Severe polyneuropathy in a patient with Churg-Strauss syndrome

We describe the clinicopathologic features of a 56-year-old woman affected with Churg-Strauss syndrome with major peripheral nerve involvement. The pa tient presented with a 1-month history of mainly distal upper-limb symmetri cal paresthesias and hypostenia (bilateral "wrist drop"), palpable purpura and eosinophilia. Multiple pulmonary infiltrates and asthma had been presen t since the age of 52. Skin biopsy demonstrated an eosinophilic necrotizing vasculitis. During the hospitalization she was submitted to cardiac, bronc hopulmonary, renal, and gastrointestinal evaluation and EMG. Peripheral ner ve and skeletal muscle biopsies were performed. Sural nerve biopsy showed a marked degree of demyelination. A perivascular cellular infiltrate within the epineurium was immunoreactive for T lymphocytes and macrophages. Strong HLA-DR immunostaining was present in the endoneurium. IgM, IgE and fibrino gen deposition was found in some epi-and endoneurial vessels. Muscle biopsy showed neurogenic changes and 1 thrombosed vessel surrounded by mononuclea r cells. Membrane attack complex (MAC) deposition was present in a few capi llaries and major histocompatibility complex products I (MHCP I) was expres sed at the subsarcolemmal level in a few isolated perivascular muscle fiber s. After immunosuppressive therapy, the patient showed progressive improvem ent of both clinical symptoms and neurophysiological parameters.