Huntington disease

R Ghosh, SJ Tabrizi - Handbook of clinical neurology, 2018 - Elsevier
Huntington disease is a monogenic neurodegenerative disorder that displays an autosomal-
dominant pattern of inheritance. It is characterized by motor, psychiatric, and cognitive …

The cognitive burden in Huntington's disease: pathology, phenotype, and mechanisms of compensation

M Papoutsi, I Labuschagne, SJ Tabrizi… - Movement …, 2014 - Wiley Online Library
Huntington's disease (HD) is an inherited autosomal dominant neurodegenerative disorder.
The most prominent sign of HD is the presence of involuntary motor movements. However …

[HTML][HTML] Microglia and complement mediate early corticostriatal synapse loss and cognitive dysfunction in Huntington's disease

DK Wilton, K Mastro, MD Heller, FW Gergits… - Nature medicine, 2023 - nature.com
Huntington's disease (HD) is a devastating monogenic neurodegenerative disease
characterized by early, selective pathology in the basal ganglia despite the ubiquitous …

Predictors of phenotypic progression and disease onset in premanifest and early-stage Huntington's disease in the TRACK-HD study: analysis of 36-month …

SJ Tabrizi, RI Scahill, G Owen, A Durr… - The Lancet …, 2013 - thelancet.com
Background TRACK-HD is a multinational prospective observational study of Huntington's
disease (HD) that examines clinical and biological findings of disease progression in …

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 …

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 …

Movement disorder society task force viewpoint: Huntington's disease diagnostic categories

CA Ross, R Reilmann, F Cardoso… - Movement …, 2019 - pmc.ncbi.nlm.nih.gov
Huntington's disease (HD) is traditionally considered as a triad of movement, cognitive, and
emotional disorders. 1–4 According to current clinical practice,“manifest HD” is diagnosed …

Gene suppression strategies for dominantly inherited neurodegenerative diseases: lessons from Huntington's disease and spinocerebellar ataxia

MS Keiser, HB Kordasiewicz… - Human molecular …, 2016 - academic.oup.com
RNA-targeting approaches are emerging as viable therapeutics that offer an alternative
method to modulate traditionally 'undrugable'targets. In the case of dominantly inherited …

Network topology and functional connectivity disturbances precede the onset of Huntington's disease

DL Harrington, M Rubinov, S Durgerian, L Mourany… - Brain, 2015 - academic.oup.com
Cognitive, motor and psychiatric changes in prodromal Huntington's disease have nurtured
the emergent need for early interventions. Preventive clinical trials for Huntington's disease …

Huntington disease: a quarter century of progress since the gene discovery

CM Testa, J Jankovic - Journal of the neurological sciences, 2019 - Elsevier
Huntington disease (HD) is an autosomal dominant neurodegenerative disorder
characterized by motor, behavioral, and cognitive manifestations. It is caused by an …