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Japanese

A CASE OF WILSON'S DISEASE WITH ENORMOUS CAVITY FORMATION OF CEREBRAL WHITE MATTER Hiroshi Ishino 1 , Takashi Mii 1 , Yasuaki Hayashi 1 , Akira Saito 1 , Saburo Otsuki 1 , Kenji Tanabe 2 1Department of Neuro-phychiatry, Okayama University Medical School 2Okayama Kenritsu Hospital pp.895-901
Published Date 1972/7/1
DOI https://doi.org/10.11477/mf.1406203159
  • Abstract
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1) Clinical course : A female, 12-year-old at the time of death, complained of drooping of the lower jaw, excess salivation, gait disturbance and became irritable at the age of 8. The patient was hospital-ized on the same year. On admissien, the facies was mask-like and speech was unintelligible. After-wards muscle rigidity, dystonic movements and generalized convulsions appeared and she remained apallic for last 2 years and nasal-fed. She died of pneumonia 3 and a half years after the onset. Laboratory findings revealed decreased serum cerulo-plasmin and moderate liver damage. Kayser-Fleis-cher rings were not observed.

2) Post Mortem findings : The liver showed a nodular cirrhosis and copper-granules were found.

The brain weighed 1070g. Frontal sections re-vealed a loss of deep and superficial white matter except for calcarine areas, hippocampal gyri and anterior portions of cingular and rectal gyri. The cortex was greatly reduced in thickness. Spongy state, active new formation of blood vessels, Alz-heimer cells and Opalski cells were found in the lenticular nuclei and cerebral cortex. Fibrous replacement of the white matter was completely lacking. In the cerebellum, dropping of Purkinje cells and glial proliferation in the molecular layer were found. There was secondary pyramidal tracts degeneration. Furthermore signs of circulatory di-sturbance such as congestion, hemorrhagic foci sur-rounded by glial wall and enlargement of perivas-cular space with proliferation of connective tissue were noted.

3) The authors discussed the pathogenesis of cavity formation.


Copyright © 1972, Igaku-Shoin Ltd. All rights reserved.

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電子版ISSN 2185-405X 印刷版ISSN 0006-8969 医学書院

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