انسفالومیلیت دمیلینیزان حاد منتشر: تظاهر غیر معمول

The acute disseminated encephalomyelitis is a rare, monophasic or multi-phasic dismyelinating disease, which affects the central nervous system and that appears shortly after either a exanthematous, non-exanthematous or vaccination. It attacks mainly children and young adults. The prognosis is usually favorable but sequelae are possible. The common therapeutic approach is the use of corticosteroids. Intravenous immunoglobulin (IVIg) has been proposed for relapses, and plasmapheresis for fulminant cases the cause of ADEM is unknown. The diagnosis is based on clinical picture, supported by radiology and laboratory tests. This 24-month-old boy was admitted with fever, vomiting and intermittent irritability preceded by a herpetic gingivostomatitis 10 days earlier. Growth and development were normal. Brain MRI at that time revealed multiple asymmetric, hyperintense and patchy white matter lesions of variable size on T2-weighted images. CSF examination revealed no cells, normal protein and glucose levels, normal IgG index and no oligoclonal bands. Gram’s and Wright’s stains and screening for viral antibodies were negative. Electroencephalography (EEG) was normal. He was started on Intrvenous immunoglobulin (400 mg/kg) intravenously for 5 days. At the end of the treatment he was afebrile with good general condition.