[A family of von Recklinghausen's disease associated with bilateral acoustic neurinomas and falx meningiomas].
A familial or inherited occurrence of von Recklinghausen's disease has been reported hitherto, some of which were associated with intracranial tumors such as gliomas, neurinomas, meningiomas or others. Authers report a family (case 1: father, case 2: son, case 3: daughter) of von Recklinghausen's disease associated with both bilateral acoustic neurinomas and falx meningioma. Case 1. A 41 year-old male was admitted to our hospital with complaints of bilateral hearing disturbance, headache and loss of visual acuity. On admission, neurological examination revealed left papilledema, right cataracta, impairment of bilateral cranial nerves V, IX, X, bilateral deafness, cerebellar ataxia, motor weakness of lower extremities and sensory disturbance of peripheral region of extremities. Twelve subcutaneous nodules were noted over the entire body and Café-au-lait spots were found slightly to be scattered over the chest and the back. Craniogram and vertebral angiography demonstrated bilateral CP angle tumors, and the diagnosis of bilateral acoustic neurinomas was made. Subtotal removal of bilateral tumors and Torkildsen's shunting were carried out. Histological diagnosis was neurinoma. Post-operative course was good. But 6 years later, he complained of gait disturbance. Computed tomography revealed falx meningioma in the frontal lobe and recurrence of bilateral acoustic neurinomas. So meningioma was totally removed. Eight months after surgery he died of aspiration pneumonia aged 47. Case 2. A 15 year-old male, son of case 1, was admitted with complaints of bilateral deafness, gait disturbance and visual disturbance. Neurological examination disclosed left papilledema, right congenital cataracta, impairment of bilateral cranial nerves V, IX, X, ataxic gait and sensory disturbance of right lower extremity.(ABSTRACT TRUNCATED AT 250 WORDS)
D000293:Adolescent; D000328:Adult; D002528:Cerebellar Neoplasms / Q000235:genetics* / Q000473:pathology; D005260:Female; D006801:Humans; D008297:Male; D008577:Meningeal Neoplasms / Q000235:genetics* / Q000473:pathology; D008579:Meningioma / Q000235:genetics* / Q000473:pathology; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local; D009378:Neoplasms, Multiple Primary / Q000235:genetics*; D009456:Neurofibromatosis 1 / Q000235:genetics* / Q000473:pathology; D009464:Neuroma, Acoustic / Q000235:genetics* / Q000473:pathology