成人定型発症を示すRigid-Akinetic FormのHuntington舞踏病の症状推移について

INSPECTION OF SYMPTOMATIC CHANGE WHICH SHOW RIGID-AKINETIC FORM OF HUNTINGTON'S CHOREA STARTING WITH TYPICAL ADULT TYPE

I．はじめに

　1908年にHamilton^12）が，筋のrigidityとakinesiaを有する慢性進行性舞踏病の数症例を報告して以後chorea-athetosisよりも筋のrigidityとakinesiaを特徴とするHuntington舞踏病（以下H．　C．病と略す。）の報告が，諸家によりなされ分類^2,4,14,24）されているが，それらは各々の特徴により3グループに分けられる。

　1）若年硬直型

The rigid form of Huntington's chorea have been reported from neuropsychiatrical and neuropatho-logical points of view. But the study of adult rigid form are insufficient. We inspect a case of Huntington's chorea which previously started with typical chorea and recently has showed rigid-akinetic change. We divide this clinical course following four stages and describe each stage. 1. latent partial chorea 2. progressive typical chorea 3. mixed stage 4. rigid-akinetic stage.

From the examinations of pedigree research during five generations, 25 members, 8 patients are found. Existence of two separate groups are dis-cussed. The first one, the symptoms start at the age of the fourth decade and gradually show typical Huntington's chorea. The second one, they start with typical chorea at the age of the third decade, but later they show rigid-akinetic feature. It is difficult to distinguish the latter from other extra-pyramidal diseases, for instance, Parkinsonism or Dystonia musculorum deformans without finding typical Huntington's chorea in a pedigree and her own typical choreic past history. We are interested in the differences between juvenile and adult form. Then we compare and discuss these two forms about our cases and literatures regarding with the onset, duration and some clinical features.