Rasmussen encephalitis: Predisposing factors and their potential role in unilaterality.
Rasmussen encephalitis
autoimmune diathesis
fever association
perinatal problems
Journal
Epilepsia
ISSN: 1528-1167
Titre abrégé: Epilepsia
Pays: United States
ID NLM: 2983306R
Informations de publication
Date de publication:
01 2022
01 2022
Historique:
revised:
08
11
2021
received:
11
08
2021
accepted:
08
11
2021
pubmed:
26
11
2021
medline:
21
4
2022
entrez:
25
11
2021
Statut:
ppublish
Résumé
Rasmussen encephalitis (RE) is a progressive and destructive inflammatory disease of one hemisphere. Its cause is unknown. We investigated comorbidity and laterality factors that might predispose to RE. We retrospectively compared the histories of 160 RE patients to those with genetic generalized epilepsy (n = 154) and those with focal cortical dysplasia Type II (FCD II; n = 148). The median/mean age at symptom onset in RE was 7/10 years (range = 1-53 years), and 58.1% of the patients were female. The female sex predominated in RE patients, with age > 7 years at disease manifestation. The left hemisphere was affected in 65.6%. Perinatal complications (preterm birth, twin pregnancies, early acquired brain lesions) were more frequent in RE than in control patients. Ipsilateral facial autoimmune conditions (scleroderma en coup de sabre, uveitis, or chorioretinitis) were only observed in RE patients (6.9%). Onset of RE was more frequently associated with fever than that of FCD II. In 33.1% of RE patients, ≥1 potential risk factor was found. Interestingly, 11.9% of patients had one-sided early brain lesions or facial autoimmune lesions ipsilateral to subsequent RE; none had such a lesion contralaterally. Perinatal complications and facial autoimmune conditions may act as predisposing factors for RE. Fever might trigger RE manifestation. Further genetic or infectious contributors may be identified in the future. Single or combined hits may be required to elicit or facilitate the start of the disease. Ipsilateral early comorbid lesions or facial autoimmune processes might in part explain the enigmatic unilaterality of RE.
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
108-119Informations de copyright
© 2021 The Authors. Epilepsia published by Wiley Periodicals LLC on behalf of International League Against Epilepsy.
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