Work

Impaired intracortical inhibition demonstrated in vivo in people with Dravet syndrome

Year: 2017

Type: article

Abstract: OBJECTIVE: 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 no difference between Dravet and control groups. CONCLUSIONS: There were marked differences in inhibitory networks, detected using SICI paradigms, while other inhibitory and excitatory paradigms yielded normal results. These human data showing reduced GABAergic inhibition in vivo in people with Dravet syndrome support established animal models.

Source: Neurology

DOI: 10.1212/wnl.0000000000003868

Authors: William M. Stern, Josemir W. Sander, John C. Rothwell, Sanjay M. Sisodiya

Institutions: UCL Biomedical Research Centre, Stichting Epilepsie Instellingen Nederland, UCL Queen Square Institute of Neurology, University College London, Epilepsy Society

Language: English

Topic: Transcranial Magnetic Stimulation Studies

Subfield: Neurology

Field: Neuroscience

Domain: Life Sciences

SDG: Good health and well-being

Open Access status: hybrid

Estimated APC paid: $4,983