Churg-Straus syndrome: A case report

Churg-Strauss syndrome is a rare autoimmune disorder characterized by excess circulating, tissue eosinophils, and vasculitis, which affects the lung and skin. The syndrome occurs in patients with a history of asthma or allergy.1 Neuropathy develops in approximately 3/4 of the patients usually as mononeuritis multiplex. Centrally accentuated antineutrophil cytoplasmic antibody (CANCA) is generally found in more than half of the cases, while central nervous system (CNS) manifestations are relatively unusual and include headache, convulsion, hemiplegia, and brainstem signs.2 We report here a 42-year-old man with a history of severe asthma and rhinitis in the past 4 years prior to the first admission. The patient was presented with difficulty in walking and weakness of lower limbs. In addition, he had a history of flu vaccination about 1.5 months before his neurological symptoms. The patient also had a history of skin lesions of hemorrhagic bulla and palpable purpura few days after injection on the lower limbs. At that time, a dermatologist visited the patient and a biopsy was taken with the impression of vasculitis. Eosinophilic dermal infiltration and leukocytoclastic vasculitis (LCV) were demonstrated in the biopsy specimen. Findings on the first day of admission were as follows: electromyogram/nerve conduction study (EMG/NCS) were compatible with subacute mixed type (demyelination and axonal) of inflammatory polyradiculopathy [Guillain-Barré syndrome (GBS)]. At the complete blood cell (CBC) exam, white blood cells (WBCs) were 14.21 (103/μl) with eosinophilia (40%) (Table 1). Erythrocyte sedimentation rate (ESR) was 43 and 84 at 1st and 2nd hours, respectively; C-reactive protein (CRP) was negative, and the rheumatoid factor (RF) was ++. In addition, antinuclear antibodies (ANA), anti-cyclic citrullinated peptide (anti-CCP) antibody, cytoplasmic-antineutrophil cytoplasmic antibody (c-ANCA), and perinuclear-anti-neutrophil cytoplasmic antibody (p-ANCA), and anti-phospholipid antibody were found to be negative. Lipid profiles and liver function tests were in the normal range. Brain and thoracolumbar spine magnetic resonance imaging (MRI) results were not significant, except for increased mucosal thickening at both maxillary and sphenoidal sinuses. Iranian Journal of Neurology