Al-Hashel Jasem Y, Abdelnabi Ehab A, Ibrahim Ismail Ismail
Department of Neurology, Ibn Sina Hospital, Safat, Kuwait.
Department of Medicine, Health Sciences Centre, Faculty of Medicine, Kuwait University, Jabriya, Kuwait.
Case Rep Neurol. 2020 Sep 17;12(3):291-298. doi: 10.1159/000508994. eCollection 2020 Sep-Dec.
Hirayama disease is a rare neurological entity that is characterized by initial progressive muscular wasting and weakness of the distal upper limb in young men, followed by a spontaneous arrest within several years. The disease is believed to be a result of forward displacement of the cervical dural sac and spinal cord induced by neck flexion. It is commonly seen in Asia and rarely encountered in the Middle East countries. We report a rare case of a 20-year-old Kuwaiti patient presenting with a 10-month duration of gradual left upper limb weakness and wasting. We describe his electrophysiological and radiological findings that confirmed the diagnosis, and conducted a literature review. Hirayama disease is rarely encountered in clinical settings and should be suspected in male patients presenting with unilateral or asymmetrical bilateral lower motor weakness of hands and forearms. It is a benign entity, and cervical collar is usually the only treatment needed in most cases.
平山病是一种罕见的神经系统疾病，其特征为年轻男性最初出现进行性肌肉萎缩和上肢远端无力，随后在数年内自行停止发展。该病被认为是颈部屈曲导致颈段硬膜囊和脊髓向前移位的结果。它在亚洲较为常见，在中东国家很少见。我们报告了一例罕见的20岁科威特患者，其左上肢逐渐无力和萎缩已有10个月。我们描述了确诊该疾病的电生理和影像学检查结果，并进行了文献综述。平山病在临床中很少见，对于出现单侧或不对称双侧手部和前臂下运动神经元无力的男性患者应怀疑此病。它是一种良性疾病，在大多数情况下，颈托通常是唯一需要的治疗方法。
