大脳白質に巨大空洞を形成したWilson病の1例

A CASE OF WILSON'S DISEASE WITH ENORMOUS CAVITY FORMATION OF CEREBRAL WHITE MATTER

はじめに

　Wilson^17）による肝脳疾患の報告以来，大脳では主としてレンズ核に注意が払われてきたが，時には白質や皮質の病変がレンズ核のそれを，はるかにしのぐ症例も報告されてきている。すでに1930年，v．Braunmühl^1）は前頭葉の脳回部の白質の軟化に注意を喚起している。しかし後頭葉をのぞく白質の大部分に空洞を形成した症例はまれである。私たちは最近，大脳白質に大空洞を形成し，大脳皮質，小脳にも著しい変化をみたWilson病の1例を経験したので，その臨床，病理像を報告し，あわせて白質の空洞形成について若干の考察を加えたい。

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.