Bobble-head doll syndromeを示した頭蓋底部巨大嚢胞の1例

BOBBLE-HEAD DOLL SYNDROME, REPORT OF A CASE WITH A LARGE BASAL ARACHNOID CYST

はじめに

　1966年Bentonら^2）はbobble-head doll syndrome （首振り人形症候群）なる症候群を初めて記載し，本症状を示した2症例を報告している。これらの症例は何れも第3脳室部の嚢胞とそれに基づく水頭症とを示しており，この嚢胞による脳の或る部分の変形が本症状の原因ではないかと推測している。われわれは最近，本症候群と思われる不随意運動を示し，頭蓋底部より発生して第3脳室を著明に挙上する巨大な髄液嚢胞を有する1症例を経験したので報告し若干の考按を加えたい。

A 13 year-old male case of bobble-head doll synd-rome was reported. Involuntary movements of the head in this case were not so typical as the original cases of Benton et al. in that the head movements in our case occurred only during gait and consisted of mixture of to-and-fro movements and right and left rotating movements of the head, but existenceof similar atypical cases has been described. Our case had a large basal arachnoid cyst, which was situated around the midline and caused marked de-formity of the third ventricle, and hydrocephalus. These findings are in common with the cases of Benton et al. and support their opinion that this syndrome may be caused by deformity in the region of the third ventricle. The etiology of the arach-noid cyst in our case is obscure, but history of head injury during infancy may have some relation. Fenestration between the cyst and the lateral ven-tricle combined with ventriculoperitoneostomy was performed for the purpose of obtaining a possible curative effect by collapsing the cyst, but allevia-tion of the syndrome was only slight.