Hirayama Disease (HD) is a focal motor neuron disorder generally affecting young adults with a male predominance who experience weakness and atrophy in distal upper extremity muscles in an asymmetric or unilateral pattern. Progression is insidious though significant weakness occurs during a progressive phase of the disease over 2–5 years. The long-term outcome of HD is not as well-known and, thus, this study presents self-reported outcomes from HD patients years after a diagnosis. Thirty HD patients reported quality of life (QOL) and other functional outcome measures after a mean of just over 11 years from diagnosis. Variables that predicted better or worse outcome were analyzed. Overall, QOL was affected by HD though most patients were functional with limitations. No clear attributes of patients or their disease predicted outcome.

中文翻译：

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平山病患者的长期生活质量随访和功能损伤研究

平山病 (HD) 是一种局灶性运动神经元疾病，通常影响以男性为主的年轻人，他们会出现上肢远端肌肉不对称或单侧无力和萎缩的情况。尽管在 2-5 年的疾病进展阶段会出现明显的无力，但进展是隐匿的。 HD 的长期结果尚不为人所知，因此，本研究提供了 HD 患者在诊断数年后自我报告的结果。 30 名 HD 患者在诊断后平均 11 年多后报告了生活质量 (QOL) 和其他功能结果指标。分析了预测更好或更差结果的变量。总体而言，虽然大多数患者功能有限，但生活质量受到 HD 的影响。患者或其疾病的明确特征无法预测结果。