Résumé
PURPOSE: Subacute sclerosing panencephalitis(SSPE) is a severe and usually fatal neurodegenerative disorder of childhood and adolescence. The etiology is related to previous measles infection especially during the first 2 years of life. Since recent measles epidemics in Korea may increase the late risk of SSPE, the authors investigated the clinical characteristics of SSPE focusing on brain MRI. METHODS: Six cases(4 males, 2 females) of SSPE patients were retrospectively reviewed for clinical, EEG, laboratory and brain MRI findings. RESULTS: Four of 6 had a history of measles infection in the first year of life. Clinical manifestations were as follows:myoclonus(6), falling(4), ataxia(4), dysarthria(3), seizures (2), involuntary movements(2), tremor(2), head drop(1), sleep disturbance(1). In all cases, CSF IgG, CSF IgG/albumin ratio, and CSF/serum IgG index increased, oligoclonal bands were positive, and CSF antimeasles antibodies were positive. Frontal high amplitude sigma activities and anteriorly-accentuated multifocal epileptiform discharges were noted on EEG. Brain MRI revealed T2-weighted high signal intensity of the deep white matter. CONCLUSION: The diagnosis of SSPE depends on characteristic clinical features and elevation of measles antibodies in CSF, supported by others including EEG, CSF and brain MRI findings. We hope the clinical characteristics we mentioned may be useful for the early diagnosis and active management of SSPE in Korea.
Sujets)
Adolescent , Humains , Mâle , Anticorps , Encéphale , Diagnostic , Diagnostic précoce , Électroencéphalographie , Tête , Espoir , Immunoglobuline G , Corée , Imagerie par résonance magnétique , Rougeole , Maladies neurodégénératives , Bandes oligoclonales , Études rétrospectives , Crises épileptiques , Leucoencéphalite sclérosante subaigüeRésumé
Systemic capillary leak syndrome(SCLS) is a rare disorder of unknown etiology, which is characterized by recurrent attacks of hypotension, hemoconcentration, and hypoalbuminemia. Urinary or enteric loss of protein is not demonstrated. It is often associated with monoclonal gammopathy, but does not manifest multiple myeloma. Since Clarkson et al. described the first case in a 34- year-old woman, about 50 cases have been reported in the literature. However, most of the cases were of adult age, and the mean age of onset in the reported cases was 42.6 years. In literature review, we could refer only one pediatric case of SCLC by Foeldvari et al. in 1995. We report another pediatric case of SCLS.