Subacute Sclerosing Panencephalitis in a Toddler: Changing Epidemiological Trends

Abstract

Subacute sclerosing panencephalitis (SSPE) is a devastating “slow virus” brain disease resulting from persistent measles virus infection of neurons. The age at presentation is usually 8 to 11 years with onset usually occurring 2–10 years after measles infection. We report a 2-and-half-year-old boy who presented with progressively increasing myoclonic jerks and subtle cognitive decline. He was diagnosed as a case of SSPE based on clinical features, typical electroencephalographic finding, and elevated cerebrospinal fluid/serum measles antibody titers. He had measles 4 months prior to onset of symptoms. This case along with review of recently published reports suggests progressively decreasing latency period between measles infection and onset of symptoms observed in cases with SSPE. Clinical implication would mean investigating for SSPE even in infants or toddlers with compatible clinical features and recent history of measles infection.

Variations in Clinical Presentation, Neuroimaging and Eeg Patterns of Subacute Sclerosing Panencephalitis

“…the youngest being 2 years in our study. 15 Previously SSPE was considered to have a longer incubation period of at least 8-10 years after measles infection as evident from earlier studies done in Pakistan and India while two of our SSPE patients had no recall of measles infection. 16,17 Rafique et al reported demographic and clinical profile of SSPE in their retrospective study with the majority of the patients presented with seizures i.e.…”

Atypical Subacute Sclerosing Panencephalitis in a Young Child: Implications for Change in Vaccination Strategy

“…Only a handful of cases have been reported in infants and toddlers from India and abroad, youngest child being 4 months, most of them are due to congenital measles infection. [6][7][8][9] In a review, Cáceres et al found that placental transfer, initial serum levels, and rate of decay of maternal antibodies after birth, parity of mother, gestation, maternal exposure to malaria, and maternal human immunodeficiency virus status were significant factors affecting measles antibody titers in infants. 10 It has been seen that natural infection induces more antibodies than vaccination; furthermore, infants of younger mothers have higher antimeasles antibody titers than the older mothers.…”

Subacute sclerosing panencephalitis: promising results with intraventricular interferon alpha 2b therapy and intraventricular ribavarin therapy

“…The age at presentation is usually 8 to 10 years with latent period of 2-10 years after measles infection. However, SSPE has been previously reported by us in a 2-and-half-year-old boy who presented with progressively increasing myoclonic jerks and subtle cognitive decline and changing epidemiological trends were highlighted [1]. Sahaet al had reported an annual incidence of 21 per million population in India [2] Despite vast coverage with measles vaccination campaign by Government of India, measles has still not been eliminated till date.…”