TY - JOUR
T1 - Neurological and cognitive outcomes after antibody-negative autoimmune encephalitis in children
AU - Gadian, Jonathan
AU - Eyre, Michael
AU - Konstantoulaki, Ele
AU - Almoyan, Ani
AU - Absoud, Michael
AU - Garrood, Isabel
AU - Lim, Ming
N1 - Funding Information:
This study was sponsored by Guy's & St Thomas' NHS Foundation Trust (IRAS 259608) and approved by the NHS Health Research Authority (REC 19/HRA/2000). As this study does not include patient identifiable information, individual patient consent was not required for this study.
Funding Information:
ME is supported by Action Medical Research and the British Paediatric Neurology Association.
Publisher Copyright:
© 2021 Mac Keith Press
PY - 2021/11/1
Y1 - 2021/11/1
N2 - AIM: To characterize the neurological and cognitive outcomes in children with antibody-negative autoimmune encephalitis (Ab-negative AE). METHOD: A cohort of children presenting to our institution over a 10-year period with autoimmune encephalitis was identified by structured retrospective review of medical records. Clinical features at presentation and final follow-up were recorded. Neuropsychological testing was performed in a subset of patients. Outcomes after Ab-negative AE were compared with outcomes after N-methyl-D-aspartate receptor antibody encephalitis (NMDARE). RESULTS: Forty-four patients (26 females, 18 males, median age 9y 2mo [interquartile range 4y 5mo–11y 8mo], 23 with NMDARE) with a diagnosis of autoimmune encephalitis were included. Postencephalitic epilepsy was more frequent after Ab-negative AE compared to NMDARE (61% vs 14%, p=0.002). Cognitive testing was performed in a subset of patients (n=21; Ab-negative AE=11, NMDARE=10). Full-scale IQ was lower after Ab-negative AE than NMDARE (mean IQ 75 vs 92, p=0.02), primarily because of reduced verbal comprehension index (80 vs 98, p=0.01) and working memory index (77 vs 95, p=0.09). The cognitive function most commonly impaired was executive function (80% [8/10] vs 22% [2/9]). INTERPRETATION: Ab-negative AE was associated with poorer cognitive outcomes than NMDARE at 1-year follow-up. Further studies are required to evaluate if immunotherapy can be optimized to improve outcome.
AB - AIM: To characterize the neurological and cognitive outcomes in children with antibody-negative autoimmune encephalitis (Ab-negative AE). METHOD: A cohort of children presenting to our institution over a 10-year period with autoimmune encephalitis was identified by structured retrospective review of medical records. Clinical features at presentation and final follow-up were recorded. Neuropsychological testing was performed in a subset of patients. Outcomes after Ab-negative AE were compared with outcomes after N-methyl-D-aspartate receptor antibody encephalitis (NMDARE). RESULTS: Forty-four patients (26 females, 18 males, median age 9y 2mo [interquartile range 4y 5mo–11y 8mo], 23 with NMDARE) with a diagnosis of autoimmune encephalitis were included. Postencephalitic epilepsy was more frequent after Ab-negative AE compared to NMDARE (61% vs 14%, p=0.002). Cognitive testing was performed in a subset of patients (n=21; Ab-negative AE=11, NMDARE=10). Full-scale IQ was lower after Ab-negative AE than NMDARE (mean IQ 75 vs 92, p=0.02), primarily because of reduced verbal comprehension index (80 vs 98, p=0.01) and working memory index (77 vs 95, p=0.09). The cognitive function most commonly impaired was executive function (80% [8/10] vs 22% [2/9]). INTERPRETATION: Ab-negative AE was associated with poorer cognitive outcomes than NMDARE at 1-year follow-up. Further studies are required to evaluate if immunotherapy can be optimized to improve outcome.
UR - http://www.scopus.com/inward/record.url?scp=85118310615&partnerID=8YFLogxK
U2 - 10.1111/dmcn.15101
DO - 10.1111/dmcn.15101
M3 - Article
AN - SCOPUS:85118310615
SN - 0012-1622
VL - 64
JO - Developmental Medicine and Child Neurology
JF - Developmental Medicine and Child Neurology
IS - 5
ER -