title: Acute disseminated encephalomyelitis: an uncommon presentation of hyper IgE syndrome.
abstract: The hyper-immunoglobulin E (IgE) syndrome (HIES), also known as Job's syndrome is a rare primary immunodeficiency characterized by the clinical triad of recurrent staphylococcal abscesses of skin, recurrent cyst-forming pneumonia, and an elevated serum IgE level of > 2000 IU/ml. Although, most cases are sporadic, families with autosomal dominant (AD-HIES) and recessive (AR-HIES) traits have been reported. Very few articles were published previously on central nervous system abnormalities with definite neurologic manifestations which may vary from partial facial nerve paralysis to hemiplegia in children but Acute Disseminated Encephalomyelitis (ADEM) in a child with HIES hitherto has not been reported. Here we describe a 5-year-old male child with HIES who presented with neurologic manifestations of ADEM.
authors.affiliation: Department of Paediatric Medicine, NRS Medical College and Hospital, AJC Bose Road, Kolkata, West Bengal, India.
authors.forename: Radheshyam
authors.initials: R
authors.lastname: Purkait
authors.affiliation: Department of Paediatric Medicine, NRS Medical College and Hospital, AJC Bose Road, Kolkata, West Bengal, India.
authors.forename: Sudipta
authors.initials: S
authors.lastname: Kar
authors.affiliation: Department of Radiology, NRS Medical College and Hospital, AJC Bose Road, Kolkata, West Bengal, India.
authors.forename: Ramchandra
authors.initials: R
authors.lastname: Bhadra
authors.affiliation: Department of Paediatric Medicine, NRS Medical College and Hospital, AJC Bose Road, Kolkata, West Bengal, India.
authors.forename: Tapankumar
authors.initials: T
authors.lastname: Sinhamahapatra
mesh_terms: D002648:Child; D004673:Encephalomyelitis, Acute Disseminated / Q000175:diagnosis*; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007073:Immunoglobulin E / Q000097:blood*; D007589:Job Syndrome / Q000175:diagnosis* / Q000188:drug therapy; D008297:Male; D011239:Prednisolone / Q000627:therapeutic use
chemical_list: D005938:Glucocorticoids; D007073:Immunoglobulin E; D011239:Prednisolone