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Immune-mediated steroid-responsive epileptic spasms and epileptic encephalopathy associated with VGKC-complex antibodies.
- Source :
- Developmental Medicine & Child Neurology; Nov2011, Vol. 53 Issue 11, p1058-1060, 3p
- Publication Year :
- 2011
-
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. [ABSTRACT FROM AUTHOR]
Details
- Language :
- English
- ISSN :
- 00121622
- Volume :
- 53
- Issue :
- 11
- Database :
- Complementary Index
- Journal :
- Developmental Medicine & Child Neurology
- Publication Type :
- Academic Journal
- Accession number :
- 66716221
- Full Text :
- https://doi.org/10.1111/j.1469-8749.2011.04096.x