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Peripheral nerve neurolymphomatosis: Clinical features, treatment, and outcomes.
Muscle & Nerve ( IF 3.4 ) Pub Date : 2020-08-12 , DOI: 10.1002/mus.27045
Stephen Keddie 1, 2 , Arjuna Nagendran 1 , Tom Cox 1 , Joshua Bomsztyk 3 , Zane Jaunmuktane 4 , Sebastian Brandner 4 , Hadi Manji 1 , Jeremy H Rees 5 , Alan D Ramsay 6 , Alexander Rossor 1 , Shirley D'Sa 3 , Mary M Reilly 1 , Aisling S Carr 1 , Michael P Lunn 1, 2
Affiliation  

This series characterises nine patients with neurohistopathologically proven peripheral nerve neurolymphomatosis. A search of the hospital neuropathology database from 2002 to 2019 identified biopsy proven cases. Clinical data, investigation modalities, treatments, and outcomes were collated. Median age at neuropathy onset was 47 y, the neuropathy commonly as the initial lymphoma disease manifestation. Most (8/9) presented with painful asymmetrical sensory disturbance, with additional cranial nerve involvement in three. Neurophysiology typically demonstrated multiple axonal mononeuropathies. Cerebrospinal fluid protein was often raised (6/8). Magnetic resonance imaging suggested peripheral nerve infiltration in 6/9 and positron emission tomography CT in 4/9. Bone marrow biopsy was abnormal in 6/8. Treatment involved systemic or intrathecal chemotherapy and radiotherapy. Median survival was 23 mo. Neurolymphomatosis is a rare but important cause of neuropathy, particularly in those lacking systemic evidence of lymphoma as correct aggressive treatment can prolong survival. Nerve biopsy is essential to classify lymphoma type and rule out alternatives.

中文翻译:

周围神经神经淋巴瘤病:临床特征,治疗和结局。

该系列的特点是九名神经组织病理学证实的周围神经神经淋巴瘤病患者。从2002年至2019年对医院神经病理学数据库进行的搜索确定了活检证实的病例。临床资料,研究方式,治疗和结果进行了整理。神经病发作的中位年龄为47岁,通常以神经病为最初的淋巴瘤疾病表现。大多数(8/9)表现为疼痛性不对称感觉障碍,其中三例伴有颅神经受累。神经生理学通常表现出多种轴索性单神经病。脑脊液蛋白经常升高(6/8)。磁共振成像显示6/9显示周围神经浸润,4/9显示正电子发射断层扫描CT。6/8的骨髓活检异常。治疗涉及全身或鞘内化疗和放疗。中位生存期为23 mo。神经淋巴瘤病是神经病的一种罕见但重要的原因,尤其是在那些缺乏全身证据的淋巴瘤患者中,因为正确的积极治疗可以延长生存期。神经活检对于分类淋巴瘤类型和排除替代方法至关重要。
更新日期:2020-10-19
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