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摘要


本病例為一8歲男性病患,在6歲時曾有暫時性視力喪失,後來逐漸地肌肉張力增加,肌肉力量減少,步伐困難,智力輕微不足。腦部電腦斷層攝影顯示:豆狀核的被殼及基底核萎縮,視力:右眼0.7(0.8/cyl+1.50ax→)左眼0.2(無法矯正)。眼底檢查:雙眼視神經盤萎縮。本文報告此一罕見病例臨床經過及眼底攝影所見。

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並列摘要


Hallervorden-Spatz disease is a rare, familial (autosomal recessive) and fatal disorder first described in 1922, as a progressive degenerative disorder of the basal gangna. We present the unusual case, an 8 year-old male patient with the symptoms & signs of progressive dystonia, dysarthria, disturbance of gait. The Ocular findings includes decreased visual acuity & optic atrophy. There is no special treatment, the prognosis is poor.

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