Tsagkas Charidimos, Wendebourg Maria Janina, Mehling Matthias, Lorscheider Johannes, Lyrer Philippe, Décard Bernhard Friedrich
Neurology Clinic and Policlinic, Departments of Medicine, Clinical Research and Biomedical Engineering, University Hospital Basel and University of Basel, Basel, Switzerland.
Front Neurol. 2021 Aug 19;12:721669. doi: 10.3389/fneur.2021.721669. eCollection 2021.
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. Thorough examination of two case reports including clinical, MRI, serologic, electrophysiologic and CSF examinations as well as short-term follow-up. 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. 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.
炎性多神经根脊髓炎属于一类罕见的免疫介导疾病,可累及中枢和周围神经系统。我们旨在描述急性多神经根脊髓炎的一种不寻常表现,其脊髓病变明显,且局限于灰质。对两例病例报告进行了全面检查,包括临床、磁共振成像(MRI)、血清学、电生理和脑脊液检查以及短期随访。我们报告了两名成年急性多神经根脊髓炎患者,MRI显示其脊髓病变不寻常,局限于灰质。两名患者的临床表现、血清学、电生理和脑脊液特征各不相同,但均表现为严重的、不对称的、以下肢远端为主的运动功能障碍以及膀胱和肠道功能障碍。两名患者对抗炎治疗均仅部分有效。即使在症状出现数月后,严重的运动障碍和膀胱功能障碍仍然存在。据我们所知,这些是首次关于急性多神经根脊髓炎伴下胸段脊髓灰质明显受累的报告。目前,尚不清楚灰质病变是反映了一种独立的病理生理机制,还是急性多神经根脊髓炎中脊髓受累的一种极其罕见的表现。