A clinico-radiological phenotype of voltage-gated potassium channel complex antibody-mediated disorder presenting with seizures and basal ganglia changes

Yael Hacohen, Sukhvir Wright, Ata Siddiqui, Nikki Pandya, Jean Pierre Lin, Angela Vincent, Ming Lim*

*Corresponding author for this work

Research output: Contribution to journalArticle

Abstract

In childhood, central nervous system (CNS) presentations associated with antibodies to voltage-gated potassium channel (VGKC) complex include limbic encephalitis, status epilepticus, epileptic encephalopathy, and autistic regression. We report the cases of two individuals (a 6-year-old male and an 11-year-old female) who presented with an acute-onset explosive seizure disorder with positive VGKC complex antibodies and bilateral basal ganglia changes on magnetic resonance imaging (MRI). Both patients made a complete clinical recovery, without immunotherapy, with resolution of the MRI changes and normalization of the antibody levels. Extended antibody testing, including testing for leucine-rich glioma-inactivated 1 (LGI1), contactin-associated protein 2, and contactin-2 was negative. This could suggest that the clinico-radiological phenotype in our patients may in fact be associated with a novel autoreactive target(s) within the VGKC complex, as may be the case in other children with VGKC complex-mediated CNS disorders.

Original languageEnglish
Pages (from-to)1157-1159
Number of pages3
JournalDevelopmental Medicine and Child Neurology
Volume54
Issue number12
DOIs
Publication statusPublished - 1 Dec 2012

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