A biological classification of Huntington's disease: the Integrated Staging System

SJ Tabrizi, S Schobel, EC Gantman… - The Lancet …, 2022 - thelancet.com
The current research paradigm for Huntington's disease is based on participants with overt
clinical phenotypes and does not address its pathophysiology nor the biomarker changes …

World Health Organization disability assessment schedule 2.0: An international systematic review

S Federici, M Bracalenti, F Meloni… - Disability and …, 2017 - Taylor & Francis
Purpose: This systematic review examines research and practical applications of the World
Health Organization Disability Assessment Schedule (WHODAS 2.0) as a basis for …

Huntington disease

GP Bates, R Dorsey, JF Gusella, MR Hayden… - Nature reviews Disease …, 2015 - nature.com
Huntington disease is devastating to patients and their families—with autosomal dominant
inheritance, onset typically in the prime of adult life, progressive course, and a combination …

Synaptic dysfunction in neurodegenerative and neurodevelopmental diseases: an overview of induced pluripotent stem-cell-based disease models

E Taoufik, G Kouroupi, O Zygogianni… - Open …, 2018 - royalsocietypublishing.org
Synaptic dysfunction in CNS disorders is the outcome of perturbations in physiological
synapse structure and function, and can be either the cause or the consequence in specific …

Self-organizing neuruloids model developmental aspects of Huntington's disease in the ectodermal compartment

T Haremaki, JJ Metzger, T Rito, MZ Ozair, F Etoc… - Nature …, 2019 - nature.com
Harnessing the potential of human embryonic stem cells to mimic normal and aberrant
development with standardized models is a pressing challenge. Here we use micropattern …

Prediction of manifest Huntington's disease with clinical and imaging measures: a prospective observational study

JS Paulsen, JD Long, CA Ross, DL Harrington… - The Lancet …, 2014 - thelancet.com
Background Although the association between cytosine-adenine-guanine (CAG) repeat
length and age at onset of Huntington's disease is well known, improved prediction of onset …

Diagnostic criteria for Huntington's disease based on natural history

R Reilmann, BR Leavitt, CA Ross - Movement Disorders, 2014 - Wiley Online Library
Huntington's disease (HD) is currently diagnosed based on the presence of motor signs
indicating 99%“diagnostic confidence” for HD. Recent advances in the understanding of HD …

Huntington disease: a single-gene degenerative disorder of the striatum

PC Nopoulos - Dialogues in clinical neuroscience, 2016 - Taylor & Francis
Huntington disease (HD) is an autosomal dominant, neurodegenerative disorder with a
primary etiology of striatal pathology. The Huntingtin gene (HTT) has a unique feature of a …

Timing and impact of psychiatric, cognitive, and motor abnormalities in Huntington disease

B McAllister, JF Gusella, GB Landwehrmeyer, JM Lee… - Neurology, 2021 - AAN Enterprises
Objective To assess the prevalence, timing, and functional impact of psychiatric, cognitive,
and motor abnormalities in Huntington disease (HD) gene carriers, we analyzed …

Statistical approaches to longitudinal data analysis in neurodegenerative diseases: Huntington's disease as a model

TP Garcia, K Marder - Current neurology and neuroscience reports, 2017 - Springer
Understanding the overall progression of neurodegenerative diseases is critical to the timing
of therapeutic interventions and design of effective clinical trials. Disease progression can …