TY - JOUR
T1 - A recent surge of fulminant and early onset subacute sclerosing panencephalitis (SSPE) in the United Kingdom
T2 - An emergence in a time of measles
AU - Lam, Tanya
AU - Ranjan, Rajesh
AU - Newark, Kerensa
AU - Surana, Snehal
AU - Bhangu, Neeraj
AU - Lazenbury, Abigail
AU - Childs, Anne Marie
AU - Abbey, Ianthe
AU - Gibbon, Frances
AU - Thomas, Gareth
AU - Singh, Jaspal
AU - Prabhakar, Prab
AU - Kaminska, Margaret
AU - Lascelles, Karine
AU - Hacohen, Yael
AU - Brown, Kevin
AU - Lim, Ming
N1 - Funding Information:
We would like to acknowledge Helen Campbell, Jamie Lopez and Chris Verity for their thoughtful discussion about the cases from a public health and surveillance perspective, Dr Ata Siddiqui for interpretation of the MRI imaging, Dr Sushma Goyal and Dr Zaloa Agirre for interpretation of EEGs and all the physicians involved the management of the complex needs of the children.
Funding Information:
Ming Lim receives research grants from Action Medical Research, the DES society, GOSH charity, National Institute for Health Research, MS Society, and SPARKS charity; receives research support grants from the London Clinical Research Network and Evelina Appeal; has received consultation fees from CSL Behring, Novartis and Octapharma; received travel grants from Merck Serono; and was awarded educational grants to organize meetings by Novartis, Biogen Idec, Merck Serono, and Bayer. Prab Prabhakar: Research and consultation undertaken for MSD (Rizatriptan), NIHR (Propanalol, Pizotifen), BMS, Almirall, Amgen, Lilly, Council member for BASH, advisory role for OUCH, Migraine Trust, HIS.
Publisher Copyright:
© 2021 European Paediatric Neurology Society
Copyright:
Copyright 2021 Elsevier B.V., All rights reserved.
PY - 2021/9
Y1 - 2021/9
N2 - 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.
AB - 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.
KW - Developmental regression
KW - Measles
KW - Neurodegeneration
KW - Public health
KW - SSPE (Subacute sclerosing panencephalitis)
KW - Vaccination
UR - http://www.scopus.com/inward/record.url?scp=85113674853&partnerID=8YFLogxK
U2 - 10.1016/j.ejpn.2021.07.006
DO - 10.1016/j.ejpn.2021.07.006
M3 - Article
AN - SCOPUS:85113674853
SN - 1090-3798
VL - 34
SP - 43
EP - 49
JO - European Journal of Paediatric Neurology
JF - European Journal of Paediatric Neurology
ER -
