Nasu-Hakola disease: a review of its leukoencephalopathic and membranolipodystrophic features.

Nasu-Hakola disease: a review of its leukoencephalopathic and membranolipodystrophic features. Research Abstract Details 

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Nasu-Hakola disease: a review of its leukoencephalopathic and membranolipodystrophic features. Abstract Text:

The clinicopathological features of Nasu-Hakola disease are described by reviewing previously reported cases and adding consideration to newly disclosed evidence. This disease is an autosomal recessive disorder characterized by membranocystic lipodystrophy in the skeletal system and sclerosing leukoencephalopathy in the nervous system. The leukoencephalopathic alterations are demyelinization of the cerebral white matter, associated with conspicuous fibrillary gliosis and preservation of the subcortical arcuate fibers. Sudanophilic granules are focally scattered in the perivascular space or widely infiltrated in the affected white matter, and some neuronal loss with deposits of calcospherites is encountered in the basal ganglia and also in the thalamus. Spheroid formation with an increased number of neurofilaments in the neuronal axon is considered a possible pathogenesis, and a primary vascular mechanism is also suggested. Interestingly, most of the reported cases of Nasu-Hakola disease are from Japan and Finland which suggests heredofamilial background as a cause.

Nasu-Hakola disease: a review of its leukoencephalopathic and membranolipodystrophic features. Publishing Authors By Initials

For similar pathological conditions, signs and symptoms: pathologic processes: nerve degeneration research abstracts see: pathological conditions, signs and symptoms: pathologic processes: nerve degeneration research

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Nasu-Hakola disease: a review of its leukoencephalopathic and membranolipodystrophic features. Journal Published:

PUBLICATION TYPE: Review

Journal: Neuropathology : official journal of the Japanese

VOLUME: 20 Suppl

Page Numbers: S25-9

Journal Abbreviation: Neuropathology

ISSN: 0919-6544

DAY: 10

MONTH: Sep

YEAR: 2000

Nasu-Hakola disease: a review of its leukoencephalopathic and membranolipodystrophic features. Information

Number of References: 33

LANGUAGE: eng

NlmUniqueID: 9606526

Nasu-Hakola disease: a review of its leukoencephalopathic and membranolipodystrophic features. Keywords Mesh Terms:

KEYWORDS: Nerve Degeneration

MESH TERMS: pathology

Chemical & Substance for Abstract: Nasu-Hakola disease: a review of its leukoencephalopathic and membranolipodystrophic features. Information

Substance Name: Lipids

Registry Number: 0

Grant and Affiliation Information for Nasu-Hakola disease: a review of its leukoencephalopathic and membranolipodystrophic features.

AFFILIATION: Division of Neuropathology, Jikei University School of Medicine, Tokyo, Japan. jtanaka@jikei.ac.jp

Country: AUSTRALIA

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MEDLINETA: Neuropathology

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