Acute disseminated encephalomyelitis in a case of hyper IgM syndrome

Hyper igm syndrome is a rare genetically heterogenous syndrome and is characterized by an elevated or normal serum igm and decreased igg,iga and ige,indicating a defect in the class - switch recombination (csr) process. patients are prone to recurrent infections. x linked variety occurring in males is the most common form of the disease. acute disseminated encephalomyelitis (adem) in a case of hyper igm syndrome has never been reported. here we report a 10 years old female with hyper igm syndrome,possibly autosomal form of the disease,who was admitted with recurrent vomiting,seizures,encephalopathy,expressive aphasia and bladder bowel dysfunction and diagnosed to have adem. © journal of krishna institute of medical sciences university.