title: Familial Idiopathic Cranial Neuropathy in a Chinese Family.
abstract: Cranial neuropathy is usually idiopathic and familial cases are uncommon. We describe a family with 5 members with cranial neuropathy over 3 generations. All affected patients were women, indicating an X-linked dominant or an autosomal dominant mode of inheritance. Our cases and a review of the literature suggest that familial idiopathic cranial neuropathy is a rare condition which may be related to autosomal dominant vascular disorders (e.g. vascular tortuosity, sclerosis, elongation or extension), small posterior cranial fossas, anatomical variations of the posterior circulation, hypersensitivity of cranial nerves and other abnormalities. Moreover, microvascular decompression is the treatment of choice because vascular compression is the main factor in the pathogenesis. To the best of our knowledge, this is the first report of familial cranial neuropathy in China.
authors.affiliation: China-Japan Friendship Hospital, Department of Neurosurgery, Beijing, People's Republic of China.
authors.forename: Li
authors.initials: L
authors.lastname: Zhang
authors.forename: Jianfeng
authors.initials: J
authors.lastname: Liang
authors.forename: Yanbing
authors.initials: Y
authors.lastname: Yu
mesh_terms: D000368:Aged; D044466:Asian People; D002196:Capillaries / Q000473:pathology; D002869:Chromosome Aberrations; D003389:Cranial Nerve Diseases / Q000235:genetics* / Q000473:pathology* / Q000601:surgery; D003391:Cranial Nerves / Q000002:abnormalities / Q000473:pathology; D005260:Female; D040181:Genetic Diseases, X-Linked / Q000235:genetics / Q000473:pathology; D006801:Humans; D061145:Microvascular Decompression Surgery; D008875:Middle Aged; D019635:Neurosurgical Procedures; D010146:Pain / Q000209:etiology; D010375:Pedigree; D019051:Rhizotomy; D012886:Skull / Q000002:abnormalities