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 …

Huntington disease: natural history, biomarkers and prospects for therapeutics

CA Ross, EH Aylward, EJ Wild, DR Langbehn… - Nature Reviews …, 2014 - nature.com
Huntington disease (HD) can be seen as a model neurodegenerative disorder, in that it is
caused by a single genetic mutation and is amenable to predictive genetic testing, with …

Cognitive impairment in Huntington disease: diagnosis and treatment

JS Paulsen - Current neurology and neuroscience reports, 2011 - Springer
Cognition has been well characterized in the various stages of Huntington disease (HD) as
well as in the prodrome before the motor diagnosis is given. Although the clinical diagnosis …

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 …

Indexing disease progression at study entry with individuals at‐risk for Huntington disease

Y Zhang, JD Long, JA Mills, JH Warner… - American Journal of …, 2011 - Wiley Online Library
The identification of clinical and biological markers of disease in persons at risk for
Huntington disease (HD) has increased in efforts to better quantify and characterize the …

Psychological and cognitive markers of behavioral variant frontotemporal dementia–A clinical neuropsychologist's view on diagnostic criteria and beyond

A Johnen, M Bertoux - Frontiers in neurology, 2019 - frontiersin.org
Behavioral variant frontotemporal dementia (bvFTD) is the second leading cognitive
disorder caused by neurodegeneration in patients under 65 years of age. Characterized by …

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 …

An exploratory double‐blind, randomized clinical trial with selisistat, a SirT1 inhibitor, in patients with H untington's disease

SD Süssmuth, S Haider… - British journal of …, 2015 - Wiley Online Library
Aims Selisistat, a selective SirT1 inhibitor is being developed as a potentially disease‐
modifying therapeutic for Huntington's disease (HD). This was the first study of selisistat in …

Longitudinal psychiatric symptoms in prodromal Huntington's disease: a decade of data

EA Epping, JI Kim, D Craufurd… - American Journal of …, 2016 - Am Psychiatric Assoc
Objective: Psychiatric symptoms are a significant aspect of Huntington's disease, an
inherited neurodegenerative illness. The presentation of these symptoms is highly variable …

Social cognition in Huntington's disease: A meta-analysis

E Bora, D Velakoulis, M Walterfang - Behavioural Brain Research …, 2016 - Elsevier
Neurocognitive impairment in Huntington's disease (HD) frequently includes deficits in
emotion recognition, and recent studies have also provided evidence for deficits in theory of …