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題名 | A Prodromal Sign of Ataxia Disease: Machado-Joseph Shoe=早期步態不穩疾病之警訊-Machado-Joseph鞋 |
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作者姓名(中文) | 劉青山; 陳逸芸; 蔡靜姍; 郭珍菱; | 書刊名 | 彰化醫學 |
卷期 | 8:2 2003.04[民92.04] |
頁次 | 頁76-79 |
分類號 | 415.93 |
關鍵詞 | Machado-Joseph疾病; 早期症狀; 鞋; Machado-Joseph disease; Prodromal sign; Shoe; |
語文 | 英文(English) |
中文摘要 | 背景及目的:Machado-Joseph疾病(Machado-Joseph Disease, MJD)是自體顯性小腦萎縮疾病,其症狀多樣化,在本次研究中,我們發現此類病人鞋底之大拇指處有特殊之凹陷表徵,常表現在步態不穩疾病中,尤其以Machado-Joseph疾病患者為主,我們特稱為Ataxia鞋(Ataxia Shoe, AS)。 方法:總共收集31有症狀之MJD病患,第二型脊髓小腦萎縮症8人,偶發性小腦萎縮症10人及健康族群200人,並且無任何足部關節病變者,參與此項臨床問診,其中MJD與第二型脊髓小腦萎縮症患者皆經分子診斷證實。 結果:百分之五十五MJD患者在早期皆出現AS症狀,並且其出現之年齡與MJD突變基因之CAG增長數目呈反比(P=0.023)。 結論:AS可當作早期步態不穩疾病之發生警訊,尤其在MJD患者身上有較高發生率。 |
英文摘要 | Background and purpose: Machado-Joseph disease (MJD) is an autosomal dominant spinocerebellar degeneration characterized by a wide range of clinical manifestations. In the present clinical study, we investigated an early particular and prodromal sign, ataxia shoe (AS), which occurs in ataxia disorders, especially MJD. Methods: AS was defined as deep hallux imprint in the base of shoe without other abrading. The pictures and characteristics of AS were demonstrated in 31 patients with symptomatic MJD, eight patients with SCA 2, 10 patients of sporadic cerebellar atrophy, and in 200 healthy controls without any disease of plantar joints. Results: High incidence of AS was found; AS was present in 17 (55%) of 31 patients with MJD. A significantly negative correlation between the age at onset of AS and the CAG repeat number was found in patients with MJD (P=0.023). Conclusion: AS may be served as a warning sign of ataxia disease, especially in the prodromal stage of MJD for which AS may be a simple and distinctive clinical manifestatioin. |
本系統之摘要資訊系依該期刊論文摘要之資訊為主。