Articles

Article

Case Report

Exp Neurobiol 2017; 26(3): 168-171

Published online June 30, 2017

https://doi.org/10.5607/en.2017.26.3.168

© The Korean Society for Brain and Neural Sciences

Eosinophilic Granulomatosis with Polyangiitis Presented as Acute Polyneuropathy and Cerebral Vasculitis

Il-Han Yoo1, Sang Tae Choi2, Seong-Ho Choi3, Jeong-Min Kim1 and Suk-Won Ahn1*

1Department of Neurology, Chung-Ang University Hospital, Chung-Ang University College of Medicine, Seoul 06973, 2Division of Rheumatology, Department of Internal Medicine, Chung-Ang University Hospital, Chung-Ang University College of Medicine, Seoul 06973, 3Division of Infectious Diseases, Department of Internal Medicine, Chung-Ang University Hospital, Chung-Ang University College of Medicine, Seoul 06973, Korea

Correspondence to: *To whom correspondence should be addressed.
TEL: 82-2-6299-3153, FAX: 82-2-6299-3153
e-mail: icandr@hanmail.net

Received: April 1, 2017; Revised: May 28, 2017; Accepted: June 5, 2017

Abstract

Eosinophilic granulomatosis with polyangiitis (EGPA) is an immune related systemic disease that is caused by vasculitis affecting multiple organ systems. It is characterized by asthma, fever, eosinophilia, cardiac problems, renal injury, and peripheral neuropathy. In this report, we describe a patient with EGPA with concurrent cerebral infarction and acute polyneuropathy mimicking a Guillain-Barre syndrome (GBS). A 46-year-old man presented with rapidly progressing gait disturbance, muscular weakness, and tingling sensation in all four limbs. A nerve conduction study revealed sensorimotor polyneuropathy in all four limbs, and a test of the cerebrospinal fluid showed an albumin-cytologic dissociation. In addition, brain magnetic resonance imaging (MRI) using fluid-attenuated inversion recovery and diffusion weighted MRI revealed high signal intensity lesions with gadolinium enhancement on T1-weighted MRI in the right caudate nucleus. After performing laboratory tests, paranasal sinus computed tomography, and a nasal smear, the patient was diagnosed with EGPA and treated with high dose glucocorticoid and oral cyclophosphamide. In conclusion, our findings indicate that a diagnosis of EGPA should be considered when a patient presents with rapidly progressing polyneuropathy mimicking a GBS along with unusual systemic symptoms or brain lesions.

Keywords: Eosinophilic granulomatosis with polyangiitis, Churg-Strauss syndrome, EGPA, vasculitic neuropathy, cerebral vasculitis,
Guillain-Barre syndrome