Summary
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 lobes, 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 backgound.
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References
Akai M, Tateishi A, Cheng CH, Morii K, Abe M, Ohono T, Ben M (1977) Membranous lipodystrophy. A clinicopathological study of six cases. J Bone Joint Surg 59-A:802–809
Hakola HPA (1972) Neuropsychiatric and genetic aspects of a new hereditary disease characterized by progressive dementia and lipomembranous polycystic osteodysplasia. Acta Psychiatr. Scand [Suppl] 232:1–173
Harada K (1979) Ein Fall von “Membranöser Lipodystrophie (Nasu)”, unter besonderer Berücksichtigung des psychiatrischen und neuropathologischen Befundes. Folia Psychiatr Neurol Jpn 29:169–177
Inoue K, Fukuyama M, Suda A, Higuchi I (1975) Intraosseous lipomatosis. Report of a case. J Jpn Orthop Assa 49:223–230
Järvi O (1970) A new entity of phacomatosis: A. Bone lesion (Hereditary polycystic osteodysplasia). Acta Pathol Microbiol Scand [Suppl] 215:27
Laasonen EM (1975) Das Syndrom der polyzystischen Osteodysplasie mit progressiver Demenz. Forsch Röntgenstr 122:313–316
Nasu T, Tsukahara Y (1971) An autopsy case of “Membranous lipodystrophy” with myeloosteopathy of long bones and leukodystrophy of barin (abstract). The 17th Autumn Meeting of Japanese Pathologic Society, Tokyo
Nasu T, Tsukahara Y, Terayama K (1973) A lipid metabolic disease — “Membranous lipodystrophy” — An autopsy case demonstrating numerous peculiar membrane-structures composed of compound lipid in bone and bone marrow and various adipose tissue. Acta Pathol Jpn 23:539–558
Nasu T (1978) Pathology of membranous lipodystrophy (Japanese). Tr Soc Pathol Jpn 67:58–98
Schaumburg HH, Powers JM, Raine CS, Suzuki K, Richardson EP (1975) Adrenoleukodystrophy. A clinical and pathological study of 17 cases. Arch Neurol 32:577–591
Sourander P (1970) A new entity of phacomatosis: B. Brain lesions (Sclerosing leukoencephalopathy). Acta Pathol Microbiol Scand [Suppl] 215:44
Tanaka J, Garcia JH, Khurana R, Kamijyo Y, Viloria JE (1975) Unusual demyelinating disease. A form of diffuse-disseminated sclerosis. Neurology (Minneap) 25:588–593
Wolman M (1968) Involvement of nervous tissue in primary familial xanthomatosis with adrenal calcification. Pathol Eur 3:259–265
Wood C (1968) Membranous lipodystrophy of bone. Arch Pathol Lab Med 102:22–27
Yagishita S, Ito Y, Ikezaki R (1976) Lipomembranous polycystic osteodysplasia. Virchows Arch [Pathol Anat] 372:245–251
Yakumaru K, Matsuyama H, Kageyama K, Nasu T (1973) An autopsy case of membranous lipodystrophy (abstract). The 19th Autumn Meeting of Japanese Pathologic Society, Tokyo
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Tanaka, J. Leukoencephalopathic alteration in membranous lipodystrophy. Acta Neuropathol 50, 193–197 (1980). https://doi.org/10.1007/BF00688753
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DOI: https://doi.org/10.1007/BF00688753