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Título

Case report of non-systemic vasculitic neuropathy with improvement after therapy with methylprednisolone and cyclophosphamide

RESUMO

Vasculitic neuropathies are a heterogeneous and rare group of autoimmune disorders of the peripheral nerves. In primary vasculitic neuropathies, there is no clinical evidence of involvement of other organs. They usually have a multifocal distribution and develop acutely or subacutely. The most commonly affected nerves are the common peroneal nerve in the leg and the ulnar nerve in the arm. Electromyography reveals a predominantly axonal, asymmetric sensory and motor neuropathy (multiple mononeuropathies). Immunosuppression is the cornerstone of disease-modifying therapy. We describe the case of a 37-year-old patient with tingling paresthesia, shock-like pain, and paresis in the right hand, presenting after two weeks a similar picture in the topography of the left foot. She is initially evaluated by orthopedics and rheumatology for carpal tunnel syndrome. Neurological evaluation characterized multiple mononeuropathies. In a complementary investigation, asymmetric sensory and motor neuropathy of axonal nature and with signs of activity was confirmed, considering the possibility of vasculitic neuropathy. It was carried out an extensive investigation of the causes of systemic vasculitis, with negative results. The biopsy of the left sural nerve performed revealed a normal histological and immunohistochemical appearance. It was decided to perform therapy with methylprednisolone and cyclophosphamide due: first, to worsening of sensory and motor function, and second, a sustained increase in inflammatory tests. Excellent response with this treatment has been obtained. The importance of this case report lies in the fact that non-systemic vasculitic neuropathy is a challenging diagnosis. Usually, it is hard to perform a nerve biopsy. The adequate response to the instituted therapy reaffirms the relevance of the clinical evaluation to determine the beginning of the therapy, despite the negativity to the performed biopsy.

Palavras Chave

Área

Neuropatias Periféricas