A Rare Cause of Polyneuropathy : Churg Strauss Syndrome
Articles > Article · 2016
Authors: Ceren ENUSTUN, Figen GÖKÇAY, Kenan AKSU, Aysen Suzen EKINCI
JOURNAL OF NEUROLOGICAL SCIENCES-TURKISH, Journal Indexed in SCI Expanded, International, 2016, vol 33, pp 373-377
DOI: 10.1055/s-2000-8073
Summary
Churg Strauss Syndrome is a systemic necrotising vasculitis affecting small and medium sized vessels. The neuropathy may occur in 60-70% of cases. In this study, we present a 60-year-old female patient resistant to conventional immunosuppressants. Over the past year she has been followed with the diagnosis of astma bronchiale and she had purpuric lesions in both legs. Her test results showed eosinoplilia, Paranasal sinus BT showed mucosal thickening in favor of chronic sinusitis, perivascular infiltration of eosinophils were present in the skin biopsy. Axonal sensorymotor polyneuropathy was determined in first electrophysiologic evaluation. Levels of total serum IgE were high. Although pulse of prednisolone 1gr/day treatment combined with 750 mg cyclophosphamide was given, muscle weakness progressed on right hand and left leg and she had remarkable atrophy of interosseous muscle of right hand. Nerve conduction studies revealed mononeuropathy multiplex (asymmetric axonal sensorimotor neuropathy). The patient, who was considered refractory to cyclophosfamide therapy, has started using rituximab. Two months later, regeneration was observed in needle electromyography. In this case, we aim to provide the clinical, laboratory and electrophysiological findings with CSS who has responsed well to treatment with rituximab after conventional medical treatment has failed.