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Subacute Sclerosing Panencephalitis in a North African Cohort: Findings From the Post-Measles Epidemic Era
Meriem Ben Hafsa1,2, Zouhour Miladi1,2, Insaf Dkhil2,3
1Department of Child and Adolescent Neurology, Research Laboratory LR18SP04, National Institute Mongi Ben Hmida of Neurology, La Rabta, Tunis, Tunisia.
Abstract:
Subacute sclerosing panencephalitis (SSPE) is a rare, fatal encephalitis caused by chronic measles virus infection. Its recent resurgence, particularly after measles epidemics, underscores the need for vigilance in children. This study describes the clinical, paraclinical, therapeutic features, and prognosis of SSPE in 15 patients. Initial symptoms included gait disturbances, psychomotor regression, and behavioral changes. Neurological examination revealed cognitive decline, visual impairment, hypotonia, pyramidal signs, ataxia, and movement disorders. Electroencephalogram showed periodic delta wave complexes, while brain imaging identified subcortical and periventricular white matter (WM) lesions, brainstem involvement, and progressive cortical-subcortical atrophy on follow-up. All patients tested positive for anti-measles immunoglobulins G in cerebrospinal fluid and blood. Despite treatment, rapid clinical deterioration led to akinetic mutism and neurovegetative symptoms. This study alerts clinicians and radiologists to consider SSPE in children presenting with subacute cognitive decline, myoclonus and epilepsy with periodic complexes, associated with specific WM changes, particularly in the post-epidemic period.
Insights
Subacute sclerosing panencephalitis (SSPE), a fatal measles virus encephalitis, is resurging in children. This study highlights its clinical signs, brain imaging findings, and poor prognosis, urging prompt diagnosis.
Area of Science:
- Neurology
- Virology
- Pediatrics
Background:
- Subacute sclerosing panencephalitis (SSPE) is a rare, fatal neurological complication of chronic measles virus infection.
- Recent measles epidemics have led to a resurgence of SSPE, particularly in pediatric populations, necessitating increased clinical awareness.
Purpose of the Study:
- To describe the clinical presentation, diagnostic features, treatment outcomes, and prognosis of SSPE in a cohort of 15 pediatric patients.
- To enhance recognition of SSPE among clinicians and radiologists, especially in the context of recent measles outbreaks.
Main Methods:
- Retrospective analysis of clinical data, neurological examinations, electroencephalograms (EEGs), and brain imaging (MRI) from 15 SSPE patients.
- Laboratory confirmation included testing for anti-measles immunoglobulin G (IgG) in cerebrospinal fluid (CSF) and serum.
Main Results:
- Patients presented with gait disturbances, psychomotor regression, and behavioral changes, progressing to cognitive decline, visual impairment, and movement disorders.
- EEG revealed periodic delta wave complexes; MRI showed white matter lesions, brainstem involvement, and progressive atrophy.
- All patients had elevated anti-measles IgG in CSF and blood, and despite treatment, showed rapid deterioration to akinetic mutism.
Conclusions:
- SSPE should be considered in children with subacute cognitive decline, myoclonus, epilepsy, and characteristic white matter changes, particularly following measles epidemics.
- Early recognition and diagnosis are crucial, although the prognosis remains poor, emphasizing the importance of measles vaccination to prevent SSPE.
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