Journal ArticleDOI
Behavior in Huntington's disease: dissociating cognition-based and mood-based changes.
Reads0
Chats0
TLDR
The authors examine the relationship of three dimensions of behavioral change measured by the Problem Behaviors Assessment for Huntington's Disease to cognitive and motor indices of disease severity to suggest certain behavioral alterations are intrinsic to the evolution and progression of HD.Abstract:
The authors examined the relationship of three dimensions of behavioral change (Apathy, Depression, and Irritability) measured by the Problem Behaviors Assessment for Huntington's Disease (PBA-HD) to cognitive and motor indices of disease severity. The Apathy subscale was highly correlated with both cognitive and motor impairment; the Irritability and Depression subscales were not. The findings suggest that certain behavioral alterations are intrinsic to the evolution and progression of HD, whereas others are more variable and are independent of other indices of disease progression.read more
Citations
More filters
Journal ArticleDOI
Huntington's disease.
TL;DR: Effective intervention by clinicians is possible in terms of providing patients and families with accurate information about the disease, counseling them about availability of genetic testing at specialized centers, and in giving them sound advice regarding work, driving, relationships, finances, research participation, and support groups.
Journal ArticleDOI
Apathy and the Functional Anatomy of the Prefrontal Cortex–Basal Ganglia Circuits
Richard Levy,Bruno Dubois +1 more
TL;DR: The clinical signs grouped under the concept of apathy are a common feature of prefrontal and basal ganglia lesions or dysfunctions and can therefore help to improve the understanding of the functional anatomy of the prefrontal-basal ganglia system.
FEATURE ARTICLE Apathy and the Functional Anatomy of the Prefrontal Cortex--Basal Ganglia Circuits
Richard Levy,Bruno Dubois +1 more
TL;DR: The clinical signs of apathy are a common feature of prefrontal and basal ganglia lesions or dysfunctions and can therefore help to improve our understanding of the functional anatomy of the prefrontal--basal ganglia system as discussed by the authors.
Journal ArticleDOI
Huntington's disease: from pathology and genetics to potential therapies.
Sara Imarisio,Jenny Carmichael,Viktor I. Korolchuk,Chien-Wen Chen,Shinji Saiki,Claudia Rose,Gauri Krishna,Janet E. Davies,Evangelia K. Ttofi,Benjamin R. Underwood,David C. Rubinsztein +10 more
TL;DR: Not all of the effects of mutant huntingtin may be cell-autonomous, and it is possible that abnormalities in neighbouring neurons and glia may also have an impact on connected cells.
Book ChapterDOI
The Neuropathology of Huntington’s Disease
TL;DR: This variable pattern of pathology appears to reflect the highly variable symptomatology of cases with Huntington's disease even among cases possessing the same number of CAG repeats.
References
More filters
Journal ArticleDOI
Reliability, validity, and clinical correlates of apathy in Parkinson's disease.
Sergio E. Starkstein,Helen S. Mayberg,Thomas J. Preziosi,Paula L. Andrezejewski,Ramón Leiguarda,Robert G. Robinson +5 more
TL;DR: Results suggest that apathy is a frequent finding in PD, is significantly associated with specific cognitive impairments, and may have a different mechanism than depression.
Journal ArticleDOI
Huntington disease: Clinical care and evaluation
Ira Shoulson,Stanley Fahn +1 more
TL;DR: The Commission for the Control of Huntington Disease and Its Consequences, established by the Ninety-Fourth United States Congress, recently completed an appraisal of clinical care of the patient and family with Huntington disease.
Journal ArticleDOI
Apathy Is Not Depression
Morgan L. Levy,Jeffrey L. Cummings,Lynn A. Fairbanks,Donna Masterman,Bruce L. Miller,Anne H. Craig,Jane S. Paulsen,Irene Litvan +7 more
TL;DR: This article performed a cross-sectional comparison of neuropsychiatric symptoms in 30 Alzheimer's disease, 28 frontotemporal dementia, 40 Parkinson disease, 34 Huntington disease, and 22 progressive supranuclear palsy patients, using a standardized rating scale (the Neuropsychiatric Inventory).
Journal ArticleDOI
Anatomic and behavioral aspects of frontal-subcortical circuits.
TL;DR: Frontal-subcortical circuits provide a comprehensive framework for understanding the anatomy, biochemistry, and pharmacology of behavior and the actions of PCP, LSD, serotonergic antidepressants, anxiolytics, sedative-hypnotics, antipsychotic agents, and ethanol may all be partially or primarily mediated through transmitter systems and receptor effects expressed through frontal- Subcorticals.
Journal ArticleDOI
Psychiatric syndromes in Huntington's disease.
Eric D. Caine,Ira Shoulson +1 more
TL;DR: Consideration of the array of behavioral disturbances encountered in this pathogenetically unified disorder suggests that a dimensional approach to symptom classification might prove more useful heuristically than present typological methods.
Related Papers (5)
Unified huntington’s disease rating scale: Reliability and consistency
Karl Kieburtz,John B. Penney,Peter Corno,Neal G. Ranen,Ira Shoulson,Andrew Feigin,Davi Abwender,J. Timothy Greenarnyre,Donald S. Higgins,Frederick J. Marshall,Joshua L. Goldstein,Kimberly Steinberg,Charles Shih,Irene H. Richard,Charlyne Hickey,Carol Zimmerman,Constance Orme,Kathy Claude,David Oakes,Daniel S. Sax,Anthony Kim,Steven M. Hersch,Randi Jones,Alexander P. Auchus,David B. Olsen,Cheryl Bissey-Black,Allen Rubin,Rose Schwartz,Richard Dubinsky,William Mallonee,Carolyn Gray,Nan Godfrey,Greg Suter,Kathleen M. Shannon,Glenn T. Stebbins,Jean A. Jaglin,Karen Marder,Stuart Taylor,Elan D. Louis,Carol Moskowitz,Deborah Zeck Thorne,Naomi Zubin,Nancy S. Wexler,Michael R. Swenson,Jane S. Paulsen,Neal R. Swerdlow,Roger L. Albin,Christine Wernette,Francis O. Walker,Vicki Hunt +49 more
A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes
Marcy E. MacDonald,Christine Ambrose,Mabel P. Duyao,Richard H. Myers,Carol Lin,Lakshmi Srinidhi,Glenn Barnes,Sherryl A.M. Taylor,Marianne James,Nicolet Groot,Heather MacFarlane,Barbara Jenkins,Mary Anne Anderson,Nancy S. Wexler,James F. Gusella,Gillian P. Bates,Sarah Baxendale,Holger Hummerich,Susan F. Kirby,Mike North,S. Youngman,Richard Mott,Günther Zehetner,Zdenek Sedlacek,Annemarie Poustka,Anna-Maria Frischauf,Hans Lehrach,Alan Buckler,Deanna M. Church,Lynn Doucette-Stamm,Michael Conlon O'Donovan,Laura Riba-Ramirez,Manish A. Shah,Vincent P. Stanton,Scott A. Strobel,Karen M. Draths,Jennifer L. Wales,Peter B. Dervan,David E. Housman,Michael R. Altherr,Rita Shiang,Leslie M. Thompson,Thomas J. Fielder,John J. Wasmuth,Danilo A. Tagle,John Valdes,Lawrence W. Elmer,Marc W. Allard,Lucio H. Castilla,Manju Swaroop,Kris Blanchard,Francis S. Collins,Russell G. Snell,Tracey Holloway,Kathleen Gillespie,Nicole A. Datson,Duncan Shaw,Peter S. Harper +57 more
A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. The Huntington's Disease Collaborative Research Group.
Manish A. Shah,Nicole A. Datson,Lakshmi Srinidhi,Vincent P. Stanton,Marcy E. MacDonald,Marc W. Allard,S. Youngman,Anna-Maria Frischauf,Richard Mott,KM Draths,Günther Zehetner,C. O’Donovan,Thomas J. Fielder,Bruce G. Jenkins,Manju Swaroop,Sherryl A.M. Taylor,Lynn Doucette-Stamm,Heather MacFarlane,Scott A. Strobel,H. E. McFarlane,Alan Buckler,Nicolet Groot,Holger Hummerich,Deanna M. Church,M. A. Anderson,Marianne James,Glenn Barnes,M. Christine,Francis S. Collins,Mabel P. Duyao,Peter B. Dervan,Gillian P. Bates,T Holloway,Peter S. Harper,TW Mcdonald,M North,K Blanchard,John J. Wasmuth,D. Shaw,Hans Lehrach,Danilo A. Tagle,Annemarie Poustka,David E. Housman,T. Huntington,Zdenek Sedlacek,Laura Riba,Susan F. Kirby,Carol Lin,Richard H. Myers,Leslie M. Thompson,Russell G. Snell,Michael Conlon O'Donovan,K Gillespie,Rita Shiang,Nancy S. Wexler,Christine Ambrose,J. F. Gusella,Sarah Baxendale,N. Groat,John Valdes +59 more