Case Report of a 3-year-old Patient with Subacute Sclerosing Panencephalitis

Question – Summary: We report the disease trajectory of a 3-year-old patient with acute-progressive SSPE based on the clinical course and laboratory and virological results as well as the findings from EEG and cMRI. In addition, we explain our antiviral and anticonvulsive therapeutic attempts as well as possible therapeutic options.

Material - Method – Results: At the beginning of January 2019, unsteady gait as well as a developmental regression and a severe encephalopathy with high-frequency epileptic spasms of the trunk musculature, atypical absence and reduced vigilance became noticeable. In cMRI, a brain atrophy was found, in CSF, an increasing measles virus antibody titers could be shown, which led to the diagnosis of SSPE. At the same time as an implantation of a Rickham reservoir a brain biopsy obtained, genotyping revealed that it is measles virus wild type B3. Intravenous immunoglobulin administration and antiviral therapy with oral inosine, Ribavarin i.v. and interferon intrathecally showed no significant improvement in the clinical course. An anticonvulsive therapy with dronabinol, clobazam, phenytoin and vigabatrin was able to slightly improve acute seizures at the cost of vigilance. The patient was never seizure-free. We also discussed an extension of the anticonvulsive therapy to a ketogenic diet.

Discussion – Conclusion: Our patient was vaccinated against measles according to the guidelines of the German permanent advisory panel for vaccination. A measles virus disease was not amenable in our patients history. However, due to the detection of measles wild-type virus in brain tissue, it we assume that a measles infection took place in the first year before measles vaccination. In studies, an anti-viral effect of 6-fluoro-3-hydroxy-2-pyrazinecarboxamide (favipiravir) against against paramyxoviruses was detected by selective inhibition of the RNA-dependent RNA polymerase of RNA viruses and is in the current situation considered for another therapeutic attempt. Due to approval difficulties in Germany, this therapy was not possible. Children in the first year of life before their first vaccination against measles can only be protected by herd immunity to measles disease including potential severe complications as SSPE. For this reason, the consistent implementation of the vaccination recommendations of the German permanent advisory panel for vaccination (STIKO) is of particular importance.

No conflict of interest has been declared by the author(s).