Use of dietary therapies amongst patients with GLUT1 deficiency syndrome

Seizure : the Journal of the British Epilepsy Association
Hannah R KassEric H Kossoff

Abstract

GLUT-1 deficiency syndrome (GLUT1DS) is a neurologic disorder manifesting as epilepsy, abnormal movements, and cognitive delay. The currently accepted treatment of choice is the classic 4:1 ratio ketogenic diet. A 2-page survey was distributed to all attendees of a family-centered conference for GLUT1DS in July 2015. The surveys were completed by parents, collected anonymously, and information analyzed in a database. Surveys were received from 92 families, of which 90 (98%) had been treated with dietary therapies. Diets used were extremely varied: 59 were treated with the classic ketogenic diet (KD), 29 with the Modified Atkins Diet (MAD), 4 with the Medium-chain Triglyceride (MCT) Diet and 2 with the low glycemic index treatment. The mean diet duration was 5.5 years (range: 1 month-20 years). Of those with seizures, 95% of the children had >50% seizure reduction and 80% had >90% seizure reduction. Children who were seizure-free were currently younger on average (8.2 vs. 11.6 years, p=0.01) and slightly younger at GLUT1DS diagnosis (3.8 vs. 5.3 years, p=0.05). There was an equal percentage of children seizure-free receiving the KD/MCT Diets compared to the MAD/Low Glycemic Index Treatment (74% vs. 63%, p=0.30). The majority (64...Continue Reading

References

Sep 9, 2005·Journal of Cellular Biochemistry·Hei Yi WongYuan Yuan Ho
Aug 28, 2007·Developmental Medicine and Child Neurology·Joerg Klepper, Baerbel Leiendecker
Feb 25, 2009·Epilepsy & Behavior : E&B·Gerhard Luef, Markus Rauchenzauner
Aug 12, 2009·Biochimica Et Biophysica Acta·Kate Petersen ShayTory M Hagen
Mar 9, 2011·Epilepsy Research·Joerg Klepper
Apr 20, 2011·Developmental Medicine and Child Neurology·Yasushi ItoMakiko Osawa
Jul 21, 2012·Epilepsia·Amanda W PongDarryl C De Vivo
Oct 31, 2012·Epilepsia·Todor ArsovIngrid E Scheffer
Feb 28, 2013·Current Neurology and Neuroscience Reports·Toni S PearsonDarryl C De Vivo
Mar 2, 2013·Developmental Medicine and Child Neurology·Anette Ramm-PettersenKaja K Selmer
May 15, 2013·Journal of Child Neurology·Joerg Klepper, Baerbel Leiendecker
Jan 5, 2014·Epilepsy & Behavior : E&B·Eric H KossoffZahava Turner
Dec 10, 2014·Brain & Development·Yasushi ItoHirokazu Oguni
Jan 8, 2015·Seizure : the Journal of the British Epilepsy Association·Valentina De GiorgisPierangelo Veggiotti
Jun 1, 2015·Brain Research·Andrea ViggianoGiangennaro Coppola

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Citations

Oct 28, 2016·Expert Review of Neurotherapeutics·Philipp Sebastian ReifKarl Martin Klein
Feb 18, 2017·Current Opinion in Neurology·Joseph D SymondsMichael R Johnson
Apr 23, 2017·Brain & Development·Se Hee KimHeung Dong Kim
Jan 6, 2018·International Journal of Molecular Sciences·Armond DaciShaip Krasniqi
Dec 9, 2017·Epilepsy Currents·Tanya J W McDonald, Mackenzie C Cervenka
Jan 31, 2019·Zhurnal nevrologii i psikhiatrii imeni S.S. Korsakova·D A TiulganovaG A Zavyalov
Feb 5, 2019·Brain and Behavior·Valentina De GiorgisPierangelo Veggiotti
Feb 9, 2019·Journal of Inherited Metabolic Disease·Alfonso Oyarzabal, Isaac Marin-Valencia
Mar 29, 2019·Movement Disorders : Official Journal of the Movement Disorder Society·Shekeeb S MohammadRussell C Dale
Aug 6, 2019·JAMA Neurology·Hugh KearneyNorman Delanty
Jan 26, 2020·Neurotherapeutics : the Journal of the American Society for Experimental NeuroTherapeutics·Pasquale Striano, Berge A Minassian
Jul 16, 2019·Brain : a Journal of Neurology·Joseph D SymondsMargaret Wilson
Sep 3, 2016·European Journal of Pediatrics·Ludovica PascaPierangelo Veggiotti
Sep 2, 2017·Movement Disorders : Official Journal of the Movement Disorder Society·H A JinnahUNKNOWN International Parkinson's Disease Movement Disorders Society Task Force on Rare Movement Disorders
Jul 25, 2019·Nutrients·Costanza VaresioValentina De Giorgis
Jul 13, 2020·Journal of Inherited Metabolic Disease·Cezar Gavrilovici, Jong M Rho
Jun 28, 2019·Der Nervenarzt·Ulrike B S HedrichHolger Lerche
May 23, 2017·Movement Disorders Clinical Practice·Emil K GustavssonJan O Aasly
Oct 22, 2020·Zhurnal nevrologii i psikhiatrii imeni S.S. Korsakova·R G GamirovaR G Esin
Oct 23, 2020·Expert Review of Neurotherapeutics·Cécile DelormeEmmanuel Roze
Mar 29, 2019·Emerging Topics in Life Sciences·Laura A TsengClara D M van Karnebeek
Nov 7, 2020·Seminars in Neurology·Tanya J W McDonald, Mackenzie C Cervenka
Aug 15, 2020·Saudi Pharmaceutical Journal : SPJ : the Official Publication of the Saudi Pharmaceutical Society·Faleh AlqahtaniMohammed Mufadhe Alanazi
Mar 26, 2021·Developmental Medicine and Child Neurology·Audrey K S SooManju A Kurian
Jul 14, 2021·EBioMedicine·Oyarzábal AGarcía-Cazorla A
Jul 15, 2021·The New England Journal of Medicine·Phil E M Smith

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