脳表ヘモジデリン沈着症について

A CASE OF SUPERFICIAL HEMOSIDEROSIS OF THE CENTRAL NERVOUS SYSTEM

はじめに

　脳表ヘモジデリン沈着症（superficial or subpial hemo—siderosis of the central nervous system, Siderose derRandzonen des Zentralnervensystems,la siderosemarginale du systeme nerveux central）はNoetzel^18）（1940）によりはじめて報告された。これは長期間持続する，または反復するくも膜下出血に由来するhemo—siderinが，中枢神経系組織の表層に沈着することにより神経細胞（とくに小脳），脳神経，脊髄神経根の障害を招くものとされている。

　神経学的所見としては，小脳失調，神経性難聴，精神障害，脊髄症状などを主体とし慢性の経過をとる比較的まれな疾患で，Noetzel^18）の2症例以来現在まで36例が報告されている（第1表）。

The patient is a 23-year-old girl who developed gradually progressed deafness on both sides and visual disturbance followed by headache and gait disturbance. On first admission a neurological examination revealed bilateral advanced papilledema, nerve deafness, truncal ataxia and urinary retention. A lumbar puncture demonstrated blood-stained CSF. The posterior fossa was explored to show the cere-bellum and the medulla to be rusty brown in color and the foramen magnum to be obstructed. No tumor was seen. A Torkildsen procedure was done. Headache, truncal ataxia, and urinary retension were cleared up. The angiograms failed to show either vascular lesion or space taking mass. In following 3 years the patient had two attacks of subarachnoid hemorrhage in association with re-current truncal ataxia and urinary retention. The patient underwent a V-A shunt operation on the last admission. A piece of cerebral tissue was removed during operation and this showed hemo-siderosis in the superficial layer of the brain. The origin of the repeated subarachnoid hemorrhages was not known in this case.

This is the thirty seventh case of superficial hemosiderosis of the CNS to be reported in the world literature. It is agreed that the common cause of superficial hemosiderosis of the CNS is recurrent or persistent intraventricular or sub-arachnoid hemorrhages.