“近端型”平山病的磁共振成像特征：病例报告及文献复习
Magnetic resonance imaging features of "Proximal" hirayama disease: Case report and literature review.
作者信息
Narra Ramakrishna, Kamaraju Suseel Kumar
机构信息
Department of Radiodiagnosis, Katuri Medical College, Guntur, Andhra Pradesh, India.
出版信息
Surg Neurol Int. 2021 Dec 20;12:622. doi: 10.25259/SNI_1081_2021. eCollection 2021.
BACKGROUND
Proximal "Hirayama" disease (PHD) is characterized by proximal upper extremity atrophy. It is a rare variant of Hirayama disease (HD) which involves the proximal upper limb. Recognition of PHD's unique magnetic resonance (MR) findings is critical as the treatment options differ versus classical HD.
CASE DESCRIPTION
A 17-year-old male presented with gradual progressive upper extremity weakness and atrophy. On MR, PHD was demonstrated by C4-C5 kyphosis with a posterior epidural soft-tissue mass compressing the C4-C5 cord resulting in gliosis. As the patient declined surgery, he was followed for 1 year with a cervical collar during which time his deficit stabilized.
CONCLUSION
PHD, characterized by proximal upper extremity weakness and atrophy, has characteristic MR findings of kyphosis associated with cord compression and ischemia/gliosis. Select patients as the one we described who decline surgery may stabilize radiographically and clinically with the protracted utilization of a cervical collar.
背景
近端“平山病”（PHD）的特征是上肢近端萎缩。它是平山病（HD）的一种罕见变体，累及上肢近端。认识到PHD独特的磁共振（MR）表现至关重要，因为其治疗方案与经典HD不同。
病例描述
一名17岁男性，表现为上肢逐渐进行性无力和萎缩。磁共振成像显示，PHD表现为C4 - C5后凸畸形，伴有硬膜外软组织肿块压迫C4 - C5脊髓，导致胶质增生。由于患者拒绝手术，给他佩戴颈托随访1年，在此期间其功能缺损稳定。
结论
PHD以上肢近端无力和萎缩为特征，具有与脊髓压迫及缺血/胶质增生相关的后凸畸形这一特征性MR表现。像我们描述的这种拒绝手术的特定患者，通过长期使用颈托，影像学和临床症状可能会稳定。
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