Tanaka J
Acta Neuropathol. 1980;50(3):193-7. doi: 10.1007/BF00688753.
Leukoencephalopathic alteration in a case of membranous lipodystrophy which involves the systemic adipose tissue has been described. A 44-year-old Japanese man whose parents were consanguinous developed multiple cystic lesions in the limb bones with a pathological fracture, followed by progressive neuropsychiatric symptoms, and died 16 years after the onset of his illness. Brain showed diffuse and symmetrical demyelination of the cerebral white matter, particularly in the fronto-temporal lbes, with preservation of subcortical arcuate fibers, associated with the occurrence of sudanophilic granules within perivascular macrophages and conspicuous fibrous gliosis. The alteration suggests it to be a sudanophilic leukodystrophy. Deposition of calcospherites in the basal ganglia was another interesting remark. This disorder is thought to be associated with an impairment in systemic lipid metabolism affecting both cerebral myelin and fat cell membranes on a heredofamilial background.
已描述了一例累及全身脂肪组织的膜性脂肪营养不良病例中的脑白质病变。一名44岁的日本男子，其父母为近亲结婚，在四肢骨骼出现多发性囊性病变并伴有病理性骨折，随后出现进行性神经精神症状，发病16年后死亡。脑部显示大脑白质弥漫性对称性脱髓鞘，尤其是在额颞叶，皮质下弓形纤维保存完好，伴有血管周围巨噬细胞内嗜苏丹颗粒的出现和明显的纤维性胶质增生。这种改变提示为嗜苏丹性脑白质营养不良。基底神经节中钙球的沉积是另一个有趣的发现。这种疾病被认为与遗传性家族背景下影响脑髓鞘和脂肪细胞膜的全身脂质代谢受损有关。
