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Peripheral nerve neurolymphomatosis: Clinical features, treatment, and outcomes
Author(s) -
Keddie Stephen,
Nagendran Arjuna,
Cox Tom,
Bomsztyk Joshua,
Jaunmuktane Zane,
Brandner Sebastian,
Manji Hadi,
Rees Jeremy H.,
Ramsay Alan D.,
Rossor Alexander,
D'Sa Shirley,
Reilly Mary M.,
Carr Aisling S.,
Lunn Michael P.
Publication year - 2020
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.27045
Subject(s) - medicine , nerve biopsy , lymphoma , biopsy , peripheral neuropathy , radiation therapy , magnetic resonance imaging , bone marrow , radiology , neuropathology , pathology , disease , endocrinology , diabetes mellitus
This series characterises nine patients with neurohistopathologically proven peripheral nerve neurolymphomatosis. Methods: 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. Discussion: 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.