Encephalitis and GABAB receptor antibodies

Novel findings in a new case series of 20 patients

Romana Höftberger, Maarten J. Titulaer, Lidia Sabater, B. Döme, Anita Rózsás, B. Hegedűs, Mir Alireza Hoda, Viktoria Laszlo, Hendrik Jan Ankersmit, Lutz Harms, Sabas Boyero, Alicia De Felipe, Albert Saiz, Josep Dalmau, Francesc Graus

Research output: Contribution to journalArticle

178 Citations (Scopus)

Abstract

Objective: To report the clinical features of 20 newly diagnosed patients with GABAB receptor (GABABR) antibodies and determine the frequency of associated tumors and concurrent neuronal autoantibodies. Methods: Clinical data were retrospectively obtained and evaluated. Serum and CSF samples were examined for additional antibodies using methods previously reported. Results: Seventeen patients presented with seizures, memory loss, and confusion, compatible with limbic encephalitis (LE), one patient presented with ataxia, one patient presented with status epilepticus, and one patient presented with opsoclonus-myoclonus syndrome (OMS). Nineteen (95%) patients eventually developed LE during the course of the disease. Small-cell lung cancer (SCLC) was identified in 10 (50%) patients, all with LE. Treatment and outcome was available from 19 patients: 15 showed complete (n = 7) or partial (n = 8) neurologic improvement after steroids, IV immunoglobulins, or plasma exchange and oncologic treatment when indicated; 1 patient died of tumor progression shortly after the first cycle of immunotherapy, and 3 were not treated. Five patients with SCLC had additional onconeuronal antibodies (Ri, amphiphysin, or SOX1), and 2 without tumor had GAD65 and NMDAR antibodies, respectively. GABABR antibodies were not detected in serum of 116 patients with SCLC without neurologic symptoms. Conclusion: Our study confirms GABABR as an autoantigen of paraneoplastic and nonparaneoplastic LE and expands the phenotype of GABABR antibodies to ataxia, OMS, and status epilepticus. The long-term prognosis is dictated by the presence of a tumor. Recognition of syndromes associated with GABABR antibodies is important because they usually respond to treatment.

Original languageEnglish
Pages (from-to)1500-1506
Number of pages7
JournalNeurology
Volume81
Issue number17
DOIs
Publication statusPublished - Oct 22 2013

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Encephalitis
Antibodies
Limbic Encephalitis
Small Cell Lung Carcinoma
Opsoclonus-Myoclonus Syndrome
Status Epilepticus
Ataxia
Neoplasms
Case Series
Confusion
Plasma Exchange
Intravenous Immunoglobulins
Memory Disorders
Autoantigens
Neurologic Manifestations
Serum
Autoantibodies
Immunotherapy
Nervous System
Seizures

ASJC Scopus subject areas

  • Clinical Neurology
  • Arts and Humanities (miscellaneous)

Cite this

Encephalitis and GABAB receptor antibodies : Novel findings in a new case series of 20 patients. / Höftberger, Romana; Titulaer, Maarten J.; Sabater, Lidia; Döme, B.; Rózsás, Anita; Hegedűs, B.; Hoda, Mir Alireza; Laszlo, Viktoria; Ankersmit, Hendrik Jan; Harms, Lutz; Boyero, Sabas; De Felipe, Alicia; Saiz, Albert; Dalmau, Josep; Graus, Francesc.

In: Neurology, Vol. 81, No. 17, 22.10.2013, p. 1500-1506.

Research output: Contribution to journalArticle

Höftberger, R, Titulaer, MJ, Sabater, L, Döme, B, Rózsás, A, Hegedűs, B, Hoda, MA, Laszlo, V, Ankersmit, HJ, Harms, L, Boyero, S, De Felipe, A, Saiz, A, Dalmau, J & Graus, F 2013, 'Encephalitis and GABAB receptor antibodies: Novel findings in a new case series of 20 patients', Neurology, vol. 81, no. 17, pp. 1500-1506. https://doi.org/10.1212/WNL.0b013e3182a9585f
Höftberger, Romana ; Titulaer, Maarten J. ; Sabater, Lidia ; Döme, B. ; Rózsás, Anita ; Hegedűs, B. ; Hoda, Mir Alireza ; Laszlo, Viktoria ; Ankersmit, Hendrik Jan ; Harms, Lutz ; Boyero, Sabas ; De Felipe, Alicia ; Saiz, Albert ; Dalmau, Josep ; Graus, Francesc. / Encephalitis and GABAB receptor antibodies : Novel findings in a new case series of 20 patients. In: Neurology. 2013 ; Vol. 81, No. 17. pp. 1500-1506.
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AU - Rózsás, Anita

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AU - Hoda, Mir Alireza

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