Rituximab for Autoimmune Encephalitis with Epilepsy

Intractable epilepsy remains a significant medical challenge, resulting in recurrent and prolonged intensive care unit (ICU) admissions. Autoimmune encephalitis is emerging as a treatable cause of intractable epilepsy. It is characterized by antibodies against cerebral antigens, such as potassium ch...

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Main Authors: Mohankumar Kurukumbi, Rahul H. Dave, Jose Castillo, Tulsi Shah, Joanne Lau
Format: Article
Language:English
Published: Wiley 2020-01-01
Series:Case Reports in Neurological Medicine
Online Access:http://dx.doi.org/10.1155/2020/5843089
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author Mohankumar Kurukumbi
Rahul H. Dave
Jose Castillo
Tulsi Shah
Joanne Lau
author_facet Mohankumar Kurukumbi
Rahul H. Dave
Jose Castillo
Tulsi Shah
Joanne Lau
author_sort Mohankumar Kurukumbi
collection DOAJ
description Intractable epilepsy remains a significant medical challenge, resulting in recurrent and prolonged intensive care unit (ICU) admissions. Autoimmune encephalitis is emerging as a treatable cause of intractable epilepsy. It is characterized by antibodies against cerebral antigens, such as potassium channels such as leucine-rich, glioma inactivated 1 (LGI1) and contactin-associated protein 2 (CASPR2), calcium channels such as the voltage-gated calcium channel (VGCC), or neurotransmitter receptors such as the α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR), gamma aminobutyric acid receptor (GABAR), and N-methyl-D-aspartate receptor (NMDAR). Diagnosis requires a syndrome consistent with an antibody identified in serum or cerebrospinal fluid (CSF) using methods that minimize risk of false-positives. Although there is no officially approved therapy for these disorders, typical approaches involve chronic high-dose steroids, intravenous immunoglobulin (IVIG), or plasma exchange. Rituximab is effective for antibody-associated disorders such as lupus, myasthenia gravis, and neuromyelitis optica. Here, we present three patients who were admitted with recalcitrant status epilepticus and demonstrated serum antibodies against NMDAR, LGI1, or VGCC using a cell-based assay. All patients demonstrated complete, long-term epilepsy control and improvement in symptoms with rituximab.
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spelling doaj-art-640415da24f84788ad13280618d030172025-02-03T05:53:53ZengWileyCase Reports in Neurological Medicine2090-66682090-66762020-01-01202010.1155/2020/58430895843089Rituximab for Autoimmune Encephalitis with EpilepsyMohankumar Kurukumbi0Rahul H. Dave1Jose Castillo2Tulsi Shah3Joanne Lau4Department of Neurology, Inova Fairfax Hospital, Falls Church, VA, USADepartment of Neurology, Inova Fairfax Hospital, Falls Church, VA, USAVirginia Commonwealth University School of Medicine, Inova Campus, Falls Church, VA, USAVirginia Commonwealth University School of Medicine, Inova Campus, Falls Church, VA, USAVirginia Commonwealth University School of Medicine, Inova Campus, Falls Church, VA, USAIntractable epilepsy remains a significant medical challenge, resulting in recurrent and prolonged intensive care unit (ICU) admissions. Autoimmune encephalitis is emerging as a treatable cause of intractable epilepsy. It is characterized by antibodies against cerebral antigens, such as potassium channels such as leucine-rich, glioma inactivated 1 (LGI1) and contactin-associated protein 2 (CASPR2), calcium channels such as the voltage-gated calcium channel (VGCC), or neurotransmitter receptors such as the α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor (AMPAR), gamma aminobutyric acid receptor (GABAR), and N-methyl-D-aspartate receptor (NMDAR). Diagnosis requires a syndrome consistent with an antibody identified in serum or cerebrospinal fluid (CSF) using methods that minimize risk of false-positives. Although there is no officially approved therapy for these disorders, typical approaches involve chronic high-dose steroids, intravenous immunoglobulin (IVIG), or plasma exchange. Rituximab is effective for antibody-associated disorders such as lupus, myasthenia gravis, and neuromyelitis optica. Here, we present three patients who were admitted with recalcitrant status epilepticus and demonstrated serum antibodies against NMDAR, LGI1, or VGCC using a cell-based assay. All patients demonstrated complete, long-term epilepsy control and improvement in symptoms with rituximab.http://dx.doi.org/10.1155/2020/5843089
spellingShingle Mohankumar Kurukumbi
Rahul H. Dave
Jose Castillo
Tulsi Shah
Joanne Lau
Rituximab for Autoimmune Encephalitis with Epilepsy
Case Reports in Neurological Medicine
title Rituximab for Autoimmune Encephalitis with Epilepsy
title_full Rituximab for Autoimmune Encephalitis with Epilepsy
title_fullStr Rituximab for Autoimmune Encephalitis with Epilepsy
title_full_unstemmed Rituximab for Autoimmune Encephalitis with Epilepsy
title_short Rituximab for Autoimmune Encephalitis with Epilepsy
title_sort rituximab for autoimmune encephalitis with epilepsy
url http://dx.doi.org/10.1155/2020/5843089
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AT josecastillo rituximabforautoimmuneencephalitiswithepilepsy
AT tulsishah rituximabforautoimmuneencephalitiswithepilepsy
AT joannelau rituximabforautoimmuneencephalitiswithepilepsy