Sclerosing leucoencephalopathy with membranocystic lesions of adipose tissue: a contribution to the pathology of Nasu disease.

A case of sclerosing leucoencephalopathy with membranocystic lesions of adipose tissue was reported. A 38-year-old Japanese housewife, whose parents were consanguineous, developed gradually neuropsychiatric symptoms characterized by euphoria, gait disturbance and urinary incontinence, followed by spastic tetraplegia with epileptic convulsions and died eight years later. Neuropathologically a form of sudanophilic leucodystrophy with a prominent fibrillary gliosis (dissociation glio-myelinique) associated with axonal spheroids and calcospherite depositions was revealed. A peculiar membranocystic lesion of adipose tissue was recognized not only in the bone marrow but also in other areas. However, no apparent bone pathology was observed except for a ring-like pleated lamellar structure. This case was thought to be classified as heterogeneous phenotype of membranous lipodystrophy (Nasu).