The Clinical Manifestations and Prognosis of Acute Disseminated Encephalomyelitis / 대한소아신경학회지

ABSTRACT

PURPOSE: Acute disseminated encephalomyelitis is an acute demyelinating autoimmune inflammatory disease of the central nervous system, which develops after infection or vaccination. The diagnosis is dependent on its clinical features and the brain MRI finding. Severely damaging white matter, acute disseminated encephalomyelitis which can lead to marked neurologic dysfunctions, may be fatal, and produce a permanent residual static disability or it may fully recover. We performed this study to investigate the clinical manifestations and prognosis of acute disseminated encephalomyelitis. METHODS: Ten cases (5 males and 5 females) of acute disseminated encephalomyelitis, who were diagnosed in the Department of Pediatrics at Yonsei University from January 1990 to December 1998, were reviewed retrospectively. RESULTS: The age at onset of symptoms was between 2 years 8 months and 13 years 8 months, and the time to the onset of symptoms was between 1 day and 7 days. The proceding events were nonspecific upper respiratory infections in 6 cases, and the other 4 cases had no proceeding events. The initial symptoms were altered consciousness, hemiparesis, seizure, fever, headache, and vomiting. The findings of cerebrospinal fluid were normal in 7 cases and the increase of protein and pleocytosis in 3 cases. Brain MRI showed multifocal high signal intensity lesions on T2-weighted image mainly in cerebral white matter, basal ganglia, and less frequently in the brain stem and cortex. Follow-up brain MRI was taken in 7 cases, and in all 7 cases brain MRI demonstrated partial to complete resolution of the lesions. In 8 cases, EEG was performed, and all 8 cases showed generalized or focal slow waves. All patients were treated with IV globulin and dexamethasone. The durations of the treatment were 4 or 5 days for IV globulin, and 8 days to 20 days for dexamethasone. The treatment was started within the first to third day of their admission, and the symptoms were improved within the second to the thirtieth day after the treatment started. All patients were followed-up for more than 2 months and up to 5 years 8 months. Among 10 cases, 9 of them were fully recovered, and only 1 case had left hemiparesis. CONCLUSION: Acute disseminated encephalomyelitis shows various clinical manifestations depending on its involvement with brain lesions, such as altered mental state, hemiparesis, and seizure. And IV globulin and dexamethasone were very effective in treating acute disseminated encephalomyelitis. In this study, the prognosis of acute disseminated encephalomyelitis did not correlate to its initial symptoms, CSF findings, EEG findings, MRI findings, or to the duration of its treatment. In most cases, the symptoms were fully reversible; however, in some cases, there remained permanent residual neurologic consequences.