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Patients with seizures and antibodies to LGI1 or CASPR2

Investigators. Dr Sophie Binks, Prof Sarosh R Irani

Case definition / Inclusion criteria. Patients with seizures – including Faciobrachial dystonic seizures (FBDS) - and antibodies to LGI1 or CASPR2 (or the VGKC-complex if LGI1/CASPR2 not tested)

Background

  • The antibodies

VGKC-complex antibodies are found in non-autoimmune and autoimmune neurological syndromes. Antibodies against LGI1 and CASPR2 are much more specifically found in patients with autoimmune syndromes. 1,2 While VGKC-complex antibodies are still available for diagnostic testing in routine clinical practice, this practice should be phased out as LGI1 and CASPR2 antibodies offer far greater clinical utility.3 However, as VGKC-complex antibodies are still often requested through tradition, we have included these patients in our criteria.

  • The syndromes

Seizures are a cardinal, albeit not universal, feature of patients with antibodies against LGI1 or CASPR2. Faciobrachial dystonic seizures (FBDS) are increasingly recognized as a distinctive phenotype, and associate with LGI1-antibodies.4

Research aim

Our aim is to characterize the seizures, the associated cognitive phenotypes, responses to therapies and the blood-based biomarkers in these patients.

We aim to perform retrospective telephone or face-to-face interviews with patients and relatives, complete neuropsychological questionnaires and - if possible - obtain blood.

We can offer travel expenses for those who can visit Oxford.

Methods

  1. We would be grateful if you could offer your patient the opportunity to participate in the research, and, if they are keen to do so, ask them to email or telephone Dr Binks.
  2. Dr Binks can then send them the informed consent form and participant information sheet.

 

Reporting Instructions

Please contact Dr Sophie Binks about suitable cases.

References

1.    Irani SR, Alexander S, Waters P, et al. Antibodies to Kv1 potassium channel-complex proteins leucine-rich, glioma inactivated 1 protein and contactin-associated protein-2 in limbic encephalitis, Morvan's syndrome and acquired neuromyotonia. Brain 2010;133(9):2734–2748.

2.    Lai M, Huijbers MG, Lancaster E, et al. Investigation of LGI1 as the antigen in limbic encephalitis previously attributed to potassium channels: a case series. Lancet Neurol 2010;9(8):776–785.

3.    Graus F, Gorman MP. Voltage-gated potassium channel antibodies: Game over. Neurology 2016;86(18):1657–1658.

4.    Irani SR, Michell AW, Lang B, et al. Faciobrachial dystonic seizures precede Lgi1 antibody limbic encephalitis. Ann Neurol. 2011;69(5):892–900.

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