Selected Publications

Academic Article

Year Title Altmetric
2019 Utility and implications of exome sequencing in early-onset Parkinson’s diseaseMovement Disorders2019
2018 Cannabidiol improves frequency and severity of seizures and reduces adverse events in an open-label add-on prospective study.Epilepsy and Behavior2018
2018 Cannabidiol improves frequency and severity of seizures and reduces adverse events in an open-label add-on prospective study Pongkiat Kankirawatana, Dr Rani Singh, Professor David G Standaert, Dr Yuliang Liu, Dr Leon Dure, Dr Jennifer DeWollfe, Professor Gary Cutter, Dr Tyler Gaston, Dr Ashley Thomas, Professor E. Martina Bebin, Dr. Lawrence Ver HoefEpilepsy and Behavior.  (in press). 2018
2017 A randomized, double-blind, placebo-controlled trial of coenzyme Q10 in Huntington disease.Neurology.  88:152-159. 2017
2016 Physician Communication in Pediatric End-of-Life Care: A Simulation Study.American Journal of Hospice and Palliative Medicine.  33:935-941. 2016
2016 Clinical-Genetic Associations in the Prospective Huntington at Risk Observational Study (PHAROS): Implications for Clinical Trials.JAMA Neurology.  73:102-110. 2016
2014 Paroxysmal hypnogenic dyskinesia.Neurology.  82:1935. 2014
2014 UBQLN2 mutation causing heterogeneous X-linked dominant neurodegeneration.Annals of Neurology.  75:793-798. 2014
2013 Seizure occurrence following nonoptimal anticonvulsant medication management during the transition into the hospital.Journal of Child Neurology.  28:1250-1258. 2013
2013 Methodology of clinical research in rare diseases: development of a research program in juvenile neuronal ceroid lipofuscinosis (JNCL) via creation of a patient registry and collaboration with patient advocates.Controlled Clinical Trials.  35:48-54. 2013
2013 Anticonvulsant medication errors in children with epilepsy during the home-to-hospital transition.Journal of Child Neurology.  28:314-320. 2013
2013 Comprehensive behavioral intervention to improve occupational performance in children with Tourette disorder.American Journal of Occupational Therapy.  67:194-200. 2013
2012 Females experience a more severe disease course in batten diseaseJournal of Inherited Metabolic Disease.  35:549-555. 2012
2012 Pathologic features of dilated cardiomyopathy with localized noncompaction in a child with deletion 1p36 syndrome.Congenital Heart Disease.  7:59-61. 2012
2011 Quantifying physical decline in juvenile neuronal ceroid lipofuscinosis (Batten disease)Neurology.  77:1801-1807. 2011
2011 Streptococcal upper respiratory tract infections and exacerbations of tic and obsessive-compulsive symptoms: a prospective longitudinal study.Journal of the American Academy of Child and Adolescent Psychiatry.  50:108-118.e3. 2011
2009 Tremor in childhood.Seminars in Pediatric Neurology.  16:60-70. 2009
2008 A pilot assessment of parental practices and attitudes regarding risk disclosure and clinical research involving children in Huntington disease families.Genetics in Medicine.  10:811-819. 2008
2008 The social psychology of amateur ethicists: blood product recall notification and the value of reflexivity.Journal of Medical Ethics.  34:530-533. 2008
2008 Randomized controlled trial of ethyl-eicosapentaenoic acid in huntington diseaseArchives of Neurology -Chigago-.  65:1582-1589. 2008
2007 Predictors of diagnosis in Huntington disease.Neurology.  68:1710-1717. 2007
2007 Prospective Open-Label Clinical Trial of Trihexyphenidyl in Children With Secondary Dystonia due to Cerebral PalsyJournal of Child Neurology.  22:530-537. 2007
2007 The relationship between CAG repeat length and age of onset differs for Huntington's disease patients with juvenile onset or adult onset.Annals of Human Genetics.  71:295-301. 2007
2007 Nutritional vitamin D deficiency presenting as hemichorea.Journal of Child Neurology.  22:74-76. 2007
2006 Early progressive encephalopathy in boys and MECP2 mutations.Neurology.  67:164-166. 2006
2006 Pediatric neurotherapy.Neurorx.  3:131-132. 2006
2006 Standardized assessment of behavior and adaptive living skills in juvenile neuronal ceroid lipofuscinosis.Developmental Medicine and Child Neurology.  48:259-264. 2006
2006 Treatment of tics.Advances in Neurology -New York- Raven Press-.  99:191-196. 2006
2005 Atomoxetine treatment in children and adolescents with ADHD and comorbid tic disordersNeurology.  65:1941-1949. 2005
2005 Regarding “Antibiotic Prophylaxis with Azithromycin or Penicillin for Childhood-Onset Neuropsychiatric Disorders”Biological Psychiatry.  58:917-917. 2005
2005 Sequence Variants in SLITRK1 Are Associated with Tourette's SyndromeScience.  310:317-320. 2005
2005 A clinical rating scale for Batten disease: reliable and relevant for clinical trials.Neurology.  65:275-279. 2005
2005 Association between male gender and pediatric essential tremorMovement Disorders.  20:904-906. 2005
2005 Interrater agreement in the assessment of motor manifestations of Huntington's disease.Movement Disorders.  20:293-297. 2005
2004 Chorea as manifestation of epilepsia partialis continua in a child.Pediatric Neurology.  31:126-129. 2004
2004 Exclusive lower extremity mirror movements and diastematomyelia.Pediatric Neurosurgery.  40:132-135. 2004
2004 Venezuelan kindreds reveal that genetic and environmental factors modulate Huntington's disease age of onset.Proceedings of the National Academy of Sciences.  101:3498-3503. 2004
2003 Headache syndrome.Ophthalmology: Journal of The American Academy of Ophthalmology.  110:2257. 2003
2003 Paroxysmal Dyskinesias in Children.Current Treatment Options in Neurology.  5:275-278. 2003
2003 Predictors of nursing home placement in Huntington disease.Neurology.  60:998-1001. 2003
2003 Ophthalmologic involvement in the syndrome of headache, neurologic deficits, and cerebrospinal fluid lymphocytosis.Ophthalmology: Journal of The American Academy of Ophthalmology.  110:115-118. 2003
2003 Tic reduction in Tourette Syndrome: improvement with pergolide in a randomized controlled trial in childrenNeurology.  60:606-611. 2003
2002 Clinical and genetic heterogeneity in benign hereditary chorea.Neurology.  59:579-584. 2002
2002 Mutations in TITF-1 are associated with benign hereditary chorea.Human Molecular Genetics.  11:971-979. 2002
2002 Treatment of ADHD in children with tics: A randomized controlled trialNeurology.  58:527-536. 2002
2001 Essential tremor in childhood: A series of nineteen casesMovement Disorders.  16:921-923. 2001
2001 Medial medullary injury during adenoidectomy.Journal of Pediatrics.  138:772-774. 2001
2001 The epidemiology of tics and Tourette syndrome in children and adolescentsAdvances in Neurology -New York- Raven Press-.  85:261-271. 2001
2000 Mammal-like striatal functions in Anolis. II. Distribution of dopamine D(1) and D(2) receptors, and a laminar pattern of basal ganglia sub-systems.Brain, Behavior and Evolution.  56:249-258. 2000
1998 Paroxysmal dyskinesia in a patient with pseudohypoparathyroidism.Movement Disorders.  13:746-748. 1998
1998 Neonatal encephalopathy in two boys in families with recurrent Rett syndrome.Journal of Child Neurology.  13:229-231. 1998
1998 The genetic basis of Rett syndrome: candidate gene considerations.Molecular Genetics and Metabolism.  64:1-6. 1998
1997 Ectopically expressed CAG repeats cause intranuclear inclusions and a progressive late onset neurological phenotype in the mouse.Cell.  91:753-763. 1997
1997 Bobble-head doll syndrome: report of a case and review of the literature.Movement Disorders.  12:810-814. 1997
1997 Central nervous system infection associated with Bartonella quintana: a report of two cases.Pediatrics.  100:403-408. 1997
1997 Is cell death necessary for hippocampal mossy fiber sprouting?Epilepsy Research.  27:67-76. 1997
1997 Tourette syndrome.Current Opinion in Neurology.  10:153-159. 1997
1995 DNA fragmentation and immediate early gene expression in rat striatum following quinolinic acid administration.Experimental Neurology.  133:207-214. 1995
1995 Huntington's disease gene: regional and cellular expression in brain of normal and affected individuals.Annals of Neurology.  37:218-230. 1995
1994 IT15 gene expression in fetal human brain.Brain Research.  659:33-41. 1994
1994 Trinucleotide repeats in neurologic diseases: an hypothesis concerning the pathogenesis of Huntington's disease, Kennedy's disease, and spinocerebellar ataxia type I.Life Sciences.  54:1459-1464. 1994
1993 Trinucleotide repeat length instability and age of onset in Huntington's disease.Nature Genetics.  4:387-392. 1993
1992 Compartmentalization of excitatory amino acid receptors in human striatum.Proceedings of the National Academy of Sciences.  89:7688-7692. 1992
1992 Preferential loss of striato-external pallidal projection neurons in presymptomatic Huntington's disease.Annals of Neurology.  31:425-430. 1992
1992 Excitatory amino acid binding sites in the basal ganglia of the rat: a quantitative autoradiographic study.Neuroscience.  46:35-48. 1992
1991 Excitatory amino acid binding sites in the caudate nucleus and frontal cortex of Huntington's disease.Annals of Neurology.  30:785-793. 1991
1991 2,4,5-Trihydroxyphenylalanine (6-hydroxy-dopa) displaces [3H]AMPA binding in rat striatum.Neuroscience Letters.  132:55-58. 1991
1991 Excitatory amino acidergic pathways and receptors in the basal ganglia.Amino Acids.  1:339-350. 1991
1990 Excitatory amino acid binding sites in the periaqueductal gray of the rat.Neuroscience Letters.  118:112-115. 1990
1989 Chiari type I malformation in children.Journal of Pediatrics.  115:573-576. 1989
1985 Ribavirin small-particle aerosol treatment of infections caused by influenza virus strains A/Victoria/7/83 (H1N1) and B/Texas/1/84.Antimicrobial Agents and Chemotherapy.  27:309-313. 1985
1980 Covalent attachment of a progestational steroid to chick oviduct progesterone receptor by photoaffinity labellingNature.  283:784-786. 1980


Year Title Altmetric
2013 A multidisciplinary clinic for the management of Chiari i malformations.  345-350. 2013
2006 Movement disorders in childhood 2006
2006 Treatment of Tics 2006
2005 Tremor in childhood.  145-149. 2005
2004 The treatment of attention deficit disorder in Tourette Syndrome 2004
2002 DOPA-responsive dystonia 2002
2002 Paroxysmal movement disorders in childhood.  379-382. 2002
2000 Tourette syndrome and other tic disorders.  324-325. 2000
1999 Huntington's Disease.  90-91. 1999
1999 Sydenham's chorea.  216-217. 1999
1998 Neuropsychological Aspects of Movement Disorders in Children.  961-976. 1998
1996 Seizures and epilepsy.  613-624. 1996
1995 The distribution of glutamate receptor subtypes in mammalian central nervous system using quantitative in vitro autoradiography.  400-400. 1995
1992 Excitatory amino acid binding sites in the basal ganglia, primary visual cortex, and spinal cord.  179-188. 1992
1992 Excitatory amino acids in Huntington's disease.  217-222. 1992

Education And Training

  • Doctor of Medicine, Baylor College of Medicine
  • Full Name

  • Leon Dure
  • Blazerid

  • dure