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JAMA Neurol. 2018 Jan 1;75(1):65-71. doi: 10.1001/jamaneurol.2017.3196.

Seizures and Encephalitis in Myelin Oligodendrocyte Glycoprotein IgG Disease vs Aquaporin 4 IgG Disease.

Author information

1
Department of Neurology, The Walton Centre NHS Foundation Trust, Liverpool, England.
2
University of Liverpool School of Medicine, Liverpool, England.
3
Magnetic Resonance and Image Analysis Research Centre, University of Liverpool, Liverpool, England.
4
Institute of Infection and Global Health, University of Liverpool, The Walton Centre NHS Foundation Trust, Liverpool, England.
5
Autoimmune Neurology Diagnostic Laboratory, Nuffield Department of Clinical Neurosciences, John Radcliffe Hospital, Oxford, England.
6
Department of Neurology, Alder Hey Children's Hospital, Liverpool, England.
7
Department of Neurology, Royal Victoria Infirmary, Newcastle, Newcastle upon Tyne, England.

Abstract

Importance:

Antibodies to myelin oligodendrocyte glycoprotein IgG (MOG-IgG) are increasingly detected in patients with non-multiple sclerosis-related demyelination, some of whom manifest a neuromyelitis optica (NMO) phenotype. Cortical involvement, encephalopathy, and seizures are rare in aquaporin 4 antibody (AQP4-IgG)-related NMO in the white European population. However, the authors encountered several patients with seizures associated with MOG-IgG disease.

Objective:

To compare incidence of seizures and encephalitis-like presentation, or both between AQP4-IgG-positive and MOG-IgG-positive patients.

Design, Setting, and Participants:

Retrospective case series of all patients who were seropositive for MOG-IgG (nā€‰=ā€‰34) and the last 100 patients with AQP4-IgG disease (NMO spectrum disorder) seen in the NMO service between January 2013 and December 2016, and analysis was completed January 4, 2017. All patients were seen in a tertiary neurological center, The Walton Centre NHS Foundation Trust in Liverpool, England.

Main Outcomes and Measures:

The difference in seizure frequency between the AQP4-IgG-positive and MOG-IgG-positive patient groups was determined.

Results:

Thirty-four patients with MOG-IgG disease (20 female) with a median age at analysis of 30.5 years (interquartile range [IQR], 15-69 years), and 100 AQP4-IgG-positive patients (86 female) with a median age at analysis of 54 years (IQR, 12-91 years) were studied. Most patients were of white race. Five of the 34 patients with MOG-IgG (14.7%) had seizures compared with 1 patient with AQP4-IgG (2-sided Pā€‰<ā€‰.008, Fisher test). On magnetic resonance imaging, all 5 MOG-IgG-positive patients had inflammatory cortical brain lesions associated with the seizures. In 3 of the 5 MOG-IgG-positive patients, seizures occurred as part of the index event. Four of the 5 presented with encephalopathy and seizures, and disease relapsed in all 5 patients. Four of these patients were receiving immunosuppressant medication at last follow-up, and 3 continued to take antiepileptic medication. In contrast, the only AQP4-IgG-positive patient with seizures had a diagnosis of complex partial epilepsy preceding the onset of NMO by several years and experienced no encephalitic illness; her magnetic resonance imaging results demonstrated no cortical, subcortical, or basal ganglia involvement.

Conclusions and Relevance:

Patients with MOG-IgG-associated disease were more likely to have seizures and encephalitis-like presentation than patients with AQP4-IgG-associated disease.

PMID:
29131884
PMCID:
PMC5833490
[Available on 2018-11-13]
DOI:
10.1001/jamaneurol.2017.3196

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