TY - JOUR
T1 - Antiglutamic acid decarboxylase 65 (GAD65) antibody-associated epilepsy
AU - Daif, Ahmad
AU - Lukas, Rimas Vincas
AU - Issa, Naoum P.
AU - Javed, Adil
AU - VanHaerents, Stephen Anthony
AU - Reder, Anthony T.
AU - Tao, James X.
AU - Warnke, Peter
AU - Rose, Sandra
AU - Towle, Vernon L.
AU - Wu, Shasha
PY - 2018/3/1
Y1 - 2018/3/1
N2 - Glutamic acid decarboxylase (GAD) antibody-associated encephalitis causes both acute seizures and chronic epilepsy with predominantly temporal lobe onset. This condition is challenging in diagnosis and management, and the incidence of GAD antibody (Ab)-related epilepsy could be much higher than commonly believed. Imaging and CSF evidence of inflammation along with typical clinical presentations, such as adult onset temporal lobe epilepsy (TLE) with unexplained etiology, should prompt testing for the diagnostic antibodies. High serum GAD Ab titer (≥ 2000 U/mL or ≥ 20 nmol/L) and evidence of intrathecal anti-GAD Ab synthesis support the diagnosis. Unlike other immune-mediated epilepsies, antiglutamic acid decarboxylase 65 (GAD65) antibody-mediated epilepsy is often poorly responsive to antiepileptic drugs (AEDs) and only moderately responsive to immune therapy with steroids, intravenous immunoglobulin (IVIG), or plasma exchange (PLEX). Long-term treatment with more aggressive immunosuppressants such as rituximab (RTX) and/or cyclophosphamide is often necessary and may be more effective than current immunosuppressive approaches. The aim of this review is to review the physiology, pathology, clinical presentation, related ancillary tests, and management of GAD Ab-associated autoimmune epilepsy by searching the keywords and to promote the recognition and the initiation of proper therapy for this condition.
AB - Glutamic acid decarboxylase (GAD) antibody-associated encephalitis causes both acute seizures and chronic epilepsy with predominantly temporal lobe onset. This condition is challenging in diagnosis and management, and the incidence of GAD antibody (Ab)-related epilepsy could be much higher than commonly believed. Imaging and CSF evidence of inflammation along with typical clinical presentations, such as adult onset temporal lobe epilepsy (TLE) with unexplained etiology, should prompt testing for the diagnostic antibodies. High serum GAD Ab titer (≥ 2000 U/mL or ≥ 20 nmol/L) and evidence of intrathecal anti-GAD Ab synthesis support the diagnosis. Unlike other immune-mediated epilepsies, antiglutamic acid decarboxylase 65 (GAD65) antibody-mediated epilepsy is often poorly responsive to antiepileptic drugs (AEDs) and only moderately responsive to immune therapy with steroids, intravenous immunoglobulin (IVIG), or plasma exchange (PLEX). Long-term treatment with more aggressive immunosuppressants such as rituximab (RTX) and/or cyclophosphamide is often necessary and may be more effective than current immunosuppressive approaches. The aim of this review is to review the physiology, pathology, clinical presentation, related ancillary tests, and management of GAD Ab-associated autoimmune epilepsy by searching the keywords and to promote the recognition and the initiation of proper therapy for this condition.
KW - Autoimmune encephalitis
KW - Autoimmune epilepsy
KW - Limbic encephalitis
KW - Paraneoplastic limbic encephalitis
UR - http://www.scopus.com/inward/record.url?scp=85042934151&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=85042934151&partnerID=8YFLogxK
U2 - 10.1016/j.yebeh.2018.01.021
DO - 10.1016/j.yebeh.2018.01.021
M3 - Review article
C2 - 29433947
AN - SCOPUS:85042934151
SN - 1525-5050
VL - 80
SP - 331
EP - 336
JO - Epilepsy and Behavior
JF - Epilepsy and Behavior
ER -