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抗髓鞘相关糖蛋白神经病中的脊髓损伤:来自体感诱发电位的证据

Spinal Cord Impairment in Anti-Mag Neuropathy: Evidence from Somatosensory Evoked Potentials.

作者信息

Boscarino Marilisa, Lanzone Jacopo, Ricci Lorenzo, Tombini Mario, Di Lazzaro Vincenzo, Assenza Giovanni

机构信息

Unit of Neurology, Neurophysiology, Neurobiology, Department of Medicine, University Campus Bio-Medico of Rome, via Álvaro del Portillo, 21, 00128 Rome, Italy.

出版信息

Brain Sci. 2020 May 8;10(5):282. doi: 10.3390/brainsci10050282.

Abstract

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.

摘要

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。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/88c3/7287849/044be2c47110/brainsci-10-00282-g001.jpg

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