Does Autoimmunity have a Role in Myoclonic Astatic Epilepsy? A Case Report of Voltage Gated Potassium Channel Mediated Seizures

Deepa Sirsi, Alison Dolce, Benjamin M Greenberg and Drew Thodeson

Department of Neurology, Neurotherapeutics and Pediatrics, UT Southwestern Medical Center, USA

^*Correspondance to: Deepa Sirsi 

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Abstract:

Background: There is expanding knowledge about the phenotypic variability of patients with voltage gated potassium channel complex (VGKC) antibody mediated neurologic disorders. The phenotypes are diverse and involve disorders of the central and peripheral nervous systems. The central nervous system manifestations described in the literature include limbic encephalitis, status epilepticus, and acute encephalitis. Patient Description: We report a 4.5 year-old boy who presented with intractable Myoclonic Astatic Epilepsy (MAE) or Doose syndrome and positive VGKC antibodies in serum. Treatment with steroids led to resolution of seizures and electrographic normalization. Conclusion: This case widens the spectrum of etiologies for MAE to include autoimmunity, in particular VGKC auto-antibodies and CNS inflammation, as a primary or contributing factor. There is an evolving understanding of voltage gated potassium channel complex mediated autoimmunity in children and the role of inflammation and autoimmunity in MAE and other intractable pediatric epilepsy syndromes remains to be fully defined. A high index of suspicion is required for diagnosis and appropriate management of antibody mediated epilepsy syndromes.

Keywords:

VGKC antibody; Myoclonic astatic epilepsy; Doose syndrome; Autoimmune epilepsy; Generalized epilepsy

Cite the Article:

Sirsi D, Dolce A, Greenberg BM, Thodeson D. Does Autoimmunity have a Role in Myoclonic Astatic Epilepsy? A Case Report of Voltage Gated Potassium Channel Mediated Seizures. Ann Clin Case Rep. 2016; 1: 1178.