Von Recklinghausen病に合併した両側聴神経腫瘍の1家系

A Family of von Recklinghausen's Disease combined with Bilateral Acoustic Neurinomas

Ⅰ．はじめに

　当教室において，昭和51年9月までにvon Recklinghausen病と診断された症例は18例あった．それらはすべて脳または脊髄に多発性の腫瘍を合併していた．そのうち両側聴神経腫瘍を合併していたものが12例あり，その中に明らかに遺伝的関係を見出せた1家系があったので報告する．

Up to this time, in our university hospital, the authors had 12 cases of von Recklinghausen's disease combined with bilateral acoustic neurinomas, of which 3 occurred in a family through two generations, being presented in this report.

Case 1. A 36-year-old male was admitted to our university hospital with complaints of bilateral deafness and loss of visual acuity. Neurological examination revealed bilateral papilledema, gaze nystagmus, impairment of cranial verves V and VII on the left side, bilateral deafness and ataxic gait. Cafe-au-lait spots and subcutaneous nodules were not seen.