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一例颅多神经炎:从发病到原发性神经淋巴瘤的诊断

A case of cranial multinevritis: from the onset to the diagnosis of primary neurolymphomatosis.

作者信息

Vecchio Domizia, Mittino Daniela, Terazzi Emanuela, Nassi Luca, Conconi Annarita, Monaco Francesco

机构信息

Neurological Clinic, University of Eastern Piedmont and ‘Maggiore della Carità’ Hospital, Novara, Italy.

出版信息

BMJ Case Rep. 2012 Jun 14;2012:bcr0620114299. doi: 10.1136/bcr.06.2011.4299.

Abstract

Neurolymphomatosis (NL) is a rare peripheral or cranial neuropathy caused by non-Hodgkin's lymphoma (NHL). Diagnosis is often delayed and prognosis is poor. The authors described a woman in her 70s with a facial left peripheral palsy, complete right abducent palsy, left hypoacusia and balance deficit. Then she presented with low progressive hyposthenia at four limbs and cognitive impairment, sudden facial right peripheral palsy and complete left abducent palsy. The authors performed brain and spinal MRI, cerebrospinal fluid (CSF) analysis and extensive haematological examinations for infections, autoimmune and neoplastic diseases. All the results were not diagnostic. Only repeating for the third time a spinal tap, CSF presented neoplastic B cells suggestive for large B-NHL. The authors diagnosed primary NL. The patient was treated with R-CHOP but she died 2 months later. In front of rapidly progressive neuropathy, a NL has to be considered performing different examinations, especially and repeating them after a short period.

摘要

神经淋巴瘤(NL)是一种由非霍奇金淋巴瘤(NHL)引起的罕见的周围性或颅神经病变。诊断往往延迟,预后较差。作者描述了一名70多岁的女性,患有左侧面部周围性麻痹、右侧展神经完全麻痹、左侧听力减退和平衡障碍。随后她出现四肢进行性肌无力减退和认知障碍,突发右侧面部周围性麻痹和左侧展神经完全麻痹。作者进行了脑部和脊髓MRI、脑脊液(CSF)分析以及针对感染、自身免疫和肿瘤性疾病的广泛血液学检查。所有结果均无诊断意义。仅在第三次重复腰椎穿刺时,脑脊液中出现了提示大B细胞NHL的肿瘤性B细胞。作者诊断为原发性NL。患者接受了R-CHOP方案治疗,但2个月后死亡。面对快速进展的神经病变,必须考虑NL并进行不同的检查,尤其是在短时间后重复检查。

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