John Libbey Eurotext

Epileptic Disorders

The Educational Journal of the

Myoclonic status epilepticus as a presentation of caspr2 antibody-associated autoimmune encephalitis Volume 16, numéro 4, December 2014

Vidéo

  • Myoclonic status epilepticus as a presentation of caspr2 antibody-associated autoimmune encephalitis

Illustrations

  • Figure 1
Auteurs
1 Department of Neurology, Westmead Hospital, and Sydney Medical School, University of Sydney
2 Neuroimmunology group, Institute for Neuroscience and Muscle Research, The Kids Research Institute at the Children's Hospital at Westmead
3 Department of Immunology, Westmead Hospital, Sydney, Australia
* Correspondence: Andrew F Bleasel Department of Neurology, Westmead Hospital, Westmead NSW 2145, Australia
  • Mots-clés : autoimmune encephalitis, autoimmune epilepsy, VGKC complex associated encephalitis, caspr2 antibodies, myoclonic status epilepticus
  • DOI : 10.1684/epd.2014.0707
  • Page(s) : 477-81
  • Année de parution : 2014

We present a case of autoimmune encephalitis associated with antibodies targeting contactin-associated protein-like 2. This case is notable because of the presentation with myoclonic status epilepticus and the prolonged clinical course of refractory seizures, which are demonstrated in the accompanying videos, and not previously associated with this condition. Treatment with prednisone, intravenous immunoglobulin, plasma exchange, rituximab, cyclophosphamide, and mycophenolate mofetil resulted in significant functional improvement. Historically, myoclonic status epilepticus is associated with a grave prognosis and minimal chance of meaningful recovery. This case demonstrates that autoimmune encephalitis remains an important differential diagnosis in patients with such a presentation, and that early recognition and the appropriate institution of immunotherapy can result in seizure control and functional recovery. [Published with video sequences]