Movement disorders in GLUT1 deficiency syndrome respond to the modified Atkins diet

Movement disorders are a prominent feature of glucose transporter‐1 (GLUT1) deficiency syndrome (GLUT1DS). First‐choice treatment is a ketogenic diet, but compliance is poor. We have investigated the effect of the modified Atkins diet as an alternative treatment for movement disorders in GLUT1DS.

[1]  Ron A. Wevers,et al.  Cerebrospinal Fluid Glucose and Lactate: Age-Specific Reference Values and Implications for Clinical Practice , 2012, PloS one.

[2]  J. Klepper.  GLUT1 deficiency syndrome in clinical practice , 2012, Epilepsy Research.

[3]  T. Bast,et al.  Glucose Transporter-1 (GLUT1) Deficiency Syndrome: Diagnosis and Treatment in Late Childhood , 2012, Neuropediatrics.

[4]  E. Kossoff,et al.  Long-Term Follow-Up of Children Treated With the Modified Atkins Diet , 2012, Journal of child neurology.

[5]  W. van Paesschen,et al.  Paroxysmal choreoathetosis/spasticity (DYT9) is caused by a GLUT1 defect , 2011, Neurology.

[6]  H. Oguni,et al.  A modified Atkins diet is promising as a treatment for glucose transporter type 1 deficiency syndrome , 2011, Developmental medicine and child neurology.

[7]  M. Vidailhet,et al.  Dystonic tremor caused by mutation of the glucose transporter gene GLUT1 , 2011, Journal of Inherited Metabolic Disease.

[8]  S. Berkovic,et al.  Mild adolescent/adult onset epilepsy and paroxysmal exercise‐induced dyskinesia due to GLUT1 deficiency , 2010, Epilepsia.

[9]  B. Weschke,et al.  Glucose transporter-1 deficiency syndrome: the expanding clinical and genetic spectrum of a treatable disorder. , 2010, Brain : a journal of neurology.

[10]  J. Hardy,et al.  GLUT1 gene mutations cause sporadic paroxysmal exercise‐induced dyskinesias , 2009, Movement disorders : official journal of the Movement Disorder Society.

[11]  L. Morrison,et al.  Optimal clinical management of children receiving the ketogenic diet: Recommendations of the International Ketogenic Diet Study Group , 2009, Epilepsia.

[12]  J. Klepper.  Glucose transporter deficiency syndrome (GLUT1DS) and the ketogenic diet , 2008, Epilepsia.

[13]  B. Castellotti,et al.  Paroxysmal movement disorders in GLUT1 deficiency syndrome , 2008, Neurology.

[14]  W. van Paesschen,et al.  Paroxysmal exercise-induced dyskinesia and epilepsy is due to mutations in SLC2A1, encoding the glucose transporter GLUT1 , 2008, Brain : a journal of neurology.

[15]  F. Mottaghy,et al.  GLUT1 mutations are a cause of paroxysmal exertion-induced dyskinesias and induce hemolytic anemia by a cation leak. , 2008, The Journal of clinical investigation.

[16]  H. Oguni,et al.  Modified Atkins diet therapy for a case with glucose transporter type 1 deficiency syndrome , 2008, Brain and Development.

[17]  E. Thiele,et al.  Atypical GLUT1 deficiency with prominent movement disorder responsive to ketogenic diet , 2006, Movement disorders : official journal of the Movement Disorder Society.

[18]  B. Robinson,et al.  Prenatal Diagnosis of Disorders of Energy Metabolism , 2001, Seminars in neurology.

[19]  J. Wheless.  The Ketogenic Diet: Fa(c)t or Fiction , 1995, Journal of child neurology.