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Query: UMLS:C0032285 (pneumonia)
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An autopsied case of Pick's disease, having an extraordinary cerebral change in the anterior portion of Lobus frontalis and temporalis, was reported. Our case is a 71 year-old woman at death with a fourteen year history of chronic progressive dementia and mental deterioration, and it may be stressed that the existence lasted 8 years, over the latter half of clinical course, was depended on the tube feeding. The first symptoms suddenly appeared in 1964, 2 months after her husband's death of illness, when she was 57. She prepared the table for breakfast late at night, calculated wrongly in her domestic account book, and stole foods in the grocery. Two years later, her illness was diagnosed as presenile dementia by characteristic personality change and marked dilatation of anterior horn of lateral ventriculus. On admission to National Musashi Sanatorium, three years after the first symptoms' appearance, she presented restless walking, insomnia, memory loss, weakness of concentration, and high degree of disorientation. Particularly, it was noticeable that she behaved with bizzare contact. After 1970, tube feeding was introduced continuously, because of swallowing difficulty. Death occurred in July 1978 from a general weakness and a broncho-pneumonia, 14 years after the onset of the first symptoms. Autopsy revealed small and atrophied brain weighed 820 g. Cerebral cortical atrophy extended to frontal, temporal, insular, and parietal lobes, but right T-1 was relatively well preserved. On section, frontal and temporal ventriculus were remarkably enlarged and caudate nuclei were extremely atrophic.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case of Pick's disease with long duration--an extraordinary cerebral change in the fore part of cerebrum]. 381 43

We report a patient whose clinicopathological findings are compatible with those of the case reported by Menzel in 1891. This case was briefly reported by Kinoshita et al in 1967 as hereditary ataxia of Menzel type. But the concept of this disease has been confused, especially in the early investigations for the hereditary olivo-ponto-cerebellar atrophy (OPCA) in Japan. The Kinoshita's patient should be considered as the first Japanese case whose findings are identical with Menzel's report. This report represents a precise study of the case reported by Kinoshita et al. The patient was a 42-year-old Japanese woman. Her mother and one of her brothers suffered from the same disease. She began to experience progressive ataxia at the age of 30. At age 42, she was admitted to another hospital because of inability to walk and mental deterioration. Neurological examination revealed cerebellar ataxia in the extremities and trunk, childish personality change, dementia, diminished deep tendon reflexes with extensor plantar response bilaterally, slowness and hypokinesia in the movement, generalized muscular atrophy, and sensory disturbance prominent in deep sensory. She had no involuntary movement and dysautonomia. She had no retinal degeneration, nystagmus, nor progressive nuclear oculomotor palsy. She died of pneumonia. Neuropathological findings revealed brain weight of 850g.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[An autopsied Japanese case of hereditary olivo-ponto-cerebellar atrophy compatible with the original one of Menzel's report (1891)]. 833 24

An autopsy case with clinically and molecular genetically diagnosed Huntington's disease (HD) accompanied with minimal non-specific neuropathological features was reported. When the patient was 45 years old, he had faulty memory, mood swing, personality change and agitation. Neurological and psychiatric examinations revealed choreoathetoid movements in limbs and trunk, generalized hyperreflexia and mental deterioration. However, cerebellar ataxia and muscle rigidity were not disclosed. Neuroimaging study did not show a definite atrophy of heads of caudate nuclei. Neuroacanthocytosis and Wilson's disease were ruled out by the peripheral blood examination and serum Cu and ceruloplasmin examination. At the age of 55 he died of pneumonia. Post-mortem examination revealed minimal non-specific neuropathological features for HD (Vonsattel's grade 0), that is, no visible fibrillary gliosis in the striatum, and few neuronal loss and only proliferation of astrocytes (astrocytosis) in the striatum. Molecular-genetic study the patient's brain tissues and his youngest son's blood was performed. These studies revealed 40 CAG repeats in the patient, 56 CAG repeats in his youngest son. These results suggest they may be HD. Vonsattel et al. [ 1998] insist that grade 0 comprises 1% of all HD brains, and grade 1 comprises 4% of all HD brains. But we could not find any reports in which the clinical and neuropathological features were described in detail on the cases with clinically and molecular genetically diagnosed HD without specific pathological findings. Therefore, we present in detail the clinical and neuropathological features of such case.
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PMID:An autopsy case with clinically and molecular genetically diagnosed Huntington's disease with only minimal non-specific neuropathological findings. 1074 90