[A case of "membranous lipodystrophy (Nasu)" with emphasis on psychiatric and neuropathologic aspects (author's transl)].
A housewife, 42 years old, died from a chronic progressive neuro-psychiatric illness of 15 years duration characterized by memory disturbance, moria-syndrome, euphoria, social disorder and extrapyramidal symptoms combined with a severe bone disease. Pathologically, the adipose tissue in bone marrow, and also in other body areas such as the periadrenal region and renal pelvis showed a characteristic degeneration with "membranous cycle" structures. Neuropathological examination showed an extensive atrophy of cerebral white matter, especially in the frontal and temporal lobes, with a marked fibrillary gliosis, a moderate destruction of myelin sheath without grossly visible distinct demyelination and with a relatively normal cerebral cortex: a picture resembling sclerosing leukoencephalopathy. From these findings the case was identified as "membranous lipodystrophy--Nasu." Moreover, the case may be related to the "lipomembranous polycystic osteodysplasia with progressive dementia" of Järvi, Hakola et. al. In cases with an unknown atrophic illness of cerebral white matter there should be carried out a pathological examination of adipose tissue, especially of the bone marrow.
D000273:Adipose Tissue / Q000473:pathology*; D000328:Adult; D001847:Bone Diseases / Q000150:complications / Q000235:genetics* / Q000473:pathology; D001853:Bone Marrow / Q000473:pathology*; D002540:Cerebral Cortex / Q000473:pathology*; D005260:Female; D006801:Humans; D019965:Neurocognitive Disorders / Q000209:etiology*; D013344:Subacute Sclerosing Panencephalitis / Q000150:complications / Q000235:genetics* / Q000473:pathology
Ein Fall von "Membranöser Lipodystrophie (Nasu)", unter besonderer Berücksichtigung des psychiatrischen und neuropathologischen Befundes