Clinical Manifestation of Acute Disseminated Encephalomyelitis in Children

Acute disseminated encephalomyelitis(ADEM) is an acute demyelinating autoimmune inflammatory disease of the central nervous system which develops after infection of vaccination. It may be fatal, and produce a permanent residual static disability or fully recover. We retrospectively studied 14 cases to investigate the clinical findings and outcome of ADEM.

14cases of ADEM diagnosed at Ewha Womans University Mokdong Hospital from 1998 to 2003 were retrospectively reviewed.

The age of onset was 5 years±7.8 months and no seasonal clustering was found. The time of onset of symptoms was between 3 to 30 days. The preceding events were nonspecific upper respiratory infections in 9 cases, aseptic meningitis in 4 cases, and gastroenteritis in one case. The initial symptoms were seizure, altered consciousness, hemiparesis, fever, headache, and vomiting. Brain MRI showed multifocal high signal intensity lesions on T2 weighted image mainly in the cerebral whith matter, basal ganglia and periventricular white matter. EEG was performed in some cases and showed generalized or focal slow waves and only one case showed focal spikes. Patients were treated with IV globulin and methylprednisolone and the symptoms improved within 3-7 days after treatment. All patients were followed up for more than 2 months and most of them fully recovered except two.

ADEM presents in various clinical manifestations depending on its involvement of the brain lesions. Most cases recovered fully but in some cases, residual permanent neurologic sequelae remained.