Treatable, motor-sensory, axonal neuropathies with C5b-9 complement on endoneurial microvessels

Introduction:  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.