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Induction and Diverse Assessment Indicators of Experimental Autoimmune Encephalomyelitis
Published on: September 9, 2022
Measles-vaccinated Israeli boy with subacute sclerosing panencephalitis
Ronly Har-Even1, Sergio Aichenbaum, Jose M Rabey
1Department of Pediatrics, Assaf Harofeh Medical Center, Zerifin, and Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel.
Insights
Subacute sclerosing panencephalitis (SSPE) can occur in vaccinated individuals. This case highlights the importance of suspecting SSPE in young patients presenting with neurological decline, even after immunization.
Area of Science:
- Neurology
- Virology
- Immunology
Background:
- Subacute sclerosing panencephalitis (SSPE) is a rare, progressive neurologic deterioration in children and adolescents, typically associated with measles virus infection.
- While vaccination significantly reduces measles incidence, rare cases of SSPE in vaccinated individuals warrant continued vigilance and research.
Observation:
- A 16-year-old boy, fully vaccinated against measles, presented with neurological symptoms including enuresis, involuntary movements, dysarthria, and ataxia.
- Diagnostic workup revealed elevated measles-specific IgG antibodies in serum and cerebrospinal fluid (CSF) with a high CSF/serum ratio, despite negative PCR for measles.
- EEG showed characteristic slow activity with high-voltage discharges, and MRI revealed diffuse white matter changes, particularly in posterior regions and around lateral ventricles.
Findings:
- The patient's clinical presentation and laboratory findings were consistent with SSPE, despite a history of measles vaccination.
- Standard treatments including valproic acid, levetiracetam, carbamazepine, and IVIG were ineffective.
- Further treatment with inosiplex and interferon-β-1a also failed to alter the disease's fatal progression, leading to coma and death within a year.
Implications:
- This case underscores that subacute sclerosing panencephalitis should be considered in the differential diagnosis of neurological disorders in young, vaccinated populations.
- It highlights the need for further investigation into the mechanisms of SSPE pathogenesis in vaccinated individuals.
- The findings emphasize the importance of ongoing surveillance and potential re-evaluation of vaccination strategies or post-vaccination immunity monitoring for rare complications.
Abstract:
Subacute sclerosing panencephalitis is a rare neurologic disorder of childhood and adolescence. We describe a 16-year-old boy who manifested the disease despite proper vaccinations. He was hospitalized because of bedwetting, involuntary limb movements, abnormal speech, and balance disturbances. Immunoglobulin G antibodies against measles were strongly positive, with a high relative cerebrospinal fluid/serum ratio. Polymerase chain reaction for measles produced negative results. Electroencephalography registered slow activity with high voltage discharges every few seconds, and with triphasic complex morphology. Magnetic resonance imaging revealed diffuse white matter changes, mostly around the posterior regions and lateral ventricles. Treatment with valproic acid, levetiracetam, carbamazepine, and intravenous immunoglobulin G was ineffective. Inosiplex and interferon-β-1a were also administrated. The patient became comatose, with generalized myoclonic jerks, and died 1 year later. An autopsy was not performed. This patient illustrates that subacute sclerosing panencephalitis should be suspected among young vaccinated subjects.
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