Department of neurology, Jiangxi Provincial People's Hospital, The First Affiliated Hospital of Nanchang Medical College, Department of Neurology, Xiangya Hospital, Central South University, Jiangxi, National Regional Center for Neurological Diseases, Nanchang, 330000, Jiangxi, China.
Department of Neurology, Xiangya Hospital Central South University, Changsha, 410000, Hunan, China.
BMC Neurol. 2024 Sep 5;24(1):320. doi: 10.1186/s12883-024-03798-7.
Neurolymphomatosis (NL) is a rare neurologic manifestation of non-Hodgkin lymphoma (NHL) with poor prognosis. Investigations including MRI, PET/CT, nerve biopsy and cerebrospinal fluid (CSF) analysis can aid the diagnosis of NL. In this study, we presented a case of NL with co-existing myelin-associated glycoprotein (MAG) antibody. The patient first presented with symptoms of peripheral neuropathy involving multiple cranial nerves and cauda equina, and later developed obstructive hydrocephalus and deep matter lesions. He also had persistently positive MAG antibody, but did not develop electrophysiologically proven neuropathy and monoclonal immunoglobulin. The final brain biopsy confirmed diffuse large B cell lymphoma.
神经淋巴病(NL)是非霍奇金淋巴瘤(NHL)的一种罕见神经系统表现,预后较差。磁共振成像(MRI)、正电子发射断层扫描(PET)/计算机断层扫描(CT)、神经活检和脑脊液(CSF)分析等检查有助于 NL 的诊断。本研究报道了一例伴有髓鞘相关糖蛋白(MAG)抗体的 NL 病例。该患者首先表现为多颅神经和马尾神经受累的周围神经病症状,随后出现梗阻性脑积水和深部实质病变。他还持续存在 MAG 抗体阳性,但未出现电生理证实的神经病和单克隆免疫球蛋白。最终的脑活检证实为弥漫性大 B 细胞淋巴瘤。