手袋状および長靴下状筋萎縮症

GLOVE AND STOCKING MUSCULAR ATROPHY

I．はじめに

　さきにわれわれは"主として若年者にみられる手袋状および長靴下状の筋萎縮症"と題して上肢または下肢の特有な筋萎縮例31例について検討を加え若干の見解を述べた。このうち，下肢例中の2例はその後の検索で若干疑問点をみいだしたのでこれを除き，また，この報告以後も外来患者中に同様な症例がかなりえられたので，さきの報告例とあわせて53例について総括的に検討を加え，再度ここに報告することにした。

Cases of glove & stocking type muscular atrophy have already been reported by us. Since that time additional cases were expe-rienced and total 53 cases were summarized and their clinical features were discussed.

As already mentioned, atrophy develops in young ages with insidious onset and begins in distal portion of one extremity. Atrophy ceases its progression within one year. Howe-ver, some cases with the unilateral muscular atrophy in the upper limb show contralateral symmetrical muscular atrophy in several years.

Fasciculation, finger tremor, decreased skin temperature, "Kaltparese" and synchronization voltage of EMG in the atrophied region are characteristics of these disorders. In the most cases these findings are observed in the oppo-site non-atrophied extremity.

Minimal sensory disturbances are seen in some cases but pyramidal tract signs are never obtained.

Pathology of these disorders are unknown, while it may be suggested that there are simi-laritie sto Spinal Progressive Muscular Atro-phy or Neural Progressive Muscular Atrophy from clinical stand point.