Peripheral Neuropathy as Initial Manifestation of Churg-Strauss Syndrome

Abstract

Mild deficit of proximal strength in the lower limbs. Not sensory levels. He was able to walk on points and heels. Laboratory examination revealed white blood count 28.5 × 10 9 (eosinophils 61%); C-reactive protein level was 62 mg/dL (normally 0.0-5.0 mg/dL); creatine kinase and transaminase levels was increased, respectively 62 mg/dL and 93 mg/dL. Chest x-ray and head computed tomography scan were normal. The patient was admitted in the neurological ward. Blood exams confirmed leukocytosis with hypereosinophilia. Serum tests were positive for ANCA. Nerve conduction studies showed a neurogenic suffering of the investigated muscles, compatible with axonal motor nerve distress with blocks of motor conduction and sensory axonal suffering with asymmetric distribution; these findings were suggestive of multiplex mononeuritis. Nerve biopsy showed features of axonopathy. Muscle biopsy showed a picture with features of eosinophilic vasculitis. Spirometry was normal, with a FEV1/FVC rate of 77% of predicted. He was treated with intravenous methyl-prednisolone and Cyclophosphamide, followed by maintenance with azathioprine. After some days of therapy a blood exam control showed: White blood count 20.5 × 10 9 (eosinophils 5%); C-reactive protein level 5 mg/dL; creatine kinase levels 51 mg/dL, and transaminase levels 30 mg/dL. Patient improved and there was no recurrence of symptoms since about two years of follow-up.