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Special Articles   |    
Treatment of VGKC Complex Antibody-Associated Limbic Encephalitis: A Systematic Review
Guirindhra Koumar Radja, M.Sc.; Andrea Eugenio Cavanna, M.D., Ph.D.
The Journal of Neuropsychiatry and Clinical Neurosciences 2013;25:264-271. doi:10.1176/appi.neuropsych.13020022
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From the South Staffordshire and Shropshire NHS Foundation Trust UK (GK), The Michael Trimble Neuropsychiatry Research Group, University of Birmingham and BSMHFT, Birmingham, UK (AEC), Sobell Department of Motor Neuroscience and Movement Disorders, UCL and Institute of Neurology, London, UK (AEC).

Correspondence: Andrea Eugenio Cavanna, M.D., Ph.D.; e-mail: a.cavanna@ion.ucl.ac.uk

Copyright © 2013 by the American Psychiatric Association

Received February 03, 2013; Revised February 26, 2013; Accepted February 27, 2013.

Abstract

Limbic encephalitis is an autoimmune neuropsychiatric condition characterized by subacute cognitive symptoms, seizures, and affective changes. Although limbic encephalitis is usually caused by an immune reaction secondary to neoplasms, different types of potentially treatable non-paraneoplastic limbic encephalitis (nPLE) have recently been described. In particular, published studies have reported variable responses to immunosuppressive therapy in Voltage-Gated Potassium Channel (VGKC) complex antibody-associated nPLE. This systematic literature review found that the most significant improvements were reported by patients presenting with affective symptoms and consistent neuroradiological changes. In these patients, improved clinical outcomes correlated with the largest decreases in antibody titers.

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FIGURE 1. Age Distribution of the Reviewed Cases of VGKC-Associated Limbic Encephalitis
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TABLE 1.Bien and Elger’s Diagnostic Criteria3 for Limbic Encephalitis
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TABLE 2.Summary of the Reviewed Studies on the Treatment of VGKC-Associated Limbic Encephalitis
Table Footer Note

The overall outcome was rated as “++” when there was significant improvement in the clinical picture according to clinician’s observations/ratings, “+” when the improvement was minimal, “=” when there was no change, and “−” when the patient showed clinical deterioration.

Table Footer Note

IVIG: intravenous immunoglobulin; PEX: plasma exchange.

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