Immune-mediated steroid-responsive epileptic spasms and epileptic encephalopathy associated with VGKC-complex antibodies

Dev Med Child Neurol. 2011 Nov;53(11):1058-60. doi: 10.1111/j.1469-8749.2011.04096.x. Epub 2011 Aug 30.


Autoantibodies that bind to voltage-gated potassium-channel complex proteins (VGKC-complex antibodies) occur frequently in adults with limbic encephalitis presenting with cognitive impairment and seizures. Recently, VGKC-complex antibodies have been described in a few children with limbic encephalitis, and children with unexplained encephalitis presenting with status epilepticus. We report a case of infantile-onset epileptic spasms and developmental delay compatible with epileptic encephalopathy. Our patient was a female infant, aged 4 months at presentation. She had evidence of immune activation in the central nervous system with elevated cerebrospinal fluid neopterin and mirrored oligoclonal bands, which prompted testing for autoantibodies. VGKC-complex antibodies were elevated (201 pmol/L, normal<100), but extended antibody testing, including leucine-rich glioma-inactivated 1 (LGI1) and contactin-associated protein 2 (CASPR2), was negative. The patient showed a partial response to steroid treatment, which was started late in the disease course. On review at 13 months of age, her development was consistent with an age of 5 to 6 months. These results suggest that VGKC-complex antibodies might represent a marker of immune therapy responsiveness in a subgroup of patients with infantile epileptic encephalopathy.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Autoantibodies / cerebrospinal fluid
  • Child, Preschool
  • Electroencephalography
  • Epilepsy* / complications
  • Epilepsy* / drug therapy
  • Epilepsy* / immunology
  • Female
  • Humans
  • Intellectual Disability* / complications
  • Intellectual Disability* / drug therapy
  • Intellectual Disability* / immunology
  • Lennox Gastaut Syndrome
  • Magnetic Resonance Imaging
  • Potassium Channels, Voltage-Gated / immunology*
  • Spasms, Infantile* / complications
  • Spasms, Infantile* / drug therapy
  • Spasms, Infantile* / immunology
  • Steroids / therapeutic use*


  • Autoantibodies
  • Potassium Channels, Voltage-Gated
  • Steroids

Supplementary concepts

  • Epileptic encephalopathy, Lennox-Gastaut type