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1. Comparing Four Medicines to Treat Pain from Cryptogenic Sensory Polyneuropathy—The PAIN-CONTRoLS Study

Author

James Wymer, Tuan Vu, Yuebing Li, Pariwat Thaisetthawatkul, M Jacoby, Andrea Swenson, Aiesha Ahmed, Jau Shin Lou, Mamatha Pasnoor, Ted M. Burns, C Parks, Michael K. Hehir, David Walk, Stephen N. Scelsa, Shafeeq Ladha, Ghazala Hayat, Gordon Smith, Jaya Trivedi, Byron J. Gajewski, William Mallonee, Richard J. Barohn, P Shlemon, Omar Jawdat, Robert M. Pascuzzi, Matthew Wicklund, Tiyonnoh M. Cash, Noah Kolb, Sindhu Ramchandren, Jeffrey W. Ralph, L Brown, Paul Twydell, Hani Kushlaf, Gil I. Wolfe, Mazen M. Dimachkie, S Austin, Michael Pulley, Y Hussainn, David Saperstein, Stanley Iyadurai, Dianna Quan, T Liu, Chafic Karam, Amro M. Stino, D Heitzman, Anza B. Memon, Thomas H. Brannagan, A Tobon, Khema Sharma, M Ahmed, Kim S. Kimminau, Vera Bril, John T. Kissel, Christen Kutz, N Verma, M Bazant, Richard A. Lewis, Suur Biliciler, Alexandru Barboi, K Salajegheh, and Laura Herbelin

Subjects
medicine.medical_specialty, business.industry, Internal medicine, Sensory polyneuropathy, Medicine, business
Published
2020

2. Patient Assisted Intervention for Neuropathy: Comparison of Treatment in Real Life Situations (PAIN-CONTRoLS)

Author

Suur Biliciler, Chafic Karam, Alexandru Barboi, Michael K. Hehir, Ghazala Hayat, Paul Twydell, Gil I. Wolfe, Alexandra R. Brown, Pariwat Thaisetthawatkul, Dianna Quan, Moiz Ahmed, Mazen M. Dimachkie, Darryl Heitzman, Alejandro Tobon, Yuebing Li, Andrea Swenson, Jau Shin Lou, William Mallonee, Richard A. Lewis, John T. Kissel, Ted M. Burns, Mark Jacoby, Noah Kolb, Robert M. Pascuzzi, Jaya Trivedi, Tiyonnoh M. Cash, Thomas H. Brannagan, Jeffrey W. Ralph, Vera Bril, Amro M. Stino, Sindhu Ramchandren, Michael Pulley, Christen Kutz, Khema Sharma, Richard J. Barohn, Anza B. Memon, David Saperstein, Matthew Wicklund, Stanley Iyadurai, Navin Verma, Shafeeq Ladha, Gordon Smith, Kim S. Kimminau, Laura Herbelin, Dinesh Pal Mudaranthakam, Byron J. Gajewski, Mark Bazant, Hani Kushlaf, Mamatha Pasnoor, Aiesha Ahmed, Kian Salajegheh, Yessar Hussain, Sara Austin, Omar Jawdat, Tuan Vu, James Wymer, David Walk, and Stephen N. Scelsa

Subjects
medicine.medical_specialty, Randomization, Diabetic neuropathy, business.industry, Pregabalin, Interim analysis, medicine.disease, law.invention, 03 medical and health sciences, chemistry.chemical_compound, 0302 clinical medicine, Randomized controlled trial, chemistry, law, Internal medicine, Neuropathic pain, Medicine, Duloxetine, 030212 general & internal medicine, Neurology (clinical), Nortriptyline, business, 030217 neurology & neurosurgery, medicine.drug
Abstract

Importance Cryptogenic sensory polyneuropathy (CSPN) is a common generalized slowly progressive neuropathy, second in prevalence only to diabetic neuropathy. Most patients with CSPN have significant pain. Many medications have been tried for pain reduction in CSPN, including antiepileptics, antidepressants, and sodium channel blockers. There are no comparative studies that identify the most effective medication for pain reduction in CSPN. Objective To determine which medication (pregabalin, duloxetine, nortriptyline, or mexiletine) is most effective for reducing neuropathic pain and best tolerated in patients with CSPN. Design, Setting, and Participants From December 1, 2014, through October 20, 2017, a bayesian adaptive, open-label randomized clinical comparative effectiveness study of pain in 402 participants with CSPN was conducted at 40 neurology care clinics. The trial included response adaptive randomization. Participants were patients with CSPN who were 30 years or older, with a pain score of 4 or greater on a numerical rating scale (range, 0-10, with higher scores indicating a higher level of pain). Participant allocation to 1 of 4 drug groups used the utility function and treatment’s sample size for response adaptation randomization. At each interim analysis, a decision was made to continue enrolling (up to 400 participants) or stop the whole trial for success (80% power). Patient engagement was maintained throughout the trial, which helped guide the study and identify ways to communicate and disseminate information. Analysis was performed from December 11, 2015, to January 19, 2018. Interventions Participants were randomized to receive nortriptyline (n = 134), duloxetine (n = 126), pregabalin (n = 73), or mexiletine (n = 69). Main Outcomes and Measures The primary outcome was a utility function that was a composite of the efficacy (participant reported pain reduction of ≥50% from baseline to week 12) and quit (participants who discontinued medication) rates. Results Among the 402 participants (213 men [53.0%]; mean [SD] age, 60.1 [13.4] years; 343 White [85.3%]), the utility function of nortriptyline was 0.81 (95% bayesian credible interval [CrI], 0.69-0.93; 34 of 134 [25.4%] efficacious; and 51 of 134 [38.1%] quit), of duloxetine was 0.80 (95% CrI, 0.68-0.92; 29 of 126 [23.0%] efficacious; and 47 of 126 [37.3%] quit), pregabalin was 0.69 (95% CrI, 0.55-0.84; 11 of 73 [15.1%] efficacious; and 31 of 73 [42.5%] quit), and mexiletine was 0.58 (95% CrI, 0.42-0.75; 14 of 69 [20.3%] efficacious; and 40 of 69 [58.0%] quit). The probability each medication yielded the highest utility was 0.52 for nortriptyline, 0.43 for duloxetine, 0.05 for pregabalin, and 0.00 for mexiletine. Conclusions and Relevance This study found that, although there was no clearly superior medication, nortriptyline and duloxetine outperformed pregabalin and mexiletine when pain reduction and undesirable adverse effects are combined to a single end point. Trial Registration ClinicalTrials.gov Identifier:NCT02260388

Published
2021

3. The impact of oculomotor functioning on neuropsychological performance in Huntington disease

Author

William Mallonee, Thomas T. Warner, Vicki L. Wheelock, Emily P. Freney, Stephen M. Rao, Vincent A. Magnotta, Alma Macaraeg, Sigurd D. Süßmuth, Paula Wasserman, Shineeka Smith, Phil Danzer, Anne Elizabeth Rosser, Michael D. Geschwind, Brenton Maxwell, Kelsey Montross, Gabriella Satris, Albie Law, Daniel S. O'Leary, David J. Moser, Thomas Brashers-Krug, Janessa O. Carvalho, Hans J. Johnson, Martha Nance, Anita M.Y. Goh, Mark Guttman, J. Preston, Janet K. Williams, Judith Bek, Susan Perlman, Alanna Sheinberg, Wenjing Lu, Christine Reece, Pietro Mazzoni, Nadine Yoritomo, Elizabeth Aylward, W.R. Wayne Martin, Megan M. Smith, Phyllis Chua, J. Decolongon, Breanna Nickels, Pamela King, Deborah L. Harrington, Roland Zschiegner, Brian Clemente, David G. Gunn, Alex Bura, Vivien Vaughan, Samantha M Loi, Maggie Burrows, Kimberly A. Quaid, Jillian McMillan, Jason Evans, Michael Orth, Mary Wodarski, Courtney Shadrick, Sarah L Mason, H. Jeremy Bockholt, Marguerite Wieler, Elizabeth Howard, Melissa Wesson, Kathy Price, Lisa Kjer, Daniela Rae, Lynn A. Raymond, Roger A. Barker, Edmond Chiu, Holly Westervalt, Joanne Wojcieszek, Stephanie Antonopoulos, Ji In Kim, Jane S. Paulsen, Maryjane Ong, Spencer Lourens, Maria Tedesco, Katrin Barth, Christina Reeves, Jane Griffith, Peter K. Panegyres, Sarah Hunt, Cathy Wood-Siverio, David Craufurd, Holly James Westervelt, Zosia Miedzybrodzka, A. Coleman, Irita Karmalkar, Joseph W. Y. Lee, Carolin Eschenbach, Greg Suter, Christopher A. Ross, Satwinder Sran, Sharon J. Sha, Daniela Schwenk, Joel S. Perlmutter, Ali Samii, James A. Mills, Mark Varvaris, Amanda Miller, Frederick J. Marshall, Amy M. Chesire, Isabella De Soriano, Ying Zhang, Natalie Valle Guzman, Angela Komiti, Oksana Suchowersky, Clement T. Loy, Diane Erickson, Karen Marder, Lyla Mourany, Joseph Winer, Anwar Ahmed, Jatin G. Vaidya, Eun Young Kim, Stacey K. Barton, Cheryl Erwin, Sonja Trautmann, Amanda Martin, Stewart A. Factor, Elizabeth McCusker, Mariella D'Alessandro, Jeffrey D. Long, Randi Jones, Nancy R. Downing, Jared M. Bruce, Charlyne Hickey, Rajeev Kumar, Mannie Fan, and Sarah E Tomaszewski Farias

Subjects
Adult, Male, Elementary cognitive task, medicine.medical_specialty, genetic structures, Disease, Audiology, Article, 050105 experimental psychology, Ocular Motility Disorders, Executive Function, 03 medical and health sciences, 0302 clinical medicine, medicine, Humans, 0501 psychology and cognitive sciences, Psychiatry, Psychomotor learning, Multiple sclerosis, 05 social sciences, Neuropsychology, Cognition, Middle Aged, medicine.disease, Clinical Psychology, Huntington Disease, Neurology, Schizophrenia, Female, Neurology (clinical), Cognition Disorders, Psychology, Psychomotor Performance, 030217 neurology & neurosurgery
Abstract

Huntington disease (HD) is a neurodegenerative condition with prominent motor (including oculomotor), cognitive, and psychiatric effects. While neuropsychological deficits are present in HD, motor impairments may impact performance on neuropsychological measures, especially those requiring a speeded response, as has been demonstrated in multiple sclerosis and schizophrenia. The current study is the first to explore associations between oculomotor functions and neuropsychological performance in HD. Participants with impaired oculomotor functioning performed worse than those with normal oculomotor functioning on cognitive tasks requiring oculomotor involvement, particularly on psychomotor speed tasks, controlling for covariates. Consideration of oculomotor dysfunction on neuropsychological performance is critical, particularly for populations with motor deficits.

Published
2016

4. Safety of Converting From Tetrabenazine to Deutetrabenazine for the Treatment of Chorea

Author

William Mallonee, Joseph Jankovic, Erin Furr-Stimming, David Oakes, Christine Hunter, Rajeev Kumar, Christina L. Vaughan, Clement T. Loy, Andrew P. Duker, Alternatives for Reducing Chorea in Huntington Disease Investigators, Samuel Frank, Victor W. Sung, Charles S. Davis, Elise Kayson, Burton L. Scott, Jody Goldstein, David Stamler, Shirley Eberly, Daniel O. Claassen, Mark Gudesblatt, Amy Colcher, Claudia M. Testa, Lawrence Elmer, and Sandra K. Kostyk

Subjects
0301 basic medicine, medicine.medical_specialty, congenital, hereditary, and neonatal diseases and abnormalities, Tetrabenazine, 03 medical and health sciences, 0302 clinical medicine, Huntington's disease, Internal medicine, Severity of illness, mental disorders, medicine, Adverse effect, Original Investigation, business.industry, Chorea, medicine.disease, nervous system diseases, Regimen, 030104 developmental biology, Tolerability, Deutetrabenazine, Physical therapy, Neurology (clinical), medicine.symptom, business, 030217 neurology & neurosurgery, medicine.drug
Abstract

Importance Tetrabenazine is efficacious for chorea control; however, tolerability concerns exist. Deutetrabenazine, a novel molecule that reduces chorea, was well tolerated in a double-blind, placebo-controlled study. Objectives To evaluate the safety and explore the efficacy of conversion from tetrabenazine to deutetrabenazine in patients with chorea associated with Huntington disease (HD). Design, Setting, and Participants In this ongoing, open-label, single-arm study that started on December 21, 2013, 37 patients at 13 Huntington Study Group sites in the United States and Australia who were taking stable doses of tetrabenazine that provided a therapeutic benefit were switched overnight to deutetrabenazine therapy. After week 1, the deutetrabenazine dose was titrated on a weekly basis for optimal chorea control. Interventions Deutetrabenazine administration at a dosage thought to provide comparable systemic exposure to the active metabolites of the prior, stable tetrabenazine regimen. Main Outcomes and Measures Safety measures included adverse events (AEs), clinical laboratory tests, vital signs, electrocardiograms, and validated scales. Changes in the Unified Huntington’s Disease Rating Scale total maximal chorea score and total motor score were efficacy end points. Results Of the 53 patients with HD screened for the study, 37 ambulatory patients with manifest HD (mean [SD] age, 52.4 [11.5] years; 22 [59%] male and 15 [41%] female; 36 white [97.3%]) were enrolled. Deutetrabenazine was generally well tolerated, with low rates of neuropsychiatric AEs. Safety scales did not reveal subclinical toxicity with deutetrabenazine treatment. Rates of dose reduction or suspension attributable to AEs were also low. Chorea control, as measured by the total maximal chorea score, was maintained at week 1 and significantly improved at week 8 (mean [SD] change from baseline, 2.1 [3.2]; P Conclusions and Relevance In patients with chorea, overnight conversion to deutetrabenazine therapy provided a favorable safety profile and effectively maintained chorea control.

Published
2017

5. Regionally selective atrophy of subcortical structures in prodromal HD as revealed by statistical shape analysis

Author

Mary Wodarski, Melissa Wesson, David Craufurd, James A. Mills, Terry Tempkin, Gabriel Castillo, Jan Zimbelman, Karen Anderson, Christopher A. Ross, Diane Erickson, Amy M. Chesire, Mark Groves, Paula Wasserman, Alma Macaraeg, Deborah K. Moore, Karl G. Helmer, Elizabeth McCusker, Satwinder Sran, Mycah J Kimble, Thomas H. Wassink, Erik B. Beall, Sarah A. J. Reading, Steve Blanchard, Arthur W. Toga, Kevin Duff, Hugh Rickards, Brittany Lichty, Jess G. Fiedorowicz, Zosia Miedzybrodzka, Peg Nopoulos, Wenjing Lu, Irita Karmalkar, Lisa Hughes, William Mallonee, Thomas T. Warner, Ryan Wyse, Gail A. Kang, Michelle Harreld, Joseph W. Y. Lee, Marleen Van Walsem, Tom Wassink, Laurent Younes, Karla J. Anderson, Hans J. Johnson, Brian Clemente, Michael Diaz, Derek Weston, Elizabeth Howard, D. Psych, David J. Moser, Vicki L. Wheelock, Deborah Harrington, Ali Samii, Erin R. Foster, Steve Andrew, Elizabeth Aylward, Leigh J. Beglinger, Sigurd Sübmuth, Rebecca Ready, Karen Marder, Anthony L. Vaccarino, Frederick J. Marshall, Sarah L Mason, Samantha M Loi, Stacie M. Vik, Michael Phillips, Sean Thompson, Jean Paul G. Vonsattel, J. Preston, Katrin Barth, Angela Komiti, Stacey K. Barton, Lyla Mourany, Allen W. Song, Randi Jones, Jennifer D. Davis, Edmond Chiu, Anwar Ahmed, Mark J. Lowe, Chris Werling-Witkoske, Clement T. Loy, Robert A. Robinson, Megan M. Smith, Cheryl Erwin, Greg Suter, Mirza Faisal Beg, Sarah Hunt, Phil Danzer, Eun Young Kim, Jeremy Bockholt, Carol Moskowitz, Leann Davis, Vincent A. Magnotta, Roland Zschiegner, Gabriela Satris, Sonja Trautmann, Karen L. Siedlecki, J. David, Timothy Brown, Spencer Lourens, Juliet Schulz, Kelvin Lim, Judith Bek, Katherine A. Koenig, Stephen Cross, Christine Reece, Janet Willia, Oksana Suchowersky, Joanne Wojcieszek, Martha Nance, Mark Varvaris, Kelsey Montross, Amy P. Schmidt, Stephanie Antonopoulos, Pietro Mazzoni, Jessica Morison, Owen Wade, Kimberly A. Quaid, Nancy R. Downing, Roger L. Albin, Geoff Tremont, Cathy Wood-Siverio, Greg Ennis, Lynn A. Raymond, Kathy Jones, Rachel Bernier, Ian G. Dobbins, Eric A. Epping, Stewart A. Factor, Tamara Hershey, Janet K. Williams, John Ashburner, Jacquie Marietta, Alex Bura, Kate Papp, Kirsty Matheson, S.G. Potkin, Ying Zhang, Lee Anna Clark, Joel S. Perlmutter, Lisa Kjer, Sasumu Mori, David G. Gunn, Ji In Kim, Anne Elizabeth Rosser, Stephen M. Rao, Wayne R. Matson, Carissa Gehl, Michael D. Geschwind, Dawei Liu, Jane S. Paulsen, Kelly C. Rowe, Jim Smith, Pamela King, Marguerite Wieler, Noelle E. Carlozzi, Kathy Price, Georgiou-Karistianis Nellie Georgiou-Karistianis, Michael I. Miller, Daniela Rae, Susan Perlman, Anita M.Y. Goh, Mark Guttman, Alanna Sheinberg, Phyllis Chua, Mary Gover, Michael Miller, Mariella D'Alessandro, Ken Evans, Jane Griffith, Peter K. Panegyres, Eric Van Duijn, W.R. Wayne Martin, J. Decolongon, Roger A. Barker, Asa Peterson, O. Yastrubetskaya, Maggie Burrows, Marcy E. MacDonald, Michael Orth, Richard M. Myers, Greg Elias, Eric Axelson, J. F. Gusella, Jeffrey D. Long, Craig Stout, Russell L. Margolis, Holly James Westervelt, Charlyne Hickey, Rajeev Kumar, Claudia M. Testa, Sarah E Tomaszewski Farias, Jason Reed, J. Tilak Ratnanather, Greg Harris, Jessica A. Turner, and Patricia Ryan

Subjects
Pathology, medicine.medical_specialty, Large deformation diffeomorphic metric mapping, Radiological and Ultrasound Technology, Putamen, Statistical shape analysis, medicine.disease, Functional imaging, Atrophy, Globus pallidus, nervous system, Neurology, Huntington's disease, Basal ganglia, medicine, Radiology, Nuclear Medicine and imaging, Neurology (clinical), Anatomy, Psychology, Neuroscience
Abstract

Huntington disease (HD) is a neurodegenerative disorder that involves preferential atrophy in the striatal complex and related subcortical nuclei. In this article, which is based on a dataset extracted from the PREDICT-HD study, we use statistical shape analysis with deformation markers obtained through "Large Deformation Diffeomorphic Metric Mapping" of cortical surfaces to highlight specific atrophy patterns in the caudate, putamen, and globus pallidus, at different prodromal stages of the disease. On the basis of the relation to cortico-basal ganglia circuitry, we propose that statistical shape analysis, along with other structural and functional imaging studies, may help expand our understanding of the brain circuitry affected and other aspects of the neurobiology of HD, and also guide the most effective strategies for intervention.

Published
2012

6. Clinical-Genetic Associations in the Prospective Huntington at Risk Observational Study (PHAROS): Implications for Clinical Trials

Author

Alan Percy, Cathy Wood-Siverio, Oksana Suchowersky, Claudia Testa, Yvette Bordelon, Kevin M. Biglan, Francis O. Walker, David P. Richman, Rustom Sethna, Eric Siemers, Shirley Eberly, Marie Saint-Hilaire, Alicia Brocht, Madaline B. Harrison, Richard H. Myers, Carlos Singer, Karen Blindauer, Rajeev Ananda Kumar, Jody Goldstein, Diana Rosas, Anne Young, Charles H. Adler, Kimberly Quaid, James F. Gusella, Carolyn Gray, Penelope Hogarth, James B. Caress, J. B. Penney, Kelvin L. Chou, Teresa Tempkin, Christopher Ross, Jody Corey-Bloom, Brad Racette, Victoria Hunt, William Weiner, Thomas D. Bird, J. Decolongon, Greg Suter, Eric Molho, Megan Romer, Blair Leavitt, Mary Lou Klimek, Hillary Lipe, Peter Como, Dawn Radtke, Constance Nickerson, Roger L. Albin, Karen Marder, Marcy E. MacDonald, Sandra Kostyk, Christine Weaver, David Oakes, William Mallonee, Amy Duffy, Tatiana Foroud, Marguerite Wieler, Clifford W. Shults, Sarah Furtado, Julie C. Stout, William C. Johnson, Timothy Greenamyre, Ira Shoulson, Carol Moskowitz, J.S. Paulsen, Karl Kieburtz, Lauren Seeberger, Douglas Hobson, Scott R. Bundlie, Steven M. Hersch, Leon S. Dure, Arthur Watts, Vicki L. Wheelock, Donald S. Higgins, Lorne A. Clarke, Stanley Fahn, Nestor Galvez-Jimenez, Andrew Feigin, Lynn A. Raymond, Joseph Jankovic, Sylvain Chouinard, Caroline M. Tanner, Melissa Wesson, Barbara Shannon, Marie Cox, Cynthia L. Comella, John N. Caviness, Guerry M. Peavy, Adam Rosenblatt, Robert A. Hauser, Carol Zimmerman, Christine Hunter, Christine O'Neill, Juan Sanchez-Ramos, Corrine Baic, Peter Novak, Sharon Evans, Hongwei Zhao, Stewart A. Factor, W.R. Wayne Martin, Candace Cotto, Richard Dubinsky, David D. Song, M. Aileen Shinaman, Susan B. Perlman, Joel S. Perlmutter, Ali Samii, Elise Kayson, Mark Guttman, Frederick J. Marshall, Kathleen L. Shannon, and M. Nance

Subjects
0301 basic medicine, Adult, Male, medicine.medical_specialty, Neurogenetics, Cohort Studies, 03 medical and health sciences, 0302 clinical medicine, Huntington's disease, Internal medicine, medicine, Humans, Single-Blind Method, Functional ability, Longitudinal Studies, Prospective Studies, Psychiatry, Prospective cohort study, Genetic Association Studies, Randomized Controlled Trials as Topic, Repeated measures design, Middle Aged, medicine.disease, Clinical trial, 030104 developmental biology, Huntington Disease, Mutation, Observational study, Female, Neurology (clinical), Psychology, Trinucleotide Repeat Expansion, 030217 neurology & neurosurgery, Cohort study
Abstract

Identifying measures that are associated with the cytosine-adenine-guanine (CAG) expansion in individuals before diagnosis of Huntington disease (HD) has implications for designing clinical trials.To identify the earliest features associated with the motor diagnosis of HD in the Prospective Huntington at Risk Observational Study (PHAROS).A prospective, multicenter, longitudinal cohort study was conducted at 43 US and Canadian Huntington Study Group research sites from July 9, 1999, through December 17, 2009. Participants included 983 unaffected adults at risk for HD who had chosen to remain unaware of their mutation status. Baseline comparability between CAG expansion (≥37 repeats) and nonexpansion (37 repeats) groups was assessed. All participants and investigators were blinded to individual CAG analysis. A repeated-measures analysis adjusting for age and sex was used to assess the divergence of the linear trend between the expanded and nonexpanded groups. Data were analyzed from April 27, 2010, to September 3, 2013.Huntington disease mutation status in individuals with CAG expansion vs without CAG expansion.Unified Huntington's Disease Rating Scale motor (score range, 0-124; higher scores indicate greater impairment), cognitive (symbol digits modality is the total number of correct responses in 90 seconds; lower scores indicate greater impairment), behavioral (score range, 0-176; higher scores indicate greater behavioral symptoms), and functional (Total Functional Capacity score range, 0-13; lower scores indicate reduced functional ability) domains were assessed at baseline and every 9 months up to a maximum of 10 years.Among the 983 research participants at risk for HD in the longitudinal cohort, 345 (35.1%) carried the CAG expansion and 638 (64.9%) did not. The mean (SD) duration of follow-up was 5.8 (3.0) years. At baseline, participants with expansions had more impaired motor (3.0 [4.2] vs 1.9 [2.8]; P .001), cognitive (P .05 for all measures except Verbal Fluency, P = .52), and behavioral domain scores (9.4 [11.4] vs 6.5 [8.5]; P .001) but not significantly different measures of functional capacity (12.9 [0.3] vs 13.0 [0.2]; P = .23). With findings reported as mean slope (95% CI), in the longitudinal analyses, participants with CAG expansions showed significant worsening in motor (0.84 [0.73 to 0.95] vs 0.03 [-0.05 to 0.11]), cognitive (-0.54 [-0.67 to -0.40] vs 0.22 [0.12 to 0.32]), and functional (-0.08 [-0.09 to -0.06] vs -0.01 [-0.02 to 0]) measures compared with those without expansion (P .001 for all); behavioral domain scores did not diverge significantly between groups.Using these prospectively accrued clinical data, relatively large treatment effects would be required to mount a randomized, placebo-controlled clinical trial involving premanifest HD individuals who carry the CAG expansion.

Published
2015

7. Unified Huntington's disease rating scale: Reliability and consistency

Author

William Mallonee, Charlyne Hickey, Roger L. Albin, Constance Orme, Irene H. Richard, Nancy S. Wexler, Neal G. Ranen, Jean A. Jaglin, Deborah Zeck Thorne, Cheryl Bissey-Black, Greg Suter, Carolyn Gray, Charles Shih, Naomi Zubin, Elan D. Louis, Richard Dubinsky, Alexander P. Auchus, Frederick J. Marshall, Jane S. Paulsen, Glenn T. Stebbins, David Oakes, Nan Godfrey, Kimberly Steinberg, Kathleen M. Shannon, Ira Shoulson, Allen Rubin, Francis O. Walker, Joshua L. Goldstein, Davi Abwender, Rose Schwartz, Donald S. Higgins, Peter Corno, Kathy Claude, Michael R. Swenson, Karen Marder, David B. Olsen, Andrew Feigin, Christine Wernette, John B. Penney, Neal R. Swerdlow, Vicki Hunt, Carol Zimmerman, Steven M. Hersch, Anthony Kim, Karl Kieburtz, Carol Moskowitz, J. Timothy Greenarnyre, Randi Jones, Stuart Taylor, and Daniel S. Sax

Subjects
Neurology, Huntington's disease, business.industry, Consistency (statistics), Rating scale, Medicine, Neurology (clinical), business, medicine.disease, Reliability (statistics), Cognitive psychology
Published
1996

8. Chiari-1 malformation, migraine, and sudden death

Author

Dewey K. Ziegler and William Mallonee

Subjects
Male, congenital, hereditary, and neonatal diseases and abnormalities, Pediatrics, medicine.medical_specialty, Adolescent, Migraine Disorders, Autopsy, Sudden death, Syncope, Death, Sudden, medicine.artery, medicine, Basilar artery, Humans, Chiari malformation, biology, Vascular disease, business.industry, Syncope (genus), medicine.disease, biology.organism_classification, Syringomyelia, nervous system diseases, Arnold-Chiari Malformation, Neurology, Migraine, Anesthesia, embryonic structures, Neurology (clinical), business
Abstract

Sudden death occurring in a young man with Chiari-1 malformation after repeated attacks of headache and syncope that had been diagnosed as basilar migraine is reported. Autopsy revealed only Chiari malformation and syringomyelia. The ominous potential of syncope in this syndrome is emphasized.

Published
2004

9. Interrater agreement in the assessment of motor manifestations of Huntington's disease

Author

Susan L. Hickenbottom, Kathleen M. Shannon, Frederick Wooten, Danna Jennings, Lynn A. Raymond, Brad A. Racette, Elise Kayson, Joel S. Perlmutter, Mark Guttman, Robert L. Rodnitzky, James Duffy, Steven M. Hersch, Anne B. Young, Joseph Jankovic, Karen Blindauer, Tetsuo Ashizawa, Marc J. Mentis, Nancy S. Wexler, Donald S. Higgins, Neil Schimke, Robert Snodgrass, Alan K. Percy, Paul Shelton, Aileen Shinaman, Stewart A. Factor, Cynthia L. Comella, Ted E. Roberts, Christopher A. Ross, Joanne Wojcieszek, Amerigo Negrette, Thomas D. Bird, Elan D. Louis, Diana Rosas, Martha Nance, Penelope Hogarth, John N. Caviness, Charles H. Adler, Andrew Feigin, George W. Paulson, Francis O. Walker, Hubert H. Fernandez, B. Hersh, Allen Rubin, James B. Caress, Susan Perlman, John B. Penney, Carl Leventhal, Robert A. Hauser, Roger L. Albin, Leslie M. Thompson, William Weiner, Gerald Podskalny, W.R. Wayne Martin, Christopher F. O'Brien, Hongwei Zhao, Sylvain Chouinard, Jody Corey-Bloom, Ira Shoulson, Eric Siemers, Wallace Deckel, Jang Ho Cha, Timothy Greenamyre, Eric Molho, Cliff Shults, William C. Koller, Daniel S. Sax, Karen Marder, Scott R. Bundlie, David Palmer, Leon S. Dure, George R. Jackson, Peter Como, Oksana Suchowersky, Karl Kieburtz, Joseph Friedman, Margot Mejia de Young, Anne Louis Lafontaine, Kenneth Marek, Jane S. Paulsen, Timothy J Counihan, David Oakes, Richard Dubinsky, Ernesto Bonilla, Guy A. Rouleau, Kenneth H. Fischbeck, Kimberly Quiad, David P. Richman, Juan Rachez-Ramos, Constance Orme, Douglas E. Hobson, Leslie A. Shinobu, William Mallonee, William C. Johnson, Vicki L. Wheelock, and Maria Ramos

Subjects
medicine.medical_specialty, Population, Disease, Severity of Illness Index, Central nervous system disease, Degenerative disease, Huntington's disease, Basal Ganglia Diseases, Rating scale, medicine, Humans, Prospective Studies, education, Observer Variation, education.field_of_study, business.industry, Videotape Recording, medicine.disease, Clinical trial, Inter-rater reliability, Huntington Disease, Neurology, Physical therapy, Disease Progression, Neurology (clinical), Psychomotor Disorders, business
Abstract

With prospects improving for experimental therapeutics aimed at postponing the onset of illness in preclinical carriers of the Huntington's disease (HD) gene, we assessed agreement among experienced clinicians with respect to the motor manifestations of HD, a relevant outcome measure for preventive trials in this population. Seventy-five clinicians experienced in the evaluation of patients with early HD and six non-clinicians were shown a videotape compiled from the film archives of the United States–Venezuela Collaborative HD Research Project. Observers were asked to rate a 2–3-minute segment of the motor examination for each of 17 at-risk subjects. The rating scale ranged from 0 (normal) to 4 (unequivocal extrapyramidal movement disorder characteristic of HD). As measured by a weighted κ statistic, there was substantial agreement among the 75 clinicians in the judgment of unequivocal motor abnormalities comparing scale ratings of 4 with ratings that were not 4 (weighted κ = 0.67; standard error (SE) = 0.09). Agreement among the non-clinicians was only fair (weighted κ = 0.28; SE = 0.10). Even under the artificial conditions of a videotape study, experienced clinicians show substantial agreement about the signs that constitute the motor manifestations of illness in subjects at risk for HD. We expect these findings to translate to a similar level of interobserver agreement in the clinical trial setting involving experienced investigators examining live patients. © 2005 Movement Disorder Society

Published
2004

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