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Acute Polyradiculomyelitis With Spinal Cord Gray Matter Lesions: A Report of Two Cases.
Frontiers in Neurology ( IF 2.7 ) Pub Date : 2021-08-19 , DOI: 10.3389/fneur.2021.721669
Charidimos Tsagkas 1 , Maria Janina Wendebourg 1 , Matthias Mehling 1 , Johannes Lorscheider 1 , Philippe Lyrer 1 , Bernhard Friedrich Décard 1
Affiliation  

Objective: Inflammatory polyradiculomyelitis belongs to a rare group of immune-mediated diseases affecting both the central and peripheral nervous system. We aimed to describe an unusual presentation of acute polyradiculomyelitis with marked spinal cord lesions restricted to the gray matter. Methods: Thorough examination of two case reports including clinical, MRI, serologic, electrophysiologic and CSF examinations as well as short-term follow-up. Results: We present two adult patients with acute polyradiculomyelitis and unusual spinal cord lesions restricted to the gray matter on MRI. The clinical presentation, serologic, electrophysiologic and CSF features of the two patients varied, whereas both patients demonstrated severe, asymmetrical, predominantly distal, motor deficits of the lower extremities as well as bladder and bowel dysfunction. Both patients only partially responded to anti-inflammatory treatment. Severe motor impairment and bladder dysfunction persisted even months after symptom onset. Conclusions: To our best of knowledge, these are the first reports of acute polyradiculomyelitis with distinct involvement of the lower thoracic spinal cord gray matter. Currently, it remains unclear whether gray matter lesions reflect a separate pathophysiologic mechanism or an exceedingly rare presentation of spinal cord involvement in acute polyradiculomyelitis.

中文翻译:

伴有脊髓灰质病变的急性多发性脊髓灰质炎:两例报告。

目的:炎性多发性神经根脊髓炎属于一组罕见的免疫介导疾病,可影响中枢和外周神经系统。我们旨在描述急性多发性神经根脊髓炎的不寻常表现,其明显的脊髓病变仅限于灰质。方法:对两份病例报告进行全面检查,包括临床、MRI、血清学、电生理和脑脊液检查以及短期随访。结果:我们介绍了两名患有急性多发性神经根脊髓炎的成年患者,并且在 MRI 上显示了仅限于灰质的异常脊髓病变。两名患者的临床表现、血清学、电生理学和脑脊液特征各不相同,而两名患者均表现出严重的、不对称的、主要是远端的下肢运动障碍以及膀胱和肠道功能障碍。两名患者仅对抗炎治疗有部分反应。甚至在症状出现数月后,严重的运动障碍和膀胱功能障碍仍然存在。结论:据我们所知,这些是急性多发性神经根脊髓炎的首次报告,其明显累及下胸段脊髓灰质。目前,尚不清楚灰质病变是否反映了单独的病理生理机制或急性多发性神经根脊髓炎中脊髓受累的极其罕见的表现。
更新日期:2021-08-19
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