We found a match
Your institution may have access to this item. Find your institution then sign in to continue.
- Title
Immune-mediated steroid-responsive epileptic spasms and epileptic encephalopathy associated with VGKC-complex antibodies.
- Authors
SULEIMAN, JEHAN; BRENNER, TANJA; GILL, DEEPAK; TROEDSON, CHRISTOPHER; SINCLAIR, ADRIANE J; BRILOT, FABIENNE; VINCENT, ANGELA; LANG, BETHAN; DALE, RUSSELL C
- Abstract
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 (201pmol/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.
- Subjects
AUTOANTIBODIES; ENCEPHALITIS; MILD cognitive impairment; SPASMS; JUVENILE diseases; NEOPTERIN; PATIENTS
- Publication
Developmental Medicine & Child Neurology, 2011, Vol 53, Issue 11, p1058
- ISSN
0012-1622
- Publication type
Article
- DOI
10.1111/j.1469-8749.2011.04096.x