Hallervorden-Spatz Disease: An electron microscopic observation

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Electron microscopic study was performed on the brain of an autopsy case of Hallervorden-Spatz disease. The patient developed unsteady gait and night blindness at the age of 4 years, and then dystonia and opisthotonus at the age of 10 years. She died of status epilepticus 4 days after the right stereotaxic thalamotomy performed at the age of 11 years. Frontal section of the brain revealed bilateral rust-brownish discoloration in the globus pallidus and the substantia nigra, which microscopically showed the brown pigment granules and numerous spheroids. Electron microscopic obsevation on the globus pallidus showed the lysosome-like bodies containing ferritin-like granules in neurons and glial cells. Appearance of the dense material and lipid in the lysosome-like bodies was considered to be analogous to the lipofuscins, which were observed as the pigment granules in the light microscopy. Some axons were filled with abundant mitochondria, myelin-like bodies and dense bodies. A few oval bodies composed of the multilamellated bodies and lysosome-like bodies were observed, which were observed as spheroids in the light microscopy. However, the relationship to infantile neurcaxonal dystrophy and significance of the lysosome-like bodies and multilamellated bodies still remained unknown.
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