Impaired intracortical inhibition demonstrated in vivo in people with Dravet syndrome.

ABSTRACT:

Objective

Dravet syndrome is a rare neurodevelopmental disorder characterized by seizures and other neurologic problems. SCN1A mutations account for ∼80% of cases. Animal studies have implicated mutation-related dysregulated cortical inhibitory networks in its pathophysiology. We investigated such networks in people with the condition.

Methods

Transcranial magnetic stimulation using single and paired pulse paradigms was applied to people with Dravet syndrome and to 2 control groups to study motor cortex excitability.

Results

Short interval intracortical inhibition (SICI), which measures GABAergic inhibitory network behavior, was undetectable in Dravet syndrome, but detectable in all controls. Other paradigms, including those testing excitatory networks, showed

SUBMITTER: Stern WM 

PROVIDER: S-EPMC5405762 | biostudies-literature | 2017 Apr

REPOSITORIES: biostudies-literature