Course and outcome of acute limbic encephalitis with negative voltage-gated potassium channel antibodies
- 1Royal Victoria Infirmary, Newcastle upon Tyne, UK
- 2Department of Clinical Neurology, University of Oxford, Oxford, UK
- 3Department of Neuropsychology, Newcastle General Hospital, Newcastle upon Tyne, UK
- 4Department of Neurology, Newcastle General Hospital, Newcastle upon Tyne, UK
- 5Department of Neurology, Royal Victoria Infirmary, Newcastle upon Tyne, UK
- 6Newcastle University Medical School, Framlington Place, Newcastle upon Tyne, UK
- Correspondence to: Professor T D Griffiths Newcastle University Medical School, Framlington Place, Newcastle upon Tyne NE2 4HH, UK;
- Received 29 March 2006
- Accepted 23 October 2006
- Revised 10 October 2006
- Published Online First 10 November 2006
Background: Limbic encephalitis is a potentially treatable immunological condition. The presence of voltage-gated potassium channel antibodies (VGKC-Ab) in the cerebrospinal fluid (CSF) and serum of patients with the condition is a marker of the disease associated with a non-paraneoplastic form and good response to treatment. Recent work has highlighted absent serum VGKC-Ab and distinct immunology in patients with the paraneoplastic form of limbic encephalitis.
Methods: The cases of four patients with the typical clinical presentation, neuropsychological features and brain imaging of acute limbic encephalitis, in the absence of any evidence for associated cancer during a follow-up of at least 18 months are described here.
Results: All patients had negative testing for VGKC-Ab measured during their acute presentation. All patients made some recovery, although they were left with marked cognitive deficits and persistent seizures.
Conclusion: These cases demonstrate that the absence of VGKC-Ab in limbic encephalitis does not necessarily imply a paraneoplastic form. Further work is required to establish the immunological basis for the disorder in these patients, and the optimal treatment regimen.
- CPS, complex partial seizures
- CSF, cerebrospinal fluid
- VGKC-Ab, voltage-gated potassium channel antibodies
Published Online First 10 November 2006
Competing interests: None.