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Published in: BMC Neurology 1/2020

01-12-2020 | Magnetic Resonance Imaging | Research article

Clinical characteristics and prognostic analysis of anti-gamma-aminobutyric acid-B (GABA-B) receptor encephalitis in Northeast China

Authors: Xinyue Zhang, Yue Lang, Lichao Sun, Weiguanliu Zhang, Weihong Lin, Li Cui

Published in: BMC Neurology | Issue 1/2020

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Abstract

Objective

To investigate the clinical characteristics and prognosis of anti-gamma-aminobutyric acid-B (GABA-B) receptor encephalitis.

Methods

This retrospective study enrolled nineteen patients with anti-GABA-B receptor encephalitis. Clinical manifestations, radiological and electroencephalogram features, treatment and outcomes were collected and analyzed. The neurological function was evaluated according to the modified Rankin Scale (mRS).

Results

There were eleven patients in the favorable-prognosis group (mRS ≤ 2) and eight patients in the poor-prognosis group (mRS > 2). In the favorable-prognosis group, clinical symptoms included memory deterioration (n = 10; 90.9%), epileptic seizures (n = 9; 81.8%), psychiatric disorders (n = 9; 81.8%), and conscious disturbance (n = 5; 45.5%); magnetic resonance imaging (MRI) indicated an involvement of the limbic system in three (27.3%) cases in this group. Lung cancer was detected in one patient (9.1%). After an average follow-up period of 11.7 months, four (36.4%) patients were cured, and seven (63.6%) patients showed significant improvements. In the poor-prognosis group, all patients presented with memory deterioration, epileptic seizures, psychiatric disorders, and conscious disturbance; five (62.5%) patients had convulsive status epilepticus, and five (62.5%) patients developed respiratory failure; MRI indicated an involvement of the limbic system in seven (87.5%) cases. Malignant tumors were detected in five (62.5%) patients. After an average follow-up period of 14.8 months, seven (87.5%) patients died and one (12.5%) patient remained dependent in daily life.

Conclusions

The clinical manifestations of anti-GABA-B receptor encephalitis include epileptic seizures, cognitive impairment and psychiatric disorders. Patients with convulsive status epilepticus or respiratory failure have poor outcomes. In anti-GABA-B receptor encephalitis, limbic system involvement is associated with a poor prognosis in and radiological examinations can reflect disease progression. Early diagnosis and appropriate treatment should be highlighted.
Literature
1.
go back to reference Vincent A. Potassium channel antibody-associated encephalopathy: a potentially immunotherapy-responsive form of limbic encephalitis. Brain. 2004;127(Pt 3):701–12.CrossRef Vincent A. Potassium channel antibody-associated encephalopathy: a potentially immunotherapy-responsive form of limbic encephalitis. Brain. 2004;127(Pt 3):701–12.CrossRef
2.
go back to reference Corsellis JAN, Goldberg GJ, Norton AR. "limbic encephalitis" and its association with carcinoma. Brain. 1968;91(3):481–96.CrossRef Corsellis JAN, Goldberg GJ, Norton AR. "limbic encephalitis" and its association with carcinoma. Brain. 1968;91(3):481–96.CrossRef
3.
go back to reference Bakheit AM, Kennedy PG, Behan PO. Paraneoplastic limbic encephalitis: clinico-pathological correlations. J Neurol Neurosurg Psychiatry. 1990;53(12):1084–8.CrossRef Bakheit AM, Kennedy PG, Behan PO. Paraneoplastic limbic encephalitis: clinico-pathological correlations. J Neurol Neurosurg Psychiatry. 1990;53(12):1084–8.CrossRef
4.
go back to reference Lancaster E, Martinez-Hernandez E, Dalmau J. Encephalitis and antibodies to synaptic and neuronal cell surface proteins. Neurology. 2011;77(2):179–89.CrossRef Lancaster E, Martinez-Hernandez E, Dalmau J. Encephalitis and antibodies to synaptic and neuronal cell surface proteins. Neurology. 2011;77(2):179–89.CrossRef
5.
go back to reference Hughes EG, Peng X, Gleichman AJ, et al. Cellular and synaptic mechanisms of anti-NMDA receptor encephalitis. J Neurosci. 2010;30(17):5866–75.CrossRef Hughes EG, Peng X, Gleichman AJ, et al. Cellular and synaptic mechanisms of anti-NMDA receptor encephalitis. J Neurosci. 2010;30(17):5866–75.CrossRef
6.
go back to reference Van Swieten J. Interobserver agreement for the assessment of handicap in stroke patients. Stroke. 1989;20(6):828.CrossRef Van Swieten J. Interobserver agreement for the assessment of handicap in stroke patients. Stroke. 1989;20(6):828.CrossRef
7.
go back to reference Lancaster E, Lai M, Peng X, et al. Antibodies to the GABA(B) receptor in limbic encephalitis with seizures: case series and characterisation of the antigen. Lancet Neurol. 2010;9(1):67–76.CrossRef Lancaster E, Lai M, Peng X, et al. Antibodies to the GABA(B) receptor in limbic encephalitis with seizures: case series and characterisation of the antigen. Lancet Neurol. 2010;9(1):67–76.CrossRef
8.
go back to reference Höftberger R, Titulaer MJ, Sabater L, et al. Encephalitis and GABAB receptor antibodies: novel findings in a new case series of 20 patients. Neurology. 2013;81(17):1500–6.CrossRef Höftberger R, Titulaer MJ, Sabater L, et al. Encephalitis and GABAB receptor antibodies: novel findings in a new case series of 20 patients. Neurology. 2013;81(17):1500–6.CrossRef
9.
go back to reference Boronat A, Sabater L, Saiz A, et al. GABA(B) receptor antibodies in limbic encephalitis and anti-GAD-associated neurologic disorders. Neurology. 2011;76(9):795.CrossRef Boronat A, Sabater L, Saiz A, et al. GABA(B) receptor antibodies in limbic encephalitis and anti-GAD-associated neurologic disorders. Neurology. 2011;76(9):795.CrossRef
10.
go back to reference Jarius S, Steinmeyer F, Knobel A, et al. GABAB receptor antibodies in paraneoplastic cerebellar ataxia. J Neuroimmunol. 2013;256(1–2):94–6.CrossRef Jarius S, Steinmeyer F, Knobel A, et al. GABAB receptor antibodies in paraneoplastic cerebellar ataxia. J Neuroimmunol. 2013;256(1–2):94–6.CrossRef
11.
go back to reference Jeffery OJ, Lennon VA, Pittock SJ, et al. GABAB receptor autoantibody frequency in service serologic evaluation. Neurology. 2013;81(10):882.CrossRef Jeffery OJ, Lennon VA, Pittock SJ, et al. GABAB receptor autoantibody frequency in service serologic evaluation. Neurology. 2013;81(10):882.CrossRef
12.
go back to reference Dogan Onugoren M, Deuretzbacher D, Haensch CA, et al. Limbic encephalitis due to GABAB and AMPA receptor antibodies: a case series. J Neurol Neurosurg Psychiatry. 2014;86(9):965–72.CrossRef Dogan Onugoren M, Deuretzbacher D, Haensch CA, et al. Limbic encephalitis due to GABAB and AMPA receptor antibodies: a case series. J Neurol Neurosurg Psychiatry. 2014;86(9):965–72.CrossRef
13.
go back to reference Kim TJ, Lee ST, Shin JW, et al. Clinical manifestations and outcomes of the treatment of patients with GABA B encephalitis. J Neuroimmunol. 2014;270(1–2):45–50.CrossRef Kim TJ, Lee ST, Shin JW, et al. Clinical manifestations and outcomes of the treatment of patients with GABA B encephalitis. J Neuroimmunol. 2014;270(1–2):45–50.CrossRef
14.
go back to reference Serafini A, Lukas RV, VanHaerents S, et al. Paraneoplastic epilepsy. Epilepsy Behavior E & B. 2016;61:51–8. Serafini A, Lukas RV, VanHaerents S, et al. Paraneoplastic epilepsy. Epilepsy Behavior E & B. 2016;61:51–8.
15.
go back to reference Mann EO, Kohl MM, Paulsen O. Distinct roles of GABA(a) and GABA(B) receptors in balancing and terminating persistent cortical activity. J Neurosci. 2009;29(23):7513–8.CrossRef Mann EO, Kohl MM, Paulsen O. Distinct roles of GABA(a) and GABA(B) receptors in balancing and terminating persistent cortical activity. J Neurosci. 2009;29(23):7513–8.CrossRef
16.
go back to reference Gelow J, Kruer M, Yadav V, et al. Apical ballooning resulting from limbic encephalitis. Am J Med. 2009;122(6):583–6.CrossRef Gelow J, Kruer M, Yadav V, et al. Apical ballooning resulting from limbic encephalitis. Am J Med. 2009;122(6):583–6.CrossRef
17.
go back to reference Mesulam MM, Mufson EJ. Insula of the old world monkey. III: efferent cortical output and comments on function. J Comp Neurol. 1982;212(1):38–52.CrossRef Mesulam MM, Mufson EJ. Insula of the old world monkey. III: efferent cortical output and comments on function. J Comp Neurol. 1982;212(1):38–52.CrossRef
18.
go back to reference Loftspring MC, Eric L, Lindsey W, et al. GABAB encephalitis: a fifty-two-year-old man with seizures, Dysautonomia, and acute heart failure. Case Reports Neurol Med. 2015;2015:812035.CrossRef Loftspring MC, Eric L, Lindsey W, et al. GABAB encephalitis: a fifty-two-year-old man with seizures, Dysautonomia, and acute heart failure. Case Reports Neurol Med. 2015;2015:812035.CrossRef
19.
go back to reference Labar D, Barrera J, Solomon G, et al. Nonconvulsive status Epilepticus in the elderly: a case series and a review of the literature. J Epilepsy. 1998;11(2):74–8.CrossRef Labar D, Barrera J, Solomon G, et al. Nonconvulsive status Epilepticus in the elderly: a case series and a review of the literature. J Epilepsy. 1998;11(2):74–8.CrossRef
20.
go back to reference Nosadini M, Mohammad SS, Ramanathan S, et al. Immune therapy in autoimmune encephalitis: a systematic review. Expert Rev Neurother. 2015;15(12):1391–419.CrossRef Nosadini M, Mohammad SS, Ramanathan S, et al. Immune therapy in autoimmune encephalitis: a systematic review. Expert Rev Neurother. 2015;15(12):1391–419.CrossRef
21.
go back to reference Vigevano F. Immuno- and antiinflammatory therapies in epileptic disorders. Epilepsia. 2011;52(s3):45–51.PubMed Vigevano F. Immuno- and antiinflammatory therapies in epileptic disorders. Epilepsia. 2011;52(s3):45–51.PubMed
22.
go back to reference Dubey D, Konikkara J, Modur PN, et al. Effectiveness of multimodality treatment for autoimmune limbic epilepsy. Epileptic Disorders. 2015;16(4):494–9. Dubey D, Konikkara J, Modur PN, et al. Effectiveness of multimodality treatment for autoimmune limbic epilepsy. Epileptic Disorders. 2015;16(4):494–9.
23.
go back to reference Mundiyanapurath S, Jarius S, Probst C, et al. GABA-B-receptor antibodies in paraneoplastic brainstem encephalitis. J Neuroimmunol. 2013;259(1–2):88–91.CrossRef Mundiyanapurath S, Jarius S, Probst C, et al. GABA-B-receptor antibodies in paraneoplastic brainstem encephalitis. J Neuroimmunol. 2013;259(1–2):88–91.CrossRef
Metadata
Title
Clinical characteristics and prognostic analysis of anti-gamma-aminobutyric acid-B (GABA-B) receptor encephalitis in Northeast China
Authors
Xinyue Zhang
Yue Lang
Lichao Sun
Weiguanliu Zhang
Weihong Lin
Li Cui
Publication date
01-12-2020
Publisher
BioMed Central
Published in
BMC Neurology / Issue 1/2020
Electronic ISSN: 1471-2377
DOI
https://doi.org/10.1186/s12883-019-1585-y

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