Subacute sclerosing panencephalitis as a re-emerging condition due to low vaccination coverage: a case-series

Abstract

Background: Subacute sclerosing panencephalitis (SSPE) is a chronic progressive disease affecting the central nervous system (CNS) because of persistent measles virus (MeV) infection. The disease presents with a range of symptoms, starting with gradual cognitive and motor decline, progressing to coma, vegetative state, and typically resulting in death within 3-4 years of clinical onset. The incidence of SSPE is inversely related to vaccination coverage and its pathogenesis remains poorly understood. The primary risk factor is infection by the wild type of measles virus, particularly in individuals who may be predisposed to infection due to a lack of herd immunity or immunosuppressed states. Furthermore, the risk of developing SSPE increases with the younger age at which a person contracts measles.

Case presentation: We provide a description of the disease, characterizing its clinical course, diagnostic work-up and treatment options. We will also present a series of cases observed at the Santobono-Pausilipon Children's Hospital, in Naples, Italy, highlighting both typical and atypical presentations, with a particular emphasis on the pharmacological and neurosurgical treatments for the condition.

Conclusions: The objective of this study is to raise awareness about the increasing prevalence of conditions following the SARS-Cov2 pandemic, with an emphasis on the importance of vaccination adherence as the only effective prevention method for SSPE.

Keywords: Interferon alpha; Measles; SSPE; Seizures; Vaccine.

Fig. 1

First brain MRI performed in patient 1: Hyperintense in T2 FLAIR signal alteration of the periventricular white matter, corona radiata and semioval centres with bilateral distribution involving U-shaped fibers; similar signal alteration was observed at the level of the corpus callosum, which was globally thinned; this blurred alteration was also present at the putamen, caudates, thalami and mesencephalic regions; reduction in encephalic tissue trophism of the midbrain, pons and upper and middle cerebellar peduncles, associated with enlargement of the ventricular system

Fig. 2

SSPE’s typical EEG: EEG showing periodic complexes

Fig. 3

Second Brain MRI perfomed in patient 1: The exam revealed bilateral involvement of the lower occipital lobes and particular subcortical localization in the left calcarine region with extension to the Gratiolet optical radiation bilaterally, especially on the left

Fig. 4

Last brain MRI performed in patient 1: Brain MRI performed after neurosurgical treatment revealed resolution of the previously reported cortical and subcortical cytotoxic alterations in both occipital lobes and in the left middle-inferior frontal area, where signs of glial atrophy occurred

Fig. 5

Brain CT: Examination performed after electronic pump implantation for intraventricular delivery of interferon

Fig. 6

Brain MRI performed in patient 3: T2 FLAIR hyperintensity of the white matter in the posterior periventricular area (see blue arrows)

Fig. 7

Follow-up Brain MRI performed in patient 4: The exam revealed multiple areas with altered signals in the bilateral thalamic, left frontal, parietal and bilateral temporal-occipital cortical areas

Fig. 8

Brain MRI (last performed in patient 4): The exam revealed diffuse supratentorial glial atrophy involution