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Successful treatment of two paediatric cases of anti-NMDA receptor encephalitis with Cyclophosphamide: The need for early aggressive immunotherapy in tumour negative paediatric patients

Successful treatment of two paediatric cases of anti-NMDA receptor encephalitis with Cyclophosphamide: The need for early aggressive immunotherapy in tumour negative paediatric patients
Successful treatment of two paediatric cases of anti-NMDA receptor encephalitis with Cyclophosphamide: The need for early aggressive immunotherapy in tumour negative paediatric patients

We describe the clinical course and treatment of three unrelated female patients ranging in age from 27 months to 14 years with anti-NMDA receptor encephalitis. The third case is reported as an addendum to the paper. None of the cases were paraneoplastic. All received initial immunotherapy consisting of steroids and IVIg, and two of them received 3 and 8 plasma exchanges respectively, without consistent or sustained clinical improvement. All three girls were then treated with monthly cycles of Cyclophosphamide. All had resolution of their movement disorder and a dramatic and sustained clinical improvement of their other symptoms in the domains of cognition, language and behaviour. The clinical improvement began after the first cycle in two and the second cycle in the third and continued with the subsequent cycles. None developed side-effects of treatment. In light of the recent review of the condition and our own clinical experience in the paediatric age group, we propose that second line immunotherapy should be considered early after failure of first line immunotherapy.

Anti-NMDA receptor encephalitis, Cyclophosphamide, Immunotherapy, Paediatric
1090-3798
74-78
Kashyape, Pawan
53b118a0-2b39-4898-b52e-d136f72f5a16
Taylor, Ellen
209f19bc-75fc-4b46-8c60-a81110b9a666
Ng, Joanne
dd9befcf-7fe2-4524-aaaa-0c4d170d3f6a
Krishnakumar, Deepa
32b7bf0c-561e-4d7e-971e-f1a93b924d79
Kirkham, Fenella
1dfbc0d5-aebe-4439-9fb2-dac6503bcd58
Whitney, Andrea
786b3e0e-6d15-4474-8007-4453e2dcff9b
Kashyape, Pawan
53b118a0-2b39-4898-b52e-d136f72f5a16
Taylor, Ellen
209f19bc-75fc-4b46-8c60-a81110b9a666
Ng, Joanne
dd9befcf-7fe2-4524-aaaa-0c4d170d3f6a
Krishnakumar, Deepa
32b7bf0c-561e-4d7e-971e-f1a93b924d79
Kirkham, Fenella
1dfbc0d5-aebe-4439-9fb2-dac6503bcd58
Whitney, Andrea
786b3e0e-6d15-4474-8007-4453e2dcff9b

Kashyape, Pawan, Taylor, Ellen, Ng, Joanne, Krishnakumar, Deepa, Kirkham, Fenella and Whitney, Andrea (2012) Successful treatment of two paediatric cases of anti-NMDA receptor encephalitis with Cyclophosphamide: The need for early aggressive immunotherapy in tumour negative paediatric patients. European Journal of Paediatric Neurology, 16 (1), 74-78. (doi:10.1016/j.ejpn.2011.07.005).

Record type: Article

Abstract

We describe the clinical course and treatment of three unrelated female patients ranging in age from 27 months to 14 years with anti-NMDA receptor encephalitis. The third case is reported as an addendum to the paper. None of the cases were paraneoplastic. All received initial immunotherapy consisting of steroids and IVIg, and two of them received 3 and 8 plasma exchanges respectively, without consistent or sustained clinical improvement. All three girls were then treated with monthly cycles of Cyclophosphamide. All had resolution of their movement disorder and a dramatic and sustained clinical improvement of their other symptoms in the domains of cognition, language and behaviour. The clinical improvement began after the first cycle in two and the second cycle in the third and continued with the subsequent cycles. None developed side-effects of treatment. In light of the recent review of the condition and our own clinical experience in the paediatric age group, we propose that second line immunotherapy should be considered early after failure of first line immunotherapy.

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More information

Published date: January 2012
Keywords: Anti-NMDA receptor encephalitis, Cyclophosphamide, Immunotherapy, Paediatric

Identifiers

Local EPrints ID: 429120
URI: http://eprints.soton.ac.uk/id/eprint/429120
ISSN: 1090-3798
PURE UUID: 4a98fb8c-16c1-4388-ac98-26f183293335
ORCID for Fenella Kirkham: ORCID iD orcid.org/0000-0002-2443-7958

Catalogue record

Date deposited: 21 Mar 2019 17:30
Last modified: 13 Aug 2022 01:39

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Contributors

Author: Pawan Kashyape
Author: Ellen Taylor
Author: Joanne Ng
Author: Deepa Krishnakumar
Author: Fenella Kirkham ORCID iD
Author: Andrea Whitney

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