[Multiple and familial meningiomatosis. A syndrome bordering on Recklinghausen's neurofibromatosis].
The authors present three cases of multiple, intra-cranial meningiomatosis with contact hyperostosis affecting the grandmother, mother and daughter, in a very stereotypic manner. No other sign of von Recklinghausen's disease was noted, except for two tiny neurofibromas on the left hand in the mother. Only the latter suffered from bilateral deafness, but it was not possible to give definite proof of the presence of an acoustic neuroma in this patient. Anatomical verification in the first case, and surgical intervention in the last, confirmed that it was in fact a meningioma. Thus, a new clinical form of expression of neuro-fibromatosis has been found.
D000328:Adult; D001932:Brain Neoplasms / Q000150:complications / Q000235:genetics*; D005094:Exophthalmos / Q000209:etiology; D005260:Female; D006429:Hemiplegia / Q000209:etiology; D006801:Humans; D008607:Intellectual Disability / Q000235:genetics; D007427:Intracranial Pressure; D008579:Meningioma / Q000150:complications / Q000235:genetics*; D008875:Middle Aged; D009456:Neurofibromatosis 1 / Q000150:complications / Q000235:genetics*; D010211:Papilledema / Q000209:etiology
Méningiomatose multiple et familiale. Un syndrome voisin de la neurofibromatose de Recklinghausen.