HyperIgEemia and atopy have recently been reported to be related to various neurological diseases such as Hirayama disease and idiopathic myelitis. The aims of this study are to determine frequency of atopy or hyperIgEemia in idiopathic myelitis and to characterize the clinical and laboratory profiles of atopic myelitis (AM). From January 2006 to August 2008, 29 consecutive patients with idiopathic myelitis were recruited. We compared demographic data, laboratory results and radiologic findings between patients with atopic diathesis and those without. Allergic or atopic history was found in only 4 patients (13%), but hyperIgEemia and mite antigen-specific IgE were observed in 17 (58%) and 19 (65%) of idiopathic myelitis patients, respectively. Patients with AM (n=14, 48%) showed the following distinctive features: (1) younger age at onset, (2) non-acute onset and long duration of symptoms at admission, (3) predominant sensory symptoms with mild weakness, (4) low EDSS score, (5) low frequency of abnormal SEP findings, and (6) increased eosinophils in peripheral blood. Common MR findings of AM included eccentric lesions occupying more than two-thirds of spinal cord with focal peripheral enhancement on axial image. These lesions were usually extended over more than 3 to 5 vertebral segments with cord swelling. HyperIgEemia and mite antigen-specific IgE are fairly common in idiopathic myelitis patients. The AM patients show relatively homogenous clinicolaboratory and radiological features. It is noteworthy that none of these patients showed brain abnormalities suggestive of multiple sclerosis or neuromyelitis optica (NMO).