Sawada Daisuke, Fujii Katsunori, Misawa Sonoko, Shiohama Tadashi, Fukuhara Tomoyuki, Fujita Mayuko, Kuwabara Satoshi, Shimojo Naoki
Department of Pediatrics, Chiba University Graduate School of Medicine, Chiba, Japan.
Department of Pediatrics, Chiba University Graduate School of Medicine, Chiba, Japan.
Brain Dev. 2018 Oct;40(9):830-832. doi: 10.1016/j.braindev.2018.05.009. Epub 2018 May 28.
Guillain-Barré syndrome is an acute immune-mediated peripheral polyneuropathy. Neuroimaging findings from patients with this syndrome have revealed gadolinium enhancement in the cauda equina and in the anterior and posterior nerve roots, but intra-spinal lesions have never been described.
Herein, we report, for the first time, bilateral spinal anterior horn lesions in a patient with an acute motor axonal neuropathy form of Guillain-Barré syndrome.
The patient was a previously healthy 13-year-old Japanese girl, who exhibited acute-onset flaccid tetraplegia and loss of tendon reflexes.
Nerve conduction studies revealed motor axonal damage, leading to the diagnosis of acute motor axonal neuropathy. Notably, spinal magnetic resonance imaging revealed bilateral anterior horn lesions on T2-weighted imaging at the Th11-12 levels, as well as gadolinium enhancement of the cauda equina and anterior and posterior nerve roots. The anterior horn lesions were most prominent on day 18, and their signal intensity declined thereafter. Although intravenous treatment with immunoglobulins was immediately administered, the motor function was not completely regained.
We propose that anterior spinal lesions might be responsible for the prolonged neurological disability of patients with Guillain-Barré syndrome, possibly produced by retrograde progression from the affected anterior nerve roots to the intramedullary roots, and the anterior horn motor neurons.
吉兰-巴雷综合征是一种急性免疫介导的周围性多发性神经病。该综合征患者的神经影像学检查结果显示马尾、前后神经根有钆增强，但从未描述过脊髓内病变。
在此，我们首次报告1例急性运动轴索性神经病型吉兰-巴雷综合征患者出现双侧脊髓前角病变。
该患者是一名此前健康的13岁日本女孩，表现为急性起病的弛缓性四肢瘫和腱反射消失。
神经传导研究显示运动轴索损伤，导致诊断为急性运动轴索性神经病。值得注意的是，脊髓磁共振成像在T2加权像上显示胸11-12水平双侧前角病变，以及马尾、前后神经根钆增强。前角病变在第18天最为明显，此后信号强度下降。尽管立即给予静脉注射免疫球蛋白治疗，但运动功能未完全恢复。
我们认为脊髓前部病变可能是吉兰-巴雷综合征患者神经功能残疾持续时间延长的原因，可能是由受影响的前神经根向髓内神经根及前角运动神经元逆行进展所致。
