Acute Disseminated Encephalomyelitis in a case of Hyper IgM Syndrome
Journal Title: Journal of Krishna Institute of Medical Sciences University - Year 2014, Vol 3, Issue 1
Abstract
Hyper IgM syndrome is a rare genetically heterogenoussyndrome and is characterized by an elevated or normalserum IgM and decreased IgG, IgA and IgE, indicatinga defect in the class – switch recombination(CSR) process. Patients are prone to recurrent infections.X linked variety occurring in males is the mostcommon form of the disease. Acute DisseminatedEncephalomyelitis (ADEM) in a case of Hyper IgMsyndrome has never been reported. Here we report a10 years old female with hyper IgM syndrome, possiblyautosomal form of the disease, who was admittedwith recurrent vomiting, seizures, encephalopathy, expressiveaphasia and bladder bowel dysfunction anddiagnosed to have ADEM.
Authors and Affiliations
Devdeep Mukherjee , Ritabrata Kundu , Prabal Niyogi , Joydev Tudu
Keywords
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