J Korean Child Neurol Soc.  2002 Nov;10(2):281-289.

Subacute Sclerosing Panencephalitis: Clinical Experience of 6 Cases

• Lee TY
• Kim SD
• Lim BC
• Hwang H
• Kang YJ
• Chae JH
• Kim KJ
• Hwang YS
• Kim IO

Abstract

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.

Keyword

Subacute sclerosing panencephalitis; Measles; EEG; MRI

MeSH Terms

Adolescent
Antibodies
Brain
Diagnosis
Early Diagnosis
Electroencephalography
Head
Hope
Humans
Immunoglobulin G
Korea
Magnetic Resonance Imaging
Male
Measles
Neurodegenerative Diseases
Oligoclonal Bands
Retrospective Studies
Seizures
Subacute Sclerosing Panencephalitis*
Antibodies
Immunoglobulin G
Oligoclonal Bands