{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,29]],"date-time":"2025-10-29T06:08:17Z","timestamp":1761718097904},"reference-count":44,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2009,7,7]],"date-time":"2009-07-07T00:00:00Z","timestamp":1246924800000},"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":[[2010,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Objective<\/jats:title><jats:p>Glucose transporter type 1 deficiency syndrome (Glut1\u2010DS) is characterized clinically by acquired microcephaly, infantile\u2010onset seizures, psychomotor retardation, choreoathetosis, dystonia, and ataxia. The laboratory signature is hypoglycorrhachia. The 5\u2010hour oral glucose tolerance test (OGTT) was performed to assess cerebral function and systemic carbohydrate homeostasis during acute hyperglycemia, in the knowledge that GLUT1 is constitutively expressed ubiquitously and upregulated in the brain.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Thirteen Glut1\u2010DS patients completed a 5\u2010hour OGTT. Six patients had prolonged electroencephalographic (EEG)\/video monitoring, 10 patients had plasma glucose and serum insulin measurements, and 5 patients had repeated measures of attention, memory, fine motor coordination, and well\u2010being. All patients had a full neuropsychological battery prior to OGTT.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The glycemic profile and insulin response during the OGTT were normal. Following the glucose load, transient improvement of clinical seizures and EEG findings were observed, with the most significant improvement beginning within the first 30 minutes and continuing for 180 minutes. Thereafter, clinical seizures returned, and EEG findings worsened. Additionally, transient improvement in attention, fine motor coordination, and reported well\u2010being were observed without any change in memory performance.<\/jats:p><\/jats:sec><jats:sec><jats:title>Interpretation<\/jats:title><jats:p>This study documents transient neurological improvement in Glut1\u2010DS patients following acute hyperglycemia, associated with improved fine motor coordination and attention. Also, systemic carbohydrate homeostasis was normal, despite GLUT1 haploinsufficiency, confirming the specific role of GLUT1 as the transporter of metabolic fuel across the blood\u2010brain barrier. The transient improvement in brain function underscores the rate\u2010limiting role of glucose transport and the critical minute\u2010to\u2010minute dependence of cerebral function on fuel availability for energy metabolism. 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