異常脳波所見をみたCrouzon病の1例

A CASE REPORT OF CROUZON'S DISEASE WITH ABNORMAL EEG FINDING

I．緒言

　1912年Crouzonは塔状頭蓋に顔面奇形をともなう母子例を報告し，これを一独立疾患として遺伝性頭蓋顔面異骨症Dysostose cranio-faciale héréditaireと命名した。彼によれば本症は，（1）塔状頭維を思わせる頭部奇形，（2）眼球突出，（3）上顎骨発育不全による下顎突出，（4）遺伝性・家族性の4主徴をもつ先天性奇形である。

　本邦においても1930年萩野^5）が初めて本症を報告して以来，数10例におよぶが，主として眼科・小児科からの報告が多く，数例をのぞいては単発例で，しかも典型的な症例は少ない。

A case of typical Crouzon's disease with a abnor-mal EEG finding was reported. He complained of marked bilateral exophthalmus and left strabismus divergens. He had a facial distortion with a saddle nose and a prognathous lower jaw, because of his undeveloped maxilla. His skull is hypsicephalic, hyperbrachycephalic and oxycephalic with Larsen's protuberance. By an X-ray examination, there were evidenced a thinning of the cranial bones with marked digitation and prematurely closed sutures coronalis.

The EEC report of Crouzon's disease was rare but this case had a remarkable abnormal finding. The basic rhythm was characterized by moderate voltage irregular 8 to 10 per second activity in all leads, which was frequently interspersed with a short run of high voltage 6 to 8 per second slow waves confined to the frontal lead. Such a paroxy-small dysrhythmia was observed to be dominant on the right side. During hyperventilation the paroxy-smal slow waves were markedly exaggerated within the frontal areas and at the sleeping record EEG shows normal sleep record and no specific seizure pattern.