Churg-Strauss syndrome

The neurologic manifestations of systemic necrotizing vasculitis are heterogeneous and often complex. Late onset asthma, systemic vasculitis manifest by mononeuritis multiplex, and peripheral eosinophilia-the triad of which is consistent with Churg-Strauss syndrome-developed in a 59-year-old woman. Electromyography and nerve conduction studies showed both myelinating and axonal neuropathy involving multiple nerves. Muscle and nerve biopsy showed neurogenic atrophy, demyelination, and axonal changes involving the sural nerve and inflammatory necrotizing small and medium vessel arterial changes. The patient was treated with high-dose prednisone and improved rapidly. Unusual in this case was the lack of multisystemic involvement. Churg-Strauss syndrome is a disease that is being recognized with increased clinical frequency and responds quickly to methylprednisolone treatment.