A recent surge of fulminant and early onset subacute sclerosing panencephalitis (SSPE) in the United Kingdom: An emergence in a time of measles
| Autor: | Prab Prabhakar, Neeraj Bhangu, Abigail Lazenbury, Ianthe Abbey, Tanya Lam, Snehal Surana, Frances Gibbon, Karine Lascelles, Ming K. Lim, Gareth Thomas, Kerensa Newark, Jaspal Singh, Yael Hacohen, Kevin E. Brown, Anne Marie Childs, Rajesh Ranjan, Margaret Kaminska |
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| Rok vydání: | 2020 |
| Předmět: |
Pediatrics
medicine.medical_specialty Movement disorders Fulminant Neurological disorder Measles Subacute sclerosing panencephalitis medicine Humans Child business.industry Incidence (epidemiology) Vaccination Infant General Medicine medicine.disease Magnetic Resonance Imaging United Kingdom Child Preschool Pediatrics Perinatology and Child Health Neurology (clinical) Subacute Sclerosing Panencephalitis medicine.symptom business Developmental regression |
| Zdroj: | European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society. 34 |
| ISSN: | 1532-2130 |
| Popis: | Background Subacute Sclerosing Panencephalitis (SSPE) is a fatal progressive neurological disorder following measles infection. Methods Cases were collated from Paediatric Neurology centres in the UK over 24 months from 2017 to 2019 and represent all cases referred to the National Viral Reference Department (VRD). Diagnosis was established with detection of a raised measles index, demonstrating intrathecal measles antibody production. Findings Six children presented with SSPE over two years, with median age five years (range 2–7 years) and median latency period three years (range 2–6 years). The majority were exposed to measles during infancy. Atypical features were common, including visual impairment, focal and generalised tonic-clonic seizures, headache, vomiting and movement disorders. EEG demonstrated typical features in five cases, though not always at presentation. Initial MRI was normal in four cases, with two showing focal and widespread white matter changes. Antiviral and immunomodulatory treatment led to minimal or no improvement. All progressed to cognitive regression, seizures and neurological decline within six months. Interpretation These cases demonstrate the highest incidence of SSPE in the UK since 2000, all progressing to acute fulminant disease, following younger age of onset, short latency period and atypical presentations. Recent global surges in measles cases raise the importance of clinician awareness of SSPE as a potential diagnosis in children with neurological regression. Herd immunity remains the key protective mechanism for infants and groups that cannot be vaccinated. Health care providers, educators and governments must ensure resources continue to target effective education and access to immunisation programmes, the only means to combat this devastating and fatal condition. |
| Databáze: | OpenAIRE |
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