Abstract :
Introduction: Subacute sclerosing panencephalitis (SSPE) is a rare but fatal progressive neurodegenerative disorder caused by persistent measles virus infection in the brain. Although its incidence has declined in countries with widespread measles vaccination, SSPE continues to pose a significant challenge in developing regions. Increasing reports of atypical clinical presentations further complicate early diagnosis, emphasizing the need for heightened clinical awareness.Aim and Objectives: To find out Initial presentation and clinical and neuro-imaging findings of patients with Subacute sclerosing panencephalitis.Materials and Methods: This cross-sectional study was conducted at Neurology ward of Calcutta National Medical College and Hospital, a tertiary care hospital in West Bengal. Between the period June 2022 to May 2024, total 23 patients of SSPE were evaluated in neurology ward. Diagnosis was based on Modified Dyken’s Criteria. MRI Brain and Ophthalmological evaluation were done for all patients. Special emphasis was given to initial presenting symptoms.Results: Total 23 patients were studied. Mean age of our patients were 11.13 years and male female ratio was 1.875:1. More than half (69.5%) patients were from poor socio-economic status. Among 23 patients of SSPE 56.5% were vaccinated and 60 % had history of measles infection. On a thorough history it was noted that initial presenting features were myoclonus (43.5%), cognitive decline /poor school performance (52.2%), visual disturbance (17.4%), seizure (17.4%), behavioural abnormality (8.7%), Unilateral tremor (4%), and gait disturbance (8.7%). While on first examination after admission clinical signs were myoclonus (82.6%), cognitive decline (82.6%), visual disturbance (26%), dysarthria (21.7%), seizure (17.4%), behavioural abnormality (52.2%), urinary and faecal incontinence (21.7%), Unilateral tremor (4%) and gait disturbance (8.7%). Ocular findings like optic neuritis, chorioretinitis, papilledema were present in 34.8%. Neuroimaging abnormalities in 69.6% cases included parieto-occipital white matter hyperintensity, cortical atrophy and ischaemic changes. Abnormal EEG in 91.3% cases. Measles specific IgG antibody in CSF were present in 100% of cases.Conclusions: In developing countries like India where measles is still prevalent, high index of suspicion regarding uncommon presentations of SSPE like vision loss, seizures, behavioural disturbances, unilateral tremor and gait disturbance will help in early diagnosis of the disease.
Keyword :
Subacute sclerosing panencephalitis, Measles virus, Atypical clinical presentation, Neurodegenerative disease, Myoclonus, Cognitive decline.