Unusual neurological manifestations in the patients with idiopathic hypereosinophilic syndrome

초록

Background & Objectives : Idiopathic hypereosinophilic syndrome (HES) is characterized by prolonged hypereosinophilia in circulating absolute eosinophil count (>1500 cubic mm), multisystem organ involvement due to eosinophilic infiltration or eosinophil-derived product, and the lack of any secondary etiology of hypereosinophilia. Usual neurological manifestations in HES are polyneuropathy or mononeuritis multiplex, ischemic stroke, and organic psychiatric syndromes. Method : In this study we present the 4 cases showing unusual or unreported neurological manifestations in idiopathic HES excluding secondary hypereosinophilia. Results : The presenting cases are as follows: 1) 46 year-old male with motor neuron disease and insensitivity to hypercarbia syndrome in idiopathic HES, 2) 35 year-old male with hypereosinophilic myelitis confirmed by pathological tissue diagnosis, 3) 10 year-old female with multiple cranial neuropathy including bilateral abducens and facial palsy and unilateral hypoglossal palsy, 4) 65 year-old-female with multiple ophthalmoplegia. Histories and laboratory findings were compatible with the HES in all patients. Conclusion : We report cases of unusual or unreported neurological manifestations in HES.