<症例>白質が高度に崩壊し, 清掃機転の進んだと思われるsudanophilic leukodystrophyの1剖検例

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An autopsy case of adult sudanophilic leukodystrophy (SLD) with little lipid material, especially in the frontal area, is reported. A 40-year-old woman was admitted because of progressive mental deterioration and gait disturbance. Neurological and psychiatric examination on admission revealed severe dementia, muscle weakness, exaggerated deep tendon reflex in the left arm and leg, cerebellar signs, and involuntary athetosis-like movement. The patient subsequently fell into apallic syndrome and died at the age of 48. An autopsy examination disclosed severe diffuse destruction of the white matter of the centrum semiovale, especially in the frontal area. However the subcortical U-fibers were spared. Proliferation of glial fibers extended throughout the cerebral medulla and even to the deep cortex. Histologically, there were a few astrocytes and fat granular cells with sudanophilic granules in the cytoplasm. They were fewer in number in the frontal area than in the occipital area. There were few inflammatory cells in the perivascular region and degradation products of axons were also found. Lipid analysis did not reveal any cholesterol ester. In most previous reports, the transportation of the myelin breakdown products was retarded. In this case, however, the transportation appeared highly advanced as if it were at the end stage. The results of biochemical analyses corresponded with the morphological findings.
1986-06-25