Syndrome and outcome of antibody-negative limbic encephalitis

J 2018

Syndrome and outcome of antibody-negative limbic encephalitis

GRAUS, F., D. ESCUDERO, L. OLEAGA, J. BRUNA, A. VILLAREJO-GALENDE et. al.

Basic information

Original name

Syndrome and outcome of antibody-negative limbic encephalitis

Authors

GRAUS, F. (724 Spain, guarantor), D. ESCUDERO (724 Spain), L. OLEAGA (724 Spain), J. BRUNA (724 Spain), A. VILLAREJO-GALENDE (724 Spain), J. BALLABRIGA (724 Spain), M.I. BARCELO (724 Spain), F. GILO (724 Spain), S. POPKIROV (276 Germany), Pavel ŠTOURAČ (203 Czech Republic, belonging to the institution) and J. DALMAU (840 United States of America)

Edition

European Journal of Neurology, Oxford, Rapid Science Ltd. 2018, 1351-5101

Other information

Language

English

Type of outcome

Článek v odborném periodiku

Field of Study

30210 Clinical neurology

Country of publisher

United States of America

Confidentiality degree

není předmětem státního či obchodního tajemství

Impact factor

Impact factor: 4.387

RIV identification code

RIV/00216224:14110/18:00105960

Organization unit

Faculty of Medicine

DOI

http://dx.doi.org/10.1111/ene.13661

UT WoS

000437832600011

Keywords in English

antibodies; autoimmune; limbic encephalitis; paraneoplastic

Abstract

V originále

Background and purposeThe aim was to report the clinical characteristics of 12 patients with limbic encephalitis (LE) who were antibody-negative after a comprehensive immunological study. MethodsThe clinical records of 163 patients with LE were reviewed. Immunohistochemistry on rat brain, cultured neurons and cell-based assays were used to identify neuronal autoantibodies. Patients were included if (i) there was adequate clinical, cerebrospinal fluid (CSF) and magnetic resonance imaging information to classify the syndrome as LE, (ii) magnetic resonance images were accessible for central review and (iii) serum and CSF were available and were confirmed negative for neuronal antibodies. ResultsTwelve (7%) of 163 LE patients [median age 62 years; range 40-79; 9 (75%) male] without neuronal autoantibodies were identified. The most frequent initial complaints were deficits in short-term memory leading to hospital admission in a few weeks (median time 2 weeks; range 0.5-12). In four patients the short-term memory dysfunction remained as an isolated symptom during the entire course of the disease. Seizures, drowsiness and psychiatric problems were unusual. Four patients had solid tumors (one lung, one esophagus, two metastatic cervical adenopathies of unknown primary tumor) and one chronic lymphocytic leukemia. CSF showed pleocytosis in seven (58%) with a median of 13 white blood cells/mm(3) (range 9-25). Immunotherapy included corticosteroids, intravenous immunoglobulins and combinations of both drugs or with rituximab. Clinical improvement occurred in six (54%) of 11 assessable patients. ConclusionsDespite the discovery of new antibodies, 7% of LE patients remain seronegative. Antibody-negative LE is more frequent in older males and usually develops with predominant or isolated short-term memory loss. Despite the absence of antibodies, patients may have an underlying cancer and respond to immunotherapy. Click for the corresponding questions to this CME article.

Citovat

GRAUS, F., D. ESCUDERO, L. OLEAGA, J. BRUNA, A. VILLAREJO-GALENDE, J. BALLABRIGA, M.I. BARCELO, F. GILO, S. POPKIROV, Pavel ŠTOURAČ and J. DALMAU. Syndrome and outcome of antibody-negative limbic encephalitis. European Journal of Neurology. Oxford: Rapid Science Ltd., 2018, vol. 25, No 8, p. 1011-1016. ISSN 1351-5101. Available from: https://dx.doi.org/10.1111/ene.13661.

@article{1491932,
   author = {Graus, F. and Escudero, D. and Oleaga, L. and Bruna, J. and VillarejoandGalende, A. and Ballabriga, J. and Barcelo, M.I. and Gilo, F. and Popkirov, S. and Štourač, Pavel and Dalmau, J.},
   article_location = {Oxford},
   article_number = {8},
   doi = {http://dx.doi.org/10.1111/ene.13661},
   keywords = {antibodies; autoimmune; limbic encephalitis; paraneoplastic},
   language = {eng},
   issn = {1351-5101},
   journal = {European Journal of Neurology},
   title = {Syndrome and outcome of antibody-negative limbic encephalitis},
   volume = {25},
   year = {2018}
}

TY  - JOUR
ID  - 1491932
AU  - Graus, F. - Escudero, D. - Oleaga, L. - Bruna, J. - Villarejo-Galende, A. - Ballabriga, J. - Barcelo, M.I. - Gilo, F. - Popkirov, S. - Štourač, Pavel - Dalmau, J.
PY  - 2018
TI  - Syndrome and outcome of antibody-negative limbic encephalitis
JF  - European Journal of Neurology
VL  - 25
IS  - 8
SP  - 1011-1016
EP  - 1011-1016
PB  - Rapid Science Ltd.
SN  - 13515101
KW  - antibodies
KW  - autoimmune
KW  - limbic encephalitis
KW  - paraneoplastic
N2  - Background and purposeThe aim was to report the clinical characteristics of 12 patients with limbic encephalitis (LE) who were antibody-negative after a comprehensive immunological study. MethodsThe clinical records of 163 patients with LE were reviewed. Immunohistochemistry on rat brain, cultured neurons and cell-based assays were used to identify neuronal autoantibodies. Patients were included if (i) there was adequate clinical, cerebrospinal fluid (CSF) and magnetic resonance imaging information to classify the syndrome as LE, (ii) magnetic resonance images were accessible for central review and (iii) serum and CSF were available and were confirmed negative for neuronal antibodies. ResultsTwelve (7%) of 163 LE patients [median age 62 years; range 40-79; 9 (75%) male] without neuronal autoantibodies were identified. The most frequent initial complaints were deficits in short-term memory leading to hospital admission in a few weeks (median time 2 weeks; range 0.5-12). In four patients the short-term memory dysfunction remained as an isolated symptom during the entire course of the disease. Seizures, drowsiness and psychiatric problems were unusual. Four patients had solid tumors (one lung, one esophagus, two metastatic cervical adenopathies of unknown primary tumor) and one chronic lymphocytic leukemia. CSF showed pleocytosis in seven (58%) with a median of 13 white blood cells/mm(3) (range 9-25). Immunotherapy included corticosteroids, intravenous immunoglobulins and combinations of both drugs or with rituximab. Clinical improvement occurred in six (54%) of 11 assessable patients. ConclusionsDespite the discovery of new antibodies, 7% of LE patients remain seronegative. Antibody-negative LE is more frequent in older males and usually develops with predominant or isolated short-term memory loss. Despite the absence of antibodies, patients may have an underlying cancer and respond to immunotherapy. Click for the corresponding questions to this CME article.
ER  -

GRAUS, F., D. ESCUDERO, L. OLEAGA, J. BRUNA, A. VILLAREJO-GALENDE, J. BALLABRIGA, M.I. BARCELO, F. GILO, S. POPKIROV, Pavel ŠTOURAČ and J. DALMAU. Syndrome and outcome of antibody-negative limbic encephalitis. \textit{European Journal of Neurology}. Oxford: Rapid Science Ltd., 2018, vol.~25, No~8, p.~1011-1016. ISSN~1351-5101. Available from: https://dx.doi.org/10.1111/ene.13661.

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