Rapidly progressive cerebral dysfunction in a 5-year-old child: A reminder of subacute sclerosing panencephalitis (SSPE) and the importance of measles vaccination coverage

Abstract

We present a case of a preschool boy admitted to the pediatric intensive care unit with generalized weakness and encephalopathy that progressed to coma and spastic paralysis over the next few weeks. Extensive evaluation of a wide range of possible diagnoses, including infectious, post-infectious, autoimmune, and paraneoplastic disorders, proved unrevealing. Owing to concerns regarding autoimmune encephalitis, the patient received plasmapheresis, intravenous immunoglobulin, high-dose glucocorticoids, anakinra, rituximab, and empirical botulinum antitoxin. Despite these treatments, the patient's neurological condition continued to deteriorate, requiring endotracheal intubation. The patient developed repeated tremors and dystonic events, which progressed to hypertonia with gaze deviation. His-EEG in the third week of admission showed Radermecker complexes most consistent with SSPE. Because of his history of travel to Afghanistan at 8 months of age, before he was vaccinated for measles, and after a measles-like illness upon return, we checked the measles IgG titer in the CSF from the initial lumbar puncture and found it to be elevated. Global coverage with the first dose of the measles vaccine has dropped to 81 % by 2021, the lowest rate since 2008. This decline raises the concern of a possible increase in the incidence of measles and its fatal complications such as SSPE.