TY - JOUR
T1 - Successful treatment of acute autoimmune limbic encephalitis with negative VGKC and NMDAR antibodies: a case report
AU - Modoni, Anna
AU - Masciullo, Marcella
AU - Spinelli, Pietro
AU - Marra, Camillo
AU - Tartaglione, Tommaso
AU - Andreetta, Francesca
AU - Tonali, Pietro Attilio
AU - Silvestri, Gabriella
PY - 2009
Y1 - 2009
N2 - OBJECTIVE: To describe a case of acute nonherpetic limbic encephalitis (LE) with negative testing for antibodies directed against onconeuronal and cell membrane antigens, including voltage-gated potassium channels and N-methyl-D-aspartate receptor, that showed a dramatic response to immune therapy.\r\n\r\nMATERIALS AND METHODS: A 30-year-old woman manifested generalized seizures, altered consciousness, and memory impairment shortly after a prodromal viral illness. Few days later the patient developed a drug-resistant epileptic status.\r\n\r\nRESULTS: Electroencephalograph showed bitemporal slowing and paroxysmal slow wave bursts. Brain magnetic resonance imaging showed bilateral swelling in the medial temporal lobes. Cerebrospinal fluid analysis ruled out viral etiologies. A diagnostic search for cancer, including serum testing for known onconeuronal antibodies proved negative. Screening for cell membrane antigen antibodies, including voltage-gated potassium channels and N-methyl-D-aspartate receptor, was also negative. Suspecting an autoimmune etiology, we started an immunomodulatory treatment with intravenous immunoglobulin followed by a short course of oral prednisone, obtaining a full clinical recovery.\r\n\r\nCONCLUSIONS: Our report confirms previous observations of "seronegative" autoimmune LE, suggesting the presence of other, still unknown central nervous system antigens representing a target of a postinfectious, autoimmune response in these patients. Moreover, it emphasizes the importance of early recognition and treatment of acute autoimmune LE, to reduce the risk of intensive care unit-related complications and the occurrence of permanent cognitive or behavioral defects.
AB - OBJECTIVE: To describe a case of acute nonherpetic limbic encephalitis (LE) with negative testing for antibodies directed against onconeuronal and cell membrane antigens, including voltage-gated potassium channels and N-methyl-D-aspartate receptor, that showed a dramatic response to immune therapy.\r\n\r\nMATERIALS AND METHODS: A 30-year-old woman manifested generalized seizures, altered consciousness, and memory impairment shortly after a prodromal viral illness. Few days later the patient developed a drug-resistant epileptic status.\r\n\r\nRESULTS: Electroencephalograph showed bitemporal slowing and paroxysmal slow wave bursts. Brain magnetic resonance imaging showed bilateral swelling in the medial temporal lobes. Cerebrospinal fluid analysis ruled out viral etiologies. A diagnostic search for cancer, including serum testing for known onconeuronal antibodies proved negative. Screening for cell membrane antigen antibodies, including voltage-gated potassium channels and N-methyl-D-aspartate receptor, was also negative. Suspecting an autoimmune etiology, we started an immunomodulatory treatment with intravenous immunoglobulin followed by a short course of oral prednisone, obtaining a full clinical recovery.\r\n\r\nCONCLUSIONS: Our report confirms previous observations of "seronegative" autoimmune LE, suggesting the presence of other, still unknown central nervous system antigens representing a target of a postinfectious, autoimmune response in these patients. Moreover, it emphasizes the importance of early recognition and treatment of acute autoimmune LE, to reduce the risk of intensive care unit-related complications and the occurrence of permanent cognitive or behavioral defects.
KW - NMDAR Ab
KW - VGKC antibodies
KW - limbic encephalitis
KW - NMDAR Ab
KW - VGKC antibodies
KW - limbic encephalitis
UR - https://publicatt.unicatt.it/handle/10807/31879
UR - https://www.scopus.com/inward/citedby.uri?partnerID=HzOxMe3b&scp=67049107961&origin=inward
UR - https://www.scopus.com/inward/record.uri?partnerID=HzOxMe3b&scp=67049107961&origin=inward
U2 - 10.1097/WNN.0b013e318190d195
DO - 10.1097/WNN.0b013e318190d195
M3 - Article
SN - 1543-3633
VL - 22
SP - 63
EP - 66
JO - Cognitive and Behavioral Neurology
JF - Cognitive and Behavioral Neurology
IS - 1
ER -