Kalaszi Marianna, Donlon Eoghan, Ahmad Marzuki Wan, Mohamed Abdirahman Sheikh, Boers Peter
Department of Neurology, University Hospital Limerick, Limerick, Ireland.
Front Neurol. 2022 Sep 13;13:905283. doi: 10.3389/fneur.2022.905283. eCollection 2022.
Longitudinally extensive spinal cord lesions are challenging diagnostic entities as they are uncommon, but various etiologies can cause them.
We report a case of a 55-year-old man with a past medical history of hypertension. He is an ex-smoker. He presented with chest pain, followed by right lower limb weakness, preceded by 2 weeks of constipation and voiding dysfunction. The examination revealed right lower limb mild flaccid paresis, absent reflexes, reduced anal tone, and urinary retention. His symptoms deteriorated over 24 h, and he developed severe flaccid paraparesis with impaired pinprick sensation below the T4 level. MRI spine showed an abnormal, non-enhancing signal in the anterior aspect of the spinal cord extending from the T4 level to the conus without associated edema. He was commenced on intravenous steroids and had significant improvement after one dose. The imaging was felt to be consistent with spinal cord infarction, and aspirin was started. The cerebrospinal fluid analysis showed elevated protein (0.8 mg/ml). Investigations for stroke and autoimmune pathologies were negative. The Lyme immunoblot confirmed intrathecal production of IgG to Borrelia antigens. The patient was started on ceftriaxone. The paraneoplastic screen identified amphiphysin antibodies. CT-TAP and PET-CT did not identify occult malignancy. The patient had a significant improvement over 2 months, strength was almost fully recovered, and autonomic functions returned to normal.
We describe an unusual steroid-responsive, longitudinally extensive spinal cord lesion with radiological features of spinal cord infarct and a simultaneous finding of intrathecal Lyme antibodies and serum amphiphysin antibodies.
纵向广泛脊髓病变是具有挑战性的诊断实体,因为它们不常见,但多种病因可导致此类病变。
我们报告一例55岁男性,有高血压病史。他已戒烟。他最初表现为胸痛,随后出现右下肢无力,在此之前有2周的便秘和排尿功能障碍。检查发现右下肢轻度弛缓性轻瘫、反射消失、肛门张力降低及尿潴留。其症状在24小时内恶化,发展为严重的弛缓性截瘫,T4水平以下针刺觉减退。脊柱MRI显示脊髓前部从T4水平至圆锥有异常的无强化信号,无相关水肿。给予静脉注射类固醇治疗,一剂后病情有显著改善。影像学表现被认为与脊髓梗死相符,开始服用阿司匹林。脑脊液分析显示蛋白升高(0.8mg/ml)。卒中及自身免疫性疾病相关检查均为阴性。莱姆免疫印迹证实鞘内产生针对伯氏疏螺旋体抗原的IgG。患者开始使用头孢曲松治疗。副肿瘤筛查发现抗 amphiphysin 抗体。CT-TAP和PET-CT未发现隐匿性恶性肿瘤。患者在2个月内有显著改善,肌力几乎完全恢复,自主神经功能恢复正常。
我们描述了一例不寻常的对类固醇有反应的纵向广泛脊髓病变,具有脊髓梗死的影像学特征,同时发现鞘内莱姆抗体和血清抗 amphiphysin 抗体。