V．Recklinghausen病と脳腫瘍

INTRACRANIAL TUMORS ASSOCIATED WITH VON RECKLINGHAUSEN'S DISEASE

I．はじめに

　V．Recklinghausen病（以下R病と略す）がNeuro—ectodermの系統的疾患としてCafe au lait spotsや末梢神経の多発性神経鞘腫（神経線維腫）などの皮膚病変ばかりでなく，脳腫瘍をはじめとする種々の中枢神経系の病変，骨格系や内分泌系の異常などを伴うことは広く知られた事実である。

　脳の病変もdysplasiaまたはmalformationよりneo—plasmに到るまでの種々の段階のものを含み，その多彩な変化の全貌の解明は病理学者のみがなし得るであろう。したがつてここではよりpracticalな観点，脳神経外科医という臨床家の立場から，脳神経外科の治療の対象となつた症例，換言すればR病に脳腫瘍を伴つた症例について報告し，この問題に多少の考察を加えてみようと思う。

1) The authors reported 27 cases of intracranial tu-mors associated with von Recklinghausen's disease and reviewed literatures.

2) Most frequently complicated tumor in Reckling-hausen's neuromatosis is acoustic neurinoma, and 21 of our 27 intracranial tumors in neurofibromatosis were acoustic neurinomas. These acoustic neurinomas have a tendency of bilateral involvement and effected younger people.. ten year younger than the average of all acoustic neurinoma cases.

3) Next to acoustic neurinoma comes glioma. Dif-ferent from the reports by other authors, the present series had only few gliomas of optic tract and of chiasmatic region which are said to be one of the predirections of such tumors.

4) Our cases may support the conclusion of Crowe et al," Individuals with central nervous system in-volvement have, on average, less striking pigmentary changes than those individuals with primarily a peri-pheral form of neurofibromatosis ".

5) Hereditary occurrence of von Recklinghausen's disease may necessitate some eugenical treatment of patients with this disease parallel with symptoma-tological treatment of such patients.