Eosinophilic Granulomatosis with Polyangiitis Presented as Acute Polyneuropathy and Cerebral Vasculitis.
- Author:
Il Han YOO
1
;
Sang Tae CHOI
;
Seong Ho CHOI
;
Jeong Min KIM
;
Suk Won AHN
Author Information
- Publication Type:Case Report
- Keywords: Eosinophilic granulomatosis with polyangiitis; Churg-Strauss syndrome; EGPA; vasculitic neuropathy; cerebral vasculitis; Guillain-Barre syndrome
- MeSH: Asthma; Brain; Caudate Nucleus; Cerebral Infarction; Cerebrospinal Fluid; Churg-Strauss Syndrome; Cyclophosphamide; Diagnosis; Diffusion Magnetic Resonance Imaging; Eosinophilia; Eosinophils*; Extremities; Fever; Gadolinium; Gait; Granulomatosis with Polyangiitis*; Guillain-Barre Syndrome; Humans; Magnetic Resonance Imaging; Middle Aged; Muscle Weakness; Neural Conduction; Peripheral Nervous System Diseases; Polyneuropathies*; Sensation; Vasculitis; Vasculitis, Central Nervous System*
- From:Experimental Neurobiology 2017;26(3):168-171
- CountryRepublic of Korea
- Language:English
- 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.
