Ayas Zeynep Özözen, Asil Kıyasettin
Sakarya University Training and Research Hospital, Department of Neurology, Sakarya, Turkey.
Sakarya University Training and Research Hospital, Department of Radiology, Sakarya, Turkey.
Ideggyogy Sz. 2018 Jul 30;71(7-08):284-287. doi: 10.18071/isz.71.0284.
Hirayama disease is a rare, benign motor neuron disease. It has been proposed that the dura mater's posterior wall lacks sufficient elasticity in the lower cervical region and this causes the tense dura part to displace anteriorly upon flexion. The disease is described as involving unilateral upper extremity with a distal-onset. We reported weakness and atrophy of the proximal part of an extremity in a 45-year-old man who is diagnosed with Hirayama disease. Proximal onset is a rare type of Hirayama Disease. Clinicians must be alert of proximal involvement and the diagnosis should be confirmed with electrophysiological and flexion MRI studies.
平山病是一种罕见的良性运动神经元疾病。有人提出，硬脊膜后壁在颈下段缺乏足够的弹性，这导致硬脊膜紧张部分在屈曲时向前移位。该病被描述为累及单侧上肢，起病于远端。我们报告了一名45岁被诊断为平山病的男性患者肢体近端出现无力和萎缩。近端起病是平山病的一种罕见类型。临床医生必须警惕近端受累情况，诊断应通过电生理检查和屈曲位磁共振成像研究来证实。
