Subacute Sclerosing Panencephalitis in Children: The Archetype of Non-Vaccination

Abstract

Subacute sclerosing panencephalitis (SSPE) is a late complication of measles virus infection that occurs in previously healthy children. This disease has no specific cure and is associated with a high degree of disability and mortality. In recent years, there has been an increase in its incidence in relation to a reduction in vaccination adherence, accentuated by the COVID-19 pandemic. In this article, we take stock of the current evidence on SSPE and report our personal clinical experience. We emphasise that, to date, the only effective protection strategy against this disease is vaccination against the measles virus.

Keywords: measles; subacute sclerosing panencephalitis; treatment; vaccination.

Figure 1

Typical EEG at the onset of symptoms, performed according to the international 10–20 system with digital acquisition and polygraphy. EEG recording of 60 s awake (A) and during sleep (B). Pathological background activity is globally slowed down, disorganised and undifferentiated both in wakefulness and in sleep. Continuous periodic (about 5–6 s) and polyphasic complexes consisting of several high-voltage, irregular and bilateral asynchronous delta waves. The polygraphy recorded atonic seizures of limbs, head and face concomitantly with the periodic complexes.

Figure 2

EEG of patient D after 1 year of illness. The waking EEG trace showed globally disorganised, undifferentiated and asymmetrical brain activity due to the presence of hypovolted activity in the left frontal–central–temporal regions.

Figure 3

MRI of patient D. Axial T2-weighted FLAIR MRIs at the time of diagnosis (age 3.9 years) and after 1 year of illness (age 4.9 years) show, respectively, signal alteration of the bilateral posterior parieto-occipital region and severe cerebral atrophy.

Figure 4

Stage variation during and after treatment. This graphic shows the clinical stage variation at diagnosis (time 0), during IFN-α therapy (coloured box) and after therapy (white box). Intrathecal IFN-α therapy was considered the main treatment in this graphic (coloured box). As illustrated, at time 0, a diagnosis was made and coincided with the start of the treatment. Patient A took therapy for 1 year. He presented mild clinical progression in the following year, with relative stabilisation in stage 2c, even after 2 years of therapy discontinuation. Patients undergoing IFN-α therapy for a long time (5.5 years for patient B and 3 years for patient C) showed clinical stabilisation (patient B) or a mild improvement with a subsequent stabilisation even after 4 years without therapy (patient C). Patient D took therapy for a short time, with a discontinuation, and he showed clinical progression.