Paul H Gordon
- Amyotrophic lateral sclerosis: pathophysiology, diagnosis and managementPaul H Gordon
Fédération des Maladies du Système Nerveux, Centre référent maladie rare SLA, Assistance Publique Hopitaux de Paris, Hopital de la Pitie Salpetriere, Paris, France
CNS Drugs 25:1-15. 2011..Novel therapies currently in trials include potential neuroprotective agents with differing mechanisms of action, vaccine therapies, stem cell injections and diaphragmatic pacing...
- Amyotrophic lateral sclerosis and the clinical potential of dexpramipexolePhilippe Corcia
Centre SLA, CHRU de Tours, Tours, France UMR INSERM U930, Université François Rabelais de Tours PC, Tours, France
Ther Clin Risk Manag 8:359-66. 2012..Early phase trials in human ALS suggest that the drug can be taken safely by patients in doses that provide neuroprotection in preclinical models. A Phase III trial to test the efficacy of RPPX in ALS is underway...
- Predicting survival of patients with amyotrophic lateral sclerosis at presentation: a 15-year experiencePaul H Gordon
UMR_S 708, UPMC Universite Paris 06, Paris, France
Neurodegener Dis 12:81-90. 2013..To describe the clinical features at first evaluation that best predict survival of the amyotrophic lateral sclerosis (ALS) population from the Salpêtrière Hospital between 1995 and 2009...
- Improving survival in a large French ALS center cohortPaul H Gordon
INSERM, Unité U708, F 75013, Neuroépidémiologie, Hopital de la Pitie Salpetriere, 47 Boulevard de l Hopital, 75651, Paris Cedex 13, France
J Neurol 259:1788-92. 2012..At this large ALS center, survival improved after 2006. Because more aggressive use of NIV was the principal therapeutic adaptation, our data suggest that better survival resulted from improved respiratory care...
- Defining survival as an outcome measure in amyotrophic lateral sclerosisPaul H Gordon
Department of Neurology, Columbia University, New York, New York, USA
Arch Neurol 66:758-61. 2009..To examine how respiratory interventions affect survival as an outcome measure and to define survival rate for trials in amyotrophic lateral sclerosis...
- Clinical features that distinguish PLS, upper motor neuron-dominant ALS, and typical ALSP H Gordon
Department of Neurology, Columbia University, New York, NY, USA
Neurology 72:1948-52. 2009..To determine how clinical features at the first evaluation and in follow-up can be used to suggest a diagnostic outcome for patients with only upper motor neuron (UMN) signs at disease onset...
- A placebo arm is not always necessary in clinical trials of amyotrophic lateral sclerosisPaul H Gordon
The Eleanor and Lou Gehrig MDA ALS Research Center, Department of Neurology, Columbia University Medical Center, 710 West 168th Street, New York, New York 10032, USA
Muscle Nerve 39:858-60. 2009..Dose-ranging, futility, and selection trials are examples of Phase I and II trial designs that can be conducted without placebo groups...
- Progression in ALS is not linear but is curvilinearPaul H Gordon
Fédération des Maladies du Système Nerveux, AP HP, Centre référent maladie rare SLA, Hopital de la Pitie Salpetriere, 47 83, Boulevard de l Hopital, 75651, Paris, France
J Neurol 257:1713-7. 2010..Older age and bulbar signs are associated with a steeper decline, and along with more rapid initial rate of decline, but not current functional status, also predict survival...
- The range and clinical impact of cognitive impairment in French patients with ALS: a cross-sectional study of neuropsychological test performancePaul H Gordon
AP HP, Centre référent maladies rares SLA, Departement de Neurologie
Amyotroph Lateral Scler 12:372-8. 2011..As in other causes of dementia, higher education attainment may protect against clinical cognitive deterioration in ALS. French patients with severe cognitive impairment have shorter survival time...
- Changing mortality for motor neuron disease in France (1968-2007): an age-period-cohort analysisPaul H Gordon
INSERM, Unité U708, Neuroépidémiologie, Hopital de la Pitie Salpetriere, 47 Boulevard de l Hopital, 75651 Paris Cedex 13, France
Eur J Epidemiol 26:729-37. 2011..Changing environmental exposures may be a possible explanation and these findings warrant the continued search for environmental risk factors for MND...
- A prospective cohort study of neuropsychological test performance in ALSPaul H Gordon
Department of Neurology, Columbia University, New York, NY, USA
Amyotroph Lateral Scler 11:312-20. 2010..The strongest neuropsychological predictors of cognitive status were measures of executive, episodic memory and language function. Cognitively impaired patients had shorter survival time...
- Efficacy of minocycline in patients with amyotrophic lateral sclerosis: a phase III randomised trialPaul H Gordon
Department of Neurology, Columbia University, New York, NY, USA
Lancet Neurol 6:1045-53. 2007..Several trials are planned or are in progress to assess whether minocycline slows human neurodegeneration. We aimed to test the efficacy of minocycline as a treatment for amyotrophic lateral sclerosis (ALS)...
- Outcome measures for early phase clinical trialsPaul H Gordon
Department of Neurology, The Eleanor and Lou Gehrig MDA ALS Research Center, College of Physicians and Surgeons, Mailman School of Public Health, Columbia University, New York, NY 10032, USA
Amyotroph Lateral Scler 8:270-3. 2007..The ALSFRS-R most strongly predicted survival and provided the most complete data, but large changes may be necessary before patients perceive treatment effects...
- A novel, efficient, randomized selection trial comparing combinations of drug therapy for ALSPaul H Gordon
Department of Neurology, Columbia University, New York, USA
Amyotroph Lateral Scler 9:212-22. 2008..This phase II design was efficient, leading to treatment selection after just 60 patients, and can be used in other phase II trials to assess different agents...
- A screening assessment of cognitive impairment in patients with ALSPaul H Gordon
Departmen of Neurology, Columbia University, New York, USA
Amyotroph Lateral Scler 8:362-5. 2007..Behavioral and motor impairment is associated with depressive symptoms in caregivers. Studies with formal neuropsychological tests are needed to determine the sensitivity and specificity of the screen in ALS...
- An observational study of cognitive impairment in amyotrophic lateral sclerosisGregory A Rippon
Department of Neurology, Gertrude H Sergievsky Center, Taub Institute for Research on Alzheimer s Disease and the Aging Brain, Eleanor and Lou Gehrig MDA ALS Research Center, New York, NY, USA
Arch Neurol 63:345-52. 2006..Clinical and pathologic features overlap in frontotemporal lobar dementia and ALS. Demographics, respiratory status, bulbar site of onset, and disease severity are potential risk factors for cognitive impairment in ALS...
- Motor unit number estimation (MUNE) in diseases of the motor neuron: utility and comparative analysis in a multimodal biomarker studyClifton L Gooch
Department of Neurology, University of South Florida College of Medicine, Tampa, FL 33612, USA
Suppl Clin Neurophysiol 60:153-62. 2009..These features make MUNE an important tool for both the study of the pathophysiology of the motor neuron diseases, as well as an important measure for incorporation into future clinical trials...
- Excellent inter-rater, intra-rater, and telephone-administered reliability of the ALSFRS-R in a multicenter clinical trialPetra Kaufmann
Department of Neurology, The Eleanor and Lou Gehrig MDA ALS Research Center, College of Physicians and Surgeons, New York, NY, USA
Amyotroph Lateral Scler 8:42-6. 2007..97 (95% CI 0.93-0.98). We conclude that the ALSFRS-R shows excellent inter- and intra-rater reliability, and reliability of telephone administration when used as primary outcome measure in a multicenter ALS trial...
- The Timed Up and Go test: predicting falls in ALSJacqueline Montes
Department of Neurology, College of Physicians and Surgeons, The Eleanor and Lou Gehrig MDA ALS Research Center, Columbia University, New York, NY 10032, USA
Amyotroph Lateral Scler 8:292-5. 2007..The TUG test can guide management of patients with ALS; a time of 14 s can be used to prompt the recommendation for mobility aids to prevent falls...
- Modafinil treatment of fatigue in patients with ALS: a placebo-controlled studyJudith G Rabkin
Department of Psychiatry, Columbia University, New York, New York, USA
Muscle Nerve 39:297-303. 2009..The number needed to treat was 1.6 (ITT). No medically serious adverse events were reported. Modafinil may be a promising intervention for fatigue in ALS patients. Replication in a larger study is needed...
- Impaired glucose tolerance in patients with amyotrophic lateral sclerosisPierre Francois Pradat
APHP, Hopital de la Pitie Salpetriere, Fédération des Maladies du Système Nerveux, Paris, France
Amyotroph Lateral Scler 11:166-71. 2010..The origin of glucose homeostasis abnormalities in ALS may be multifactorial and deserves further investigation...
- Woman with a 26-year history of parkinsonism, supranuclear ophthalmoplegia, and loss of postural reflexesPaul H Gordon
Department of Neurology, Division of Movement Disorders, Columbia Presbyterian Medical Center, New York, New York 10032, USA
Mov Disord 19:950-61. 2004..A woman who had her first serious fall at age 48 years subsequently developed neurological symptoms. Her case is described, differential diagnoses are presented, pathology is discussed, and a final diagnosis is reached...
- Amyotrophic lateral sclerosis with ragged-red fibersMichio Hirano
Department of Neurology, Columbia University Medical Center, 3 317 Russ Berrie Medical Science Pavilion, 1150 St Nicholas Ave, New York, NY 10032, USA
Arch Neurol 65:403-6. 2008..Motor neuron diseases (amyotrophic lateral sclerosis [ALS] and spinal muscular atrophy [SMA]) have been rarely associated with mitochondrial respiratory chain defects...
- How can we improve clinical trials in amyotrophic lateral sclerosis?Paul H Gordon
Département des Maladies du Système Nerveux, Assistance Publique Hopitaux de Paris, Hopital de la Salpetriere, 47 Boulevard de l Hopital, 75013 Paris, France
Nat Rev Neurol 7:650-4. 2011..These areas include the relationships between different disease mechanisms, the challenges presented by the heterogeneity of the disease, and the need for early intervention, optimal dose selection and effective biomarkers...
- The importance of the SMN genes in the genetics of sporadic ALSPhilippe Corcia
ALS Centre, CHU Tours, INSERM, Universite Francois Rabelais, 2 boulevard Tonnelle, Tours Cedex 1, France
Amyotroph Lateral Scler 10:436-40. 2009..The existence of abnormal SMN1 copy numbers in ALS provides additional evidence that gene copy number variants may contribute to neurodegeneration and might open new approaches to treatment...
- Selecting promising ALS therapies in clinical trialsYing Kuen Cheung
Department of Biostatistics, Columbia University Mailman School of Public Health, New York, NY 10032, USA
Neurology 67:1748-51. 2006....
- Olfactory ensheathing glia injections in Beijing: misleading patients with ALSSheena Chew
Department of Neurology, Eleanor and Lou Gehrig MDA ALS Research Center, Columbia University, New York, NY 10032, USA
Amyotroph Lateral Scler 8:314-6. 2007..Clinics that give experimental and potentially harmful treatments outside the construct of well-designed clinical trials put patients at risk and do a disservice to the ALS community...
- Lou Gehrig, rawhide, and 1938Melissa Lewis
Eleanor and Lou Gehrig MDA ALS Research Center, Department of Neurology, Columbia University, New York, NY, USA
Neurology 68:615-8. 2007..A photograph of Gehrig from October 1939 showed hand atrophy. Photos from the spring of 1938 showed normal hand muscles and leg function, implying that visible signs first appeared after May 1938...
- Oxidative stress biomarkers in sporadic ALSHiroshi Mitsumoto
Eleanor and Lou Gehrig MDA ALS Research Center, Columbia University Medical Center, New York, NY 10032, USA
Amyotroph Lateral Scler 9:177-83. 2008..ELISA may be reliable and thus useful in epidemiology studies requiring large numbers of samples to determine the significance of increased oxidative stress markers in SALS. Further studies are required...
- Current clinical trials in amyotrophic lateral sclerosisJaydeep M Bhatt
Columbia University, Eleanor and Lou Gehrig MDA ALS Research Center, Department of Neurology, Neurological Institute, New York, NY 10032, USA
Expert Opin Investig Drugs 16:1197-207. 2007..There is an emphasis on the scientific evidence supporting the selection of each drug being tested, as well as on trial design...
- Advances in clinical trials for amyotrophic lateral sclerosisPaul H Gordon
Eleanor and Lou Gehrig MDA ALS Research Center, Neurological Institute, 9th Floor, 710 West 168th Street, New York, NY 10032, USA
Curr Neurol Neurosci Rep 5:48-54. 2005..This article is devoted to the recent evolution of clinical trials in ALS and discusses specific trials conducted during the past 5 years...
- Amyotrophic lateral sclerosisPaul H Gordon
Eleanor and Lou Gehrig Muscular Dystrophy Association Amyotrophic Lateral Sclerosis Research Center, Department of Neurology, College of Physicians and Surgeons, Columbia University, New York, NY 10032, USA
Sci Aging Knowledge Environ 2003:dn2. 2003..Cognition, the extraocular muscles, and the urinary sphincters were spared. Respiratory muscle weakness was a late manifestation...
- Minocycline-induced skin and dental pigmentationsTahseen Mozaffar
Department of Neurology, University of California, Irvine, 101 The City Drive South, Bldg 22 C, Route 13, Orange, CA 92868, USA
Neurology 67:2185. 2006
- ALSFRS-RPaul H Gordon
Eleanor and Lou Gehrig MDA ALS Research Center, Neurological Institute, New York, NY 10032, USA
Amyotroph Lateral Scler Other Motor Neuron Disord 5:90-3. 2004..Unlike the other standard outcome measures currently employed, the ALSFRS-R is also a measure of global function...
- Reaction time and movement time after embryonic cell implantation in Parkinson diseasePaul H Gordon
Department of Neurology and Clinical Motor Physiology Laboratory, Columbia Presbyterian Medical Center, New York, NY 10032, USA
Arch Neurol 61:858-61. 2004..Reaction time (RT) and movement time (MT) analysis, validated quantitative measures of premovement neural processing and motor execution, can be used as objective physiological markers of motor performance in PD...
- Extensor digitorum brevis test and resistance to botulinum toxin type APaul H Gordon
Department of Neurology, ACC 2, University of New Mexico, 915 Camino de Salud NE, Albuquerque, New Mexico 87131, USA
Muscle Nerve 26:828-31. 2002..Four of five resistant patients had values outside the normal range. The EDB test is a simple quantitative method of detecting resistance to BTX-A, with a sensitivity of 80% and specificity of 94%...