Long-term remission with rituximab in refractory leucine-rich glioma inactivated 1 antibody encephalitis.
Autoimmune encephalitis associated with antibodies to leucine-rich glioma inactivated 1 (LGI1) is recently described and there is a lack of detailed reports on the treatment of relapsing or refractory cases and long-term outcomes. Two case reports are presented. Both cases had faciobrachial dystonic seizures (FBDS) and received rituximab after relapsing or refractory disease. Both cases achieved sustained clinical remission of up to 15 and 56 months respectively. Rituximab use allowed withdrawal of corticosteroids and was well tolerated. Randomized clinical trials are needed in LGI1 encephalitis and other autoimmune encephalitides.
Department of Clinical Neurosciences, Addenbrooke's Hospital, University of Cambridge, United Kingdom. Electronic address: william.brown@doctors.org.uk.
J William L
JW
Brown
Department of Clinical Neurosciences, Addenbrooke's Hospital, University of Cambridge, United Kingdom. Electronic address: peter.martin@addenbrookes.nhs.uk.
Peter J
PJ
Martin
Department of Clinical Neurosciences, Addenbrooke's Hospital, University of Cambridge, United Kingdom. Electronic address: john.thorpe@addenbrookes.nhs.uk.
John W
JW
Thorpe
Department of Clinical Neurosciences, Addenbrooke's Hospital, University of Cambridge, United Kingdom. Electronic address: andrew.michell@addenbrookes.nhs.uk.
Andrew W
AW
Michell
Department of Clinical Neurosciences, Addenbrooke's Hospital, University of Cambridge, United Kingdom. Electronic address: ajc1020@medschl.cam.ac.uk.
Alasdair J
AJ
Coles
Department of Clinical Neurosciences, Addenbrooke's Hospital, University of Cambridge, United Kingdom. Electronic address: amandacox1@nhs.net.
Amanda L
AL
Cox
Neurosciences Group, Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, Oxford, United Kingdom. Electronic address: angela.vincent@ndcn.ox.ac.uk.
Angela
A
Vincent
Department of Clinical Neurosciences, Addenbrooke's Hospital, University of Cambridge, United Kingdom. Electronic address: msz20@cam.ac.uk.
Michael S
MS
Zandi
D000328:Adult; D000368:Aged; D000906:Antibodies / Q000097:blood*; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use*; D004660:Encephalitis / Q000097:blood* / Q000188:drug therapy* / Q000276:immunology; D005260:Female; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use; D047908:Intracellular Signaling Peptides and Proteins; D008297:Male; D011506:Proteins / Q000276:immunology*; D000069283:Rituximab; D055502:Secondary Prevention; D012640:Seizures / Q000188:drug therapy*; D016896:Treatment Outcome
D000906:Antibodies; D058846:Antibodies, Monoclonal, Murine-Derived; D007155:Immunologic Factors; D047908:Intracellular Signaling Peptides and Proteins; C116499:LGI1 protein, human; D011506:Proteins; D000069283:Rituximab
Autoantibody; Cognition; Encephalitis; LGI1; Rituximab; VGKC