Juvenile asymmetric segmental spinal muscular atrophy (Hirayama's disease) Three cases without evidence of “flexion myelopathy”
Juvenile asymmetric segmental spinal muscular atrophy (Hirayama's disease) Three cases without evidence of “flexion myelopathy”
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青少年不对称节段性脊髓性肌萎缩症（平山病） 无“屈曲脊髓病”证据的三例
DOI：
10.1034/j.1600-0404.2001.00074.x
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发表时间：
2001
影响因子：
3.5
通讯作者：
W. Poewe
中科院分区：
文献类型：
--
作者：
J. Willeit;S. Kiechl;U. Kiechl;S. Golaszewski;S. Peer;W. Poewe
颈部运动时颈髓受压（屈曲性脊髓病）被认为是幼年型不对称性脊髓性肌萎缩症（JASSMA）的主要病理机制。我们介绍了3例具有JASSMA临床表现和下颈脊髓前角细胞区典型高强度信号（T2加权磁共振图像）但无动态脊髓压迫证据的患者。在所有这些患者中，必须假设与机械损伤不同的病理机制。
Compression of the cervical spinal cord during neck movements (“flexion myelopathy”) was proposed to be the main pathomechanism of juvenile asymmetric spinal muscular atrophy (JASSMA). We present 3 patients with the clinical appearance of JASSMA and typical high‐intensity signals in the anterior horn cell region of the lower cervical spinal cord (T2‐weighted magnetic resonance images) but without evidence of dynamic spinal cord compression. In all these patients pathomechanism distinct from mechanical damage must be assumed.