The analysis of the course of disability in Werdnig-Hoffman disease (chronic type)

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  • Okawa Yayoi
    Central Rehabilitation Service, University of Tokyo Hospital
  • Eto Fumio
    Central Rehabilitation Service, University of Tokyo Hospital
  • Ueda Satoshi
    Central Rehabilitation Service, University of Tokyo Hospital

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  • Ｗｅｒｄｎｉｇ‐Ｈｏｆｆｍａｎｎ病（慢性経過型）の障害経過についての検討
  • Werdning Hoffmannビョウ マンセイ ケイカガタ ノ ショウガイ

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The analysis of the course of disability in 27 cases [12 males and 15 females] with chronic type of Werdnig-Hoffmann disease [intermediate type of SPMA according to Emery 1981] revealed the following.<br>1) A new scale of Motor Ability Level for Werdnig-Hoffmann disease was constructed based on the analysis which, incidentally, were revealed to be the same as the scale of Fukuyama-type congenital muscular dystrophy.<br>2) There was no consistent pattern in the course of disability in Werdnig-Hoffmann disease, but quite a disversity was witnessed depending on individual cases. But we could divide them into four groups according to the rate of motor progress, and the peak motor level.<br>3) The patients of Werdnig-Hoffmann disease are usually severely disabled physically, but not at all impaired intellectually and they could be generally accepted to regular education.<br>4) There is a conspicuous superiority in female cases in motor development, especially gait, suggesting a strong sex difference in phonotypes of this hereditary conditions.

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