Sturge-Weber症候群にみられたatlanto-occipital assimilation—クモ膜下出血を思わせる激しい頭痛発作で発症した症例

STURGE-WEBER SYNDROME ASSOCIATED WITH ATLANTO-OCCIPITAL ASSIMILATION : A REPORT OF A CASE

I．はじめに

　Sturge-Weber syndrome （SWS）はすでに記載されていた3つのphacomatosisに続いて，1936年van derHoeveにより第4番目に加えられた疾患である^9）。一方，craniovertebral junctionは同部が胎生学的に複雑な発育過程をとるため，骨および神経組織の奇形が起り易く，また他の奇形としばしば合併して起ることもよく知られている^35,36）。

　著者らは，突然の後頭〜後頸部痛で発症したSWSの患者にatlanto-occipital assimilation （AOA）を合併していた症例を経験した。J．SchmittはSWSの総説の中で「この疾患の神経皮膚病変以外の臨床所見に関する報告や記載がほとんどみられないことは驚くべきである。」^30）と述べているが，著者らの渉猟し得た範囲でも，両疾患の合併症は文献上見つからなかつた。そこで，本症例についてのいくつかの臨床的問題点とともに，若干の文献的考察を加えて報告する。

A unique association of Sturge-Weber syndrome and atlanto-occipital assimilation is presented. A 18-year-old male was admitted in emergency because of the sudden severe headache and vomiting. He had vascular nevus in the right half of the face at birth and several episodes of generalized con-vulsive seizures. On admission craniogram demon-strated calcification in the right occipital area. Neurological examination revealed tenderness in the nuchal region, moderate limitation of cervical mobility in a antero-posterior direction, Bruns-Cushing type nystagmus, bilaterally diminished gag reflex, and positive Romberg's test. Spinal tap showed crystal clear CSR with normal pressure. EEG showed paroxysmal slowing focus in the right parieto-occipital area. Polytomography of the craniovertebral junction demonstrated the unilateral atlanto-occipital assimilation on the left associated with the aplasia of the right posterior arch.Myelography was negative. A right carotid angio-graphy disclosed the dilatation of the basal vein of Rosenthal and abnormal venous vasculature. CT-scan demonstrated the calcified region of the right occipital area more distinctly than the plain roento-genogram, but the enhancement study of the lepto-meningeal angioma of the Sturge-Weber syndrome was negtaive.

Never been found this rare association in a review of the literature, the authors discussed the clinical and radiological findings of both diseases.