Leucodyetrophy With Deposits Rosembling Rosenthal Fibres（Alexasnder type）の1例

One Case of Leucodystrophy with Deposits Resembling Rosenthal Fibres

I．緒言

　本疾患はProgressive fibrinoid degeneration offibrillary astrocytes associated with mental retardation in a hidrocephalic infantとしてAlexander（1949）^1）が記載して以来，欧米諸国で7例が報告されている。わが国では，第7回神経病理学会（1966）における松山^5）の報告につぐ第2例である。報告者によりさまざまの名称でよばれ，megalencephaly associated with hyalinepanneuropathy（L. Crome^2）），encephalopathy withpeculiar deposits throughout the brain and spinal cord（Wohlwillら^11）），leukcdystrophy with diffuseRosenthal fibre formation（Vogel ＆ Hallervorden^10））などとよばれ，最近はAlexander病と名づけられることも多い。われわれは，Greenfieldにしたがつてleucodystrophy with deposits resembling Rosenthalfibers（Alexander type）と名づけ，その臨床・病理的所見に電顕所見†をつけ加えて報告したい。

1) The clinical and pathological findings are described in a case of Alexander's disease (leucodystrophy with deposits resembling Rosenthal fibres), late infantile type, which began at 9 years and died at 21. Main clinical symptomes were speach disturbances, ataxia, bulbar palsy, pyramidal signs and mental deterioration.

2) Pathologiaclly the findings coinside with Alexander's disease. In additon to the changes in cerebrum, coalescent or patchy, dysmyelinated foci were disseminated in the various portions of the brain stem: these were lesions in the internal capsule, thalamus, cerebral peduncle, a part of red nucleus, pyramis, and around the olivary and dentate nuclei.

3) Clinical and neuropathological features were discussed with the reference to the cases hitherto reported.

4) Rosenthal fibres were studied with light and electron-microscops. Electron-microscopic examination disclosed two new findings in neurofibril and perivascural areas. Close relation of Rosenthal fiber to astrocytes was suspected through its figure and location, but confirmable evidence of its relation to thier cell bodies was not shown.

5) It was suspected that the luncodystrophy in this case was dysmelinogenic one.