<原著>Hallervorden-Spatz病の1剖検例

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Clinical, neuropathological and histochemical studies were done of a 11 year old girl with Hallervorden-Spatz disease. Clinically, unsteady gait and night blindness were noticed and retinal degeneration was found in physical examination at 6 years old of age. Neurologic findings on her final admission included articulation disorder, hypertonus of muscles, incoordination of extremities, athetoid movements and mental deterioration, and these symptoms gradually developed. On 23 October, 1970,she expired from bronchopneumonia and malnutrition. The total duration of the illness was approximately 6 years. At autopsy, the brain weighed 1,090g and the characteristic finding was light brown pigmentation in the globus pallidus bilaterally. On microscopic examination, a number of brown pigment granules were found intra- and extra-cellularly in the parenchyma of the globus pallidus, and spheroids were distributed numerously in the globus pallidus and the internal capsule. Histochemically, the pigment granules were variably positive in iron, PAS, fat, acid-fast, argentaffin and nile blue sulfate reactions, and negative in bleach in H_2O_2. It seemed that these granules were compcsed of lipofuscin-like pigments, melanin-like pigments and iron containing pigments, and the increase of them complicatedly related with degenerative change of pigment metabolism and abnormal senility. The spheroids, stained positive in PAS and bromphenol blue reactions, had continuity to the neuroaxons. Spheroids seemed to originate from the dystrophic neuroaxons.
近畿大学の論文
1977-06-25

<原著>Hallervorden-Spatz病の1剖検例

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