{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T05:56:09Z","timestamp":1790747769328,"version":"4.1.0"},"reference-count":70,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2009,3,26]],"date-time":"2009-03-26T00:00:00Z","timestamp":1238025600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Annals of Neurology"],"published-print":{"date-parts":[[2009,8]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Objective<\/jats:title><jats:p>The mechanisms of epileptogenesis in Sturge\u2010Weber syndrome (SWS) are unknown. We explored the properties of neurons from human pediatric SWS cortex <jats:italic>in vitro<\/jats:italic> and tested in particular whether gamma\u2010aminobutyric acid (GABA) excites neurons in SWS cortex, as has been suggested for various types of epilepsies.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Patch\u2010clamp and field potential recordings and dynamic biphoton imaging were used to analyze cortical tissue samples obtained from four 6\u2010 to 14\u2010month\u2010old pediatric SWS patients during surgery.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Neurons in SWS cortex were characterized by a relatively depolarized resting membrane potential, as was estimated from cell\u2010attached recordings of N\u2010methyl\u2010D\u2010aspartate channels. Many cells spontaneously fired action potentials at a rate proportional to the level of neuronal depolarization. The reversal potential for GABA\u2010activated currents, assessed by cell\u2010attached single channel recordings, was close to the resting membrane potential. All spontaneously firing neurons recorded in cell\u2010attached mode or imaged with biphoton microscopy were inhibited by GABA. Spontaneous epileptiform activity in the form of recurrent population bursts was suppressed by glutamate receptor antagonists, the GABA(A) receptor agonist isoguvacine, and the positive allosteric GABA(A) modulator diazepam. Blockade of GABA(A) receptors aggravated spontaneous epileptiform activity. The NKCC1 antagonist bumetanide had little effect on epileptiform activity.<\/jats:p><\/jats:sec><jats:sec><jats:title>Interpretation<\/jats:title><jats:p>SWS cortical neurons have a relatively depolarized resting membrane potential and spontaneously fire action potentials that may contribute to increased network excitability. In contrast to previous data depicting excitatory and proconvulsive actions of GABA in certain pediatric and adult epilepsies, GABA plays mainly an inhibitory and anticonvulsive role in SWS pediatric cortex. 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