Werner P, Kiechl S, Löscher W, Poewe W, Willeit J
Department of Neurology, Innsbruck University, Innsbruck, Austria.
Acta Neurol Scand. 2003 Sep;108(3):209-11. doi: 10.1034/j.1600-0404.2003.00136.x.
In Myasthenia gravis (MG) proximal limb, ocular and/or bulbar muscles are most commonly affected, whereas distal extremity muscles are typically spared. The aim of the current study was to assess the frequency of primarily distal MG in the Tyrol and to describe its clinical peculiarities.
Over the past 20 years 84 patients with MG have undergone follow-up at the Department of Neurology of Innsbruck University. Types of presentation, clinical course and treatment response were followed over a period of 20 years (1980-2000).
Six of 84 MG patients showed a predominance of muscle weakness and fatigability in distal limb muscles (two at presentation, four over the later course of the illness). There was no difference between distal MG and MG with a more typical distribution of muscle weakness regarding age, gender and response to therapy.
The case series indicates that predominantly distal presentations of otherwise typical MG are more frequent than generally assumed and should be considered in the differential diagnosis of diseases with distal limb weakness.
在重症肌无力（MG）中，近端肢体、眼部和/或延髓肌肉最常受累，而远端肢体肌肉通常不受累。本研究的目的是评估蒂罗尔州原发性远端型重症肌无力的发生率，并描述其临床特点。
在过去20年中，84例重症肌无力患者在因斯布鲁克大学神经病学系接受了随访。对其临床表现类型、临床病程及治疗反应进行了为期20年（1980 - 2000年）的跟踪观察。
84例重症肌无力患者中有6例表现为远端肢体肌肉为主的肌无力和疲劳感（2例初诊时即如此，4例在疾病后期出现）。远端型重症肌无力与肌无力分布更为典型的重症肌无力在年龄、性别及治疗反应方面无差异。
该病例系列表明，原本典型的重症肌无力以远端表现为主的情况比一般认为的更为常见，在鉴别诊断伴有远端肢体无力的疾病时应予以考虑。
