Clinical features, investigations, and outcomes of pediatric limbic encephalitis: A multicenter study.
Ann Clin Transl Neurol
; 9(1): 67-78, 2022 01.
Article
em En
| MEDLINE
| ID: mdl-35015932
ABSTRACT
OBJECTIVES:
To describe the clinical presentation, investigations, management, and disease course in pediatric autoimmune limbic encephalitis (LE).METHODS:
In this retrospective observational study, from the UK Childhood Neuroinflammatory Disease network, we identified children from six tertiary centers with LE <18 years old between 2008 and 2021. Clinical and paraclinical data were retrieved from medical records.RESULTS:
Twenty-five children fulfilling LE criteria were identified, with median age of 11 years (IQR 8, 14) and median follow-up of 24 months (IQR 18, 48). All children presented with seizures; 15/25 (60%) were admitted to intensive care. Neuroimaging demonstrated asymmetric mesial temporal changes in 8/25 (32%), and extra-limbic changes with claustrum involvement in 9/25 (38%). None were positive for LGI1/CASPR2 antibodies (Abs), 2/25 were positive for serum anti-NMDAR Abs, and 2/15 positive for anti-Hu Abs; one died from relapsing neuroblastoma. Two children had serum and CSF anti-GAD antibodies. Initial immune therapy included steroids in 23/25 (92%), intravenous immunoglobulin (IVIg) in 14/25 (56%), and plasma exchange in 7/25 (28%). The commonest second-line treatment was rituximab in 15/25 (60%). Median duration of hospital admission was 21 days (IQR 11, 30). At last follow-up, 13/25 (52%) had refractory seizures and 16/25 (64%) had memory impairment. Six children (24%) had modified Rankin Scale (mRS) scores ≥3. There was no significant difference in mRS, or long-term cognitive and epilepsy outcomes in those who received rituximab versus those who did not.INTERPRETATION:
A diagnosis of autoimmune LE was associated with significant morbidity and adverse outcomes in this pediatric cohort.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Troca Plasmática
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Autoanticorpos
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Doenças Autoimunes
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Encefalite Límbica
/
Fatores Imunológicos
Tipo de estudo:
Observational_studies
/
Prognostic_studies
/
Risk_factors_studies
Limite:
Adolescent
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Child
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Child, preschool
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Female
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Humans
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Male
Idioma:
En
Revista:
Ann Clin Transl Neurol
Ano de publicação:
2022
Tipo de documento:
Article