The analysis of the course of disability in Werdnig-Hoffman disease (chronic type)
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概要
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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.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.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.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.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.
- 社団法人 日本リハビリテーション医学会の論文
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