An effective immunotherapy regimen for VGKC antibody-positive limbic encephalitis

Department of Neurology, The Walton Centre for Neurology and Neurosurgery, Liverpool, UK.
Journal of neurology, neurosurgery, and psychiatry (Impact Factor: 6.81). 10/2010; 81(10):1167-9. DOI: 10.1136/jnnp.2009.178293
Source: PubMed


Voltage-gated potassium channel antibody-positive limbic encephalitis (VGKC+LE) frequently improves with immunotherapy, although the optimum regimen is unknown. The effectiveness of a combination immunomodulatory regimen was tested in consecutive VGKC+LE patients.
This was an open-label prospective study of nine VGKC+LE patients. All patients had plasma exchange (50 ml/kg), intravenous immunoglobulin (2 g/kg) and intravenous methylprednisolone (1 g×3), followed by maintenance oral prednisolone (1 mg/kg/day). Mycophenolate (2 g/day) was used in the first three patients. Assessments included serial clinical, cognitive, brain MRI and VGKC antibody testing.
Within 1 week, seizures and hyponatraemia remitted in all affected patients. Cognitive function improved in all patients within 3 months. MRI appearances improved substantially within 9 months, with remission of inflammation in the majority of patients. All achieved immunological remission with normal VGKC antibody titres within 1-4 months. Major adverse events of therapy included one septicaemia and one thrombosis on plasma exchange and one death from sepsis after incidental bowel surgery. One patient remains in remission after 40 months of follow up, 26 months after being off all treatment.
Our immunotherapy regimen was effective for the treatment of the clinical, cognitive and immunological features of VGKC+LE. Radiological improvement was seen in the majority. Pending randomised controlled trials, this regimen is proposed for the treatment of VGKC+LE.

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    • "The patients typically present after an insidious onset with rapidly progressive cognitive decline, frequently with a fluctuating course and inflammatory cerebrospinal fluid findings [4,5,6]. These patients often have autoantibody markers, including anti-cation channel complex antibodies, and respond to immunosuppression [5,7,8]. However, autoimmune phenomena are common in the normal adult population, particularly if defined as the presence of antibodies against cellular components. "
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    ABSTRACT: Background Autoimmunity is considered an uncommon but under-recognised cause of cognitive decline. Methods Serum samples from 3,253 randomly selected subjects enrolled in the Hunter Community Study, aged 55-85 years, were assayed for thyrotropin stimulatory hormone, anti-thyroid peroxidase antibodies (TPO-Ab), anti-nuclear antibodies (ANA) and extractable nuclear antigens (ENA). Cognitive function was assessed using the Audio Recorded Cognitive Screen (ARCS) tool. Results TPO-Ab were found in 8.4% and ANA in 27.9% of the study population, of whom 3% had positive ENA findings. No relationship was found between the ARCS score and either TPO-Ab (coefficient = 0.133; 95% CI −0.20, 0.82, p = 0.616), ANA at a low (coefficient = 1.01; 95% CI −2.58, 0.55, p = 0.203) or a high titre (coefficient = −0.65; 95% CI −2.59, 1.28, p = 0.508), or ENA antibodies (coefficient = 5.12; 95% CI −0.53, 10.77; p = 0.076). Conclusions Autoantibody findings are common in an aging population and are not associated with cognitive decline.
    05/2014; 4(2):140-6. DOI:10.1159/000362716
    • "Optimum treatment of VGKC-Abs associated LE has not been studied in prospective randomized studies. Wong et al.[13] in an open-label prospective study of 9 patients described a combination of immuno-modulatory treatment in patients with VGKC-Abs associated LE. All patients had plasma exchange, immunoglobulin and methyprednisolone in the acute phase, followed by oral prednisolone. "
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    ABSTRACT: Autoimmune limbic encephalitis (LE) associated with voltage gated potassium channel antibodies (VGKC-Abs) in children is more common than previously thought and is not always paraneoplastic. Non-neoplastic, autoimmune LE associated with VGKC-Abs has been described recently. However, only few case reports in children as the disease is predominantly described in the adult population. It is likely that this type of autoimmune encephalitis is currently under-diagnosed and hence, under-treated, especially in children. We present a 13-year-old previously fit and healthy African girl diagnosed with LE and we reviewed the literature for its current management.
    Annals of Indian Academy of Neurology 10/2013; 16(4):593-6. DOI:10.4103/0972-2327.120483 · 0.60 Impact Factor
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    • "Plasma treatment was used in a few patients with steroid responsive encephalopathy associated with autoimmune thyroiditis (SREAT) (also referred as Hashimoto's encephalitis) who did not benefit from corticosteroids (Nagpal & Pande, 2004; Hussain et al., 2005). More recently, experiences with plasma treatment have been increasingly reported in patients with seizure disorders with auto-Abs to specific neuronal proteins, such as voltage-gated potassium channels (VGKCs), N-methyl-Daspartate (NMDA) receptors, and glutamic acid decarboxylase (GAD) (Vincent et al., 2004; Florance et al., 2009; Wong et al., 2010). In most case series, likely due to the severity of these syndromes, plasma treatment was carried out in association with other immunotherapy (usually steroids or sequential IVIGs) and the proper effect of the different treatments cannot be exactly specified. "
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    ABSTRACT: Several experimental and clinical studies demonstrated an immunologic basis for different forms of epilepsy. A wide range of immune abnormalities have been reported suggesting the existence of various subtypes of epileptic syndromes with different immunopathogenetic mechanisms. This evidence gives rise to the development of immunologic and immunomodulatory treatments such as usage of steroids, plasmapheresis, and intravenous immunoglobulins, which will be discussed briefly in this article.
    Epilepsia 05/2011; 52 Suppl 3(s3):45-51. DOI:10.1111/j.1528-1167.2011.03036.x · 4.57 Impact Factor
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