Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) presenting with polyneuropathy--a case series. J Clin Neuromuscul Dis 2015 Mar;16(3):125-30
Date
02/20/2015Pubmed ID
25695917DOI
10.1097/CND.0000000000000068Scopus ID
2-s2.0-84924158121 (requires institutional sign-in at Scopus site) 9 CitationsAbstract
OBJECTIVES: We aim to characterize a group of patients with eosinophilic granulomatosis with polyangiitis (EGPA) with an initial presentation of peripheral neuropathy.
METHODS: A retrospective analysis of 11 patients with EGPA.
RESULTS: The most common chief complaint was neuropathic limb pain and numbness (100%), followed by extremity weakness (82%). Nine (82%) patients had acute to subacute onset. All patients had a history of asthma and serum eosinophilia of more than 11%. Combining clinical and electrophysiological data, 10 (91%) patients demonstrated notable asymmetric involvement, whereas 1 patient presented with a length-dependent symmetrical axonal polyneuropathy. All patients improved significantly after immunotherapy, with average time to improvement of 9 weeks. Ten (91%) patients improved steadily and experienced no relapse, whereas 1 relapsed within the first year.
CONCLUSIONS: EGPA should be suspected in patients with asymmetric axonal peripheral neuropathy associated with asthma and eosinophilia. Early diagnosis and aggressive treatment help to achieve favorable outcome.
Author List
Santos-Pinheiro F, Li YMESH terms used to index this publication - Major topics in bold
EosinophiliaFemale
Follow-Up Studies
Granulomatosis with Polyangiitis
Humans
Immunosuppressive Agents
Male
Middle Aged
Neurologic Examination
Polyneuropathies
Retrospective Studies