Exp Neurobiol.  2017 Jun;26(3):168-171. 10.5607/en.2017.26.3.168.

Eosinophilic Granulomatosis with Polyangiitis Presented as Acute Polyneuropathy and Cerebral Vasculitis

• Yoo IH
• Choi ST
• Choi SH
• Kim JM
• Ahn SW

• KMID: 2383786
• DOI: http://doi.org/10.5607/en.2017.26.3.168

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.

Keyword

Eosinophilic granulomatosis with polyangiitis; Churg-Strauss syndrome; EGPA; vasculitic neuropathy; cerebral vasculitis; Guillain-Barre syndrome

MeSH Terms

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*
Cyclophosphamide
Gadolinium