An effective immunotherapy regimen for VGKC antibody-positive limbic encephalitis
- 1Department of Neurology, The Walton Centre for Neurology and Neurosurgery, Liverpool, UK
- 2Department of Neuroradiology, The Walton Centre for Neurology and Neurosurgery, Liverpool, UK
- Correspondence to Dr Sui Wong, Neurology Specialist Registrar, The Walton Centre for Neurology and Neurosurgery, Lower Lane, Liverpool L9 7LJ, UK;
Contributors All authors contributed to the writing of the manuscript. Dr Ian Hart designed this study and supervised the manuscript writting process.
- Received 17 March 2009
- Revised 29 May 2009
- Accepted 1 June 2009
- Published Online First 26 July 2010
Background 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.
Methods 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.
Results 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.
Conclusions 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.
- Addenbrookes Cognitive Examination
- Addenbrookes Cognitive Examination-Revised
- intravenous immunoglobulins
- plasma exchange
- voltage-gated potassium channel antibody-positive limbic encephalitis
↵* Dr Ian Hart died on 10 November 2008
Competing interests None.
Patient consent Obtained.
provenance and peer review Not commissioned; externally peer reviewed.