneumonia at age of 74, he had no neurological signs throughout the clinical course. The weight of his brain was 1,420 g. Macroscopic examination of the brain revealed prominent depigmentation of the substantia nigra and locus ceruleus. Histological examination disclosed neuronal loss with astrocytosis and the appearance of the Lewy bodies in the nucleus basalis of Meynert, substantia nigra, locus ceruleus, and dorsal vagal nucleus. There were widespread senile plaques in the brain, including the precentral gyrus, which was compatible with Braak stage C. A small number of neurofibrillary changes were present in the limbic areas, consistent with Braak stage III. This case is consistent with brain stem dominance with the pathological diagnosis of the Consortium on Dementia with Lewy Bodies International Workshop. That is, it is compatible with Parkinson's disease. We postulate that the clinical features of Parkinson's disease are more widespread than previously considered.
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PMID:[An autopsy case of senile dementia with pathological features of Parkinson's disease]. 1537 89

We report an autopsy case showing neuropathologically abundant Lewy bodies and argyrophilic grains. A Japanese woman without hereditary burden developed parkinsonian gait at the age of 74, following by insomnia, memory disturbance, delirium, resting tremor, rigidity, and retropulsion. About 8 months later, a visual hallucination, concerning small worms, children, and so on, became obvious. About 16 months later, malignant lymphoma was detected. About 17 months later, she died of pneumonia. The total duration of illness was approximately one year and five months. The weight of the brain was 1153 g before fixation. Depigmentation of the substantia nigra and locusceruleus was prominent. Many argyrophilic grains were seen in the temporal lobe (T3, T4), amygdala, and hippocampal CA1. Some ballooned neurons were found in the amygdala. Many Lewy bodies were encountered in the transentorhinal region and cingulated gyrus. A few Lewy bodies were seen in the temporal, frontal, and parietal lobes. In this case, neuropathological examination is compatible for dementia of Lewy bodies and argyrophilic grain dementia, and clinical course is consistent with dementia of Lewy bodies. This report may contribute to the elucidation of the clinicopathological hallmarks of argyrophilic grain dementia and dementia with Lewy bodies.
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PMID:[Autopsy case of Lewy body dementia associated with abundant argyrophilic grains]. 1614 14