Managing juvenile Huntington's disease

OWJ Quarrell, MA Nance, P Nopoulos… - Neurodegenerative …, 2013 - Taylor & Francis
Huntington's disease (HD) is a well-recognized progressive neurodegenerative disorder that
follows an autosomal dominant pattern of inheritance. Onset is insidious and can occur at …

Huntington's disease: neuropsychiatric manifestations of Huntington's disease

AMY Goh, P Wibawa, SM Loi… - Australasian …, 2018 - journals.sagepub.com
Objectives: Huntington's disease (HD) is a profoundly incapacitating, and ultimately fatal,
neurodegenerative disease. HD is presently incurable, so the current goal is to allow …

Psychiatric and cognitive difficulties as indicators of juvenile huntington disease onset in 29 patients

P Ribaï, K Nguyen, V Hahn-Barma… - Archives of …, 2007 - jamanetwork.com
Background Juvenile Huntington disease (JHD) is a rare clinical entity characterized by an
age at onset younger than 20 years. Patients usually have an expansion of more than 60 …

Postnatal and adult consequences of loss of huntingtin during development: Implications for Huntington's disease

EE Arteaga-Bracho, M Gulinello, ML Winchester… - Neurobiology of …, 2016 - Elsevier
The mutation in huntingtin (mHtt) leads to a spectrum of impairments in the developing
forebrain of Huntington's disease (HD) mouse models. Whether these developmental …

[HTML][HTML] A two years longitudinal study of a transgenic Huntington disease monkey

AWS Chan, Y Xu, J Jiang, T Rahim, D Zhao… - BMC neuroscience, 2014 - Springer
Background A two-year longitudinal study composed of morphometric MRI measures and
cognitive behavioral evaluation was performed on a transgenic Huntington's disease (HD) …

Association of brain atrophy with functional outcome and recovery trajectories after thrombectomy: post hoc analysis of the ESCAPE-NA1 trial

F Benali, J Fladt, T Jaroenngarmsamer, F Bala… - Neurology, 2023 - AAN Enterprises
Background and Objectives Brain frailty may impair the ability of acute stroke patients to
cope with the injury, irrespective of their chronologic age, resulting in impaired recovery. We …

The role of the cerebellum in Huntington's disease: a systematic review

GL Franklin, CHF Camargo, AT Meira, NSC Lima… - The Cerebellum, 2021 - Springer
Huntington's disease (HD) is a rare neurological disorder characterized by progressive
motor, cognitive, and psychiatric disturbances. Although striatum degeneration might justify …

Biotin-responsive basal ganglia disease: neuroimaging features before and after treatment

H Kassem, A Wafaie, S Alsuhibani… - American Journal of …, 2014 - Am Soc Neuroradiology
BACKGROUND AND PURPOSE: Biotin-responsive basal ganglia disease is an autosomal
recessive neurometabolic disorder presenting with subacute encephalopathy that can cause …

Mediation of Age and Thrombectomy Outcome by Neuroimaging Markers of Frailty in Patients With Stroke

F Benali, N Singh, J Fladt… - JAMA Network …, 2024 - jamanetwork.com
Importance Age is a leading predictor of poor outcomes after brain injuries like stroke. The
extent to which age is associated with preexisting burdens of brain changes, visible on …

Global cerebral atrophy in early stages of Huntington's disease: quantitative MRI study

J Kassubek, GB Landwehrmeyer, D Ecker… - …, 2004 - journals.lww.com
Global brain atrophy was determined in 70 patients suffering from Huntington's disease (HD)
and 70 healthy controls, using brain parenchymal fractions calculated from 3D MRI data in a …