Boscarino Marilisa, Lanzone Jacopo, Ricci Lorenzo, Tombini Mario, Di Lazzaro Vincenzo, Assenza Giovanni
Unit of Neurology, Neurophysiology, Neurobiology, Department of Medicine, University Campus Bio-Medico of Rome, via Álvaro del Portillo, 21, 00128 Rome, Italy.
Brain Sci. 2020 May 8;10(5):282. doi: 10.3390/brainsci10050282.
Anti-Myelin Associated Glycoprotein (anti-MAG) neurological involvement classically manifests as a peripheral neuropathy with prominent sensitive symptoms. We describe a case report of a patient with positive anti-MAG antibodies presenting with clinical and neurophysiological evidence of spinal cord impairment. A 69-year-old woman came to our attention with subacute onset of dysesthesias at lower limbs and ataxia. Blood routine tests and hematological work-up led to a diagnosis of monoclonal gammopathy of undetermined significance. High titers of anti-MAG antibodies was revealed (34,594.70 BTU/ml, normal range 0-1000). Nerve conduction studies (NCS) ruled out a polyneuropathy at lower limbs. Somatosensory evoked potentials (SSEPs) showed prolonged central conduction time (CTT) at lower limbs, suggesting a dorsal column damage. Brain and spinal cord Magnetic Resonance Imaging (MRI) did not reveal any significant lesion. Analysis of cerebrospinal fluid (CSF) evidenced an albumin-cytologic dissociation. She was treated with corticosteroids with temporary remission of sensory symptoms and normalization of CTT. Subsequently, she developed a multineuropathy which was successfully treated with Rituximab. We discuss the potential role of anti-MAG antibodies in the pathophysiology of dorsal column impairment and the clinical usefulness of SSEPs in monitoring the evolution of anti-MAG neuropathy.
抗髓鞘相关糖蛋白(抗-MAG)神经受累通常表现为伴有明显感觉症状的周围神经病。我们报告一例抗-MAG抗体阳性患者,其具有脊髓损伤的临床和神经生理学证据。一名69岁女性因下肢感觉异常和共济失调亚急性起病前来就诊。血常规检查和血液学检查诊断为意义未明的单克隆丙种球蛋白病。检测发现抗-MAG抗体滴度很高(34,594.70 BTU/ml,正常范围0-1000)。神经传导研究(NCS)排除了下肢多发性神经病。体感诱发电位(SSEPs)显示下肢中枢传导时间(CTT)延长,提示后索损伤。脑和脊髓磁共振成像(MRI)未发现任何明显病变。脑脊液(CSF)分析显示蛋白细胞分离。给予她皮质类固醇治疗后,感觉症状暂时缓解,CTT恢复正常。随后,她发展为多发性神经病,使用利妥昔单抗治疗成功。我们讨论了抗-MAG抗体在背柱损伤病理生理学中的潜在作用以及SSEPs在监测抗-MAG神经病进展中的临床应用价值。