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Treatable, motor‐sensory, axonal neuropathies with C5b‐9 complement on endoneurial microvessels
Author(s) -
Trikamji Bhavesh,
Pestronk Alan
Publication year - 2021
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.27155
Subject(s) - medicine , axon , nerve biopsy , pathology , weakness , biopsy , superficial peroneal nerve , radial nerve , sensory loss , sensory nerve , sensory system , diabetes mellitus , surgery , peripheral neuropathy , anatomy , endocrinology , neuroscience , biology , ankle
Identification and treatment of immune‐mediated polyneuropathies may lead to improved strength and function. We studied the clinical and laboratory features, and treatment response, in patients with motor‐sensory axonal polyneuropathies who were found to have C5b‐9 complement staining on endoneurial microvessels. Methods Retrospective review of 16 consecutive adults with motor‐sensory axonal polyneuropathies who were then found to have C5b‐9 staining of endoneurial microvessels on nerve biopsy, and subsequently treated with intravenous corticosteroids (1 g methylprednisolone for 5 consecutive days, and then weekly). Strength measurements were done using quantitative handheld dynamometry. Nerve biopsy analysis included frozen and fixed tissue. Results Patients (mean onset age, 59 ± 4 years; range, 34‐83 years; 12 of 16 were males; 9 of 16 had diabetes) had progressive (median duration, 2 years), asymmetric, distal weakness, in the lower extremities (16 of 16) and/or upper extremities (7 of 16), and panmodal sensory loss. Electrodiagnostic studies showed axon loss. Nerve pathology showed abnormal C5b‐9 staining on endoneurial microvessels. Axon loss was present in all nerves, often varied among fascicles. Inflammation was uncommon. Distal strength usually improved (mean improvement of 34 ± 6% of normal strength; P = .0003) with corticosteroid treatment. Discussion Motor‐sensory axonal polyneuropathies having noninflammatory, humoral immune pathology with C5b‐9 staining of endoneurial microvessels (HIEM) frequently manifest progressive asymmetric, distal, lower extremity with or without upper extremity weakness that improves rapidly during corticosteroid treatment. HIEM may represent a new class of noninflammatory‐vasculopathic, treatable axonal motor‐sensory neuropathies.