Dados do Trabalho

Título

Mononeuropathy multiplex: a 100-case series

Resumo

Introduction
Mononeuropathy multiplex is a group of uncommon disorders. Definition is involvement of two or more noncontiguous peripheral nerves. National studies are needed to understand local epidemiology, as numerous causes have been identified.
Methods
We retrospectively reviewed data of patients with an electrodiagnostic diagnosis of mononeuropathy multiplex. Etiology was divided as systemic vasculitis, isolated peripheral nerve vasculitis, chronic inflammatory neuropathy, infection (including leprosy), genetic, neoplastic disorders, and undetermined. Electrodiagnostic pattern was classified as axonal, demyelinating, intermediated (mixed), and associated pattern (clearly demyelinating lesions in some nerves and axonal lesion in others). When available, tissue biopsy was also reviewed.
Results:
A total of 100 patients were included – mean age was 47,4 years, 48 were male. Most frequent etiology was systemic vasculitis with 51 patients. Among these, lupus was the most common diagnosis, but rarer vasculitis that are seldom associated with mononeuritis multiplex were also found. Isolated peripheral vasculitis was seen in three patients. Leprosy was the most frequent infection with 28 patients, only two presenting with pure neural leprosy. Chronic inflammatory neuropathy was represented by three cases of multifocal CIDP and 03 cases of multifocal motor neuropathy. Genetic was represented by two cases of HNPP. EDX was consistent with an axonal pattern in 70 cases, demyelinating in 12, intermediated in 13, associated in five. Entrapment neuropathies pointed towards an infectious etiology. A total of 17 nerve biopsies, 44 skin biopsies, three salivary gland biopsies, and seven renal biopsies were available to evaluation. Histology strongly suggested vasculitis in 33 biopsies, and 19 biopsies were diagnostic of leprosy.
Conclusion:
Facing a mononeuropathy multiplex, systemic vasculitis is the most frequent diagnosis. Very rare vasculitis types should also be considerate. In our population, leprosy should always be a concern in asymmetric neuropathy. Although a diagnostic challenge, an etiology can be clearly identified in majority of patients. Non neural tissue biopsies can be very helpful with much less distress.

Palavras Chave

Mononeuritis Multiplex, Electromyography, Peripheral Nerves, Vasculitis, Leprosy,