Clinical Presentation and Features of Juvenile-Onset Huntington's Disease: A Systematic Review [0.03%]
青少年亨廷顿舞蹈病的临床表现和特征：系统评价
Thomas Cronin,Anne Rosser,Thomas Massey
Thomas Cronin
Background: Juvenile-onset Huntington's disease (JHD) is defined by onset at the age of 20 or younger and represents approximately 5% of all HD cases. Patients with JHD present with a broad range of symptoms and signs tha...
Late Onset Huntington Disease: Phenotypic and Genotypic Characteristics of 10 Cases in Argentina [0.03%]
阿根廷迟发性亨廷顿舞蹈病患者的临床及基因特征分析
Natalia González Rojas,Javier Enrique Ziliani,Martin Emilano Cesarini et al.
Natalia González Rojas et al.
Background: Huntington's disease (HD) is a neurodegenerative disorder that includes motor, psychiatric and cognitive manifestations with typical onset of symptoms is in the forties. A percentage of patients (4.4% - 11.5%)...
Tamara Matheis,Craig Evinger,Robin Schubert et al.
Tamara Matheis et al.
Background: The ability of healthy individuals to detect biological motion by using a small number of moving points is well established in animals and humans. Perception of human movements may depend on internal models th...
Striatal Cholesterol Precursors Are Altered with Age in Female Huntington's Disease Model Mice [0.03%]
亨廷顿舞蹈病模型小鼠纹状体胆固醇前体随年龄变化而改变（仅限女性）
Anna C Pfalzer,Phillip A Wages,Ned A Porter et al.
Anna C Pfalzer et al.
Background: Cholesterol is necessary for proper neurodevelopment and neuronal health. The brain relies on neural and astrocytic de novo cholesterol synthesis. Huntington's disease presents with altered levels of cholester...
Amit Dhalla,Susil Pallikadavath,Claire V Hutchinson
Amit Dhalla
It is well-documented that patients with Huntington's disease (HD) exhibit specific deficits in visual cognition. A less well-documented literature also exists that suggests people with HD experience a number of disease-related changes to m...
Concurrent Cross-Sectional and Longitudinal Analyses of Multivariate White Matter Profiles and Clinical Functioning in Pre-Diagnosis Huntington Disease [0.03%]
震颤病前诊断的多变量白质谱系和临床功能的并行横断面及纵向分析
Jennifer A Ciarochi,Hans J Johnson,Vince D Calhoun et al.
Jennifer A Ciarochi et al.
Background: Gray matter (GM) atrophy in the striatum and across the brain is a consistently reported feature of the Huntington Disease (HD) prodrome. More recently, widespread prodromal white matter (WM) degradation has a...
Nicolas Arbez,Elaine Roby,Sergey Akimov et al.
Nicolas Arbez et al.
Background: The potential benefit of cysteamine for Huntington's disease has been demonstrated in HD animal models. Cysteamine and its derivate cystamine were shown to reduce neuropathology and prolong lifespan. Human stu...
Assessment of the Performance of a Modified Motor Scale as Applied to Juvenile Onset Huntington's Disease [0.03%]
改良版评分系统用于青年人亨廷顿舞蹈病评定的可行性研究
Mike C Horton,Peggy Nopoulos,Martha Nance et al.
Mike C Horton et al.
Background: Huntington's disease can present at almost any age but traditionally, those with an onset ≤20 years are described as having juvenile onset Huntington's disease (JOHD). They are more likely to have bradykinesi...
Huntingtin Aggregates and Mitochondrial Pathology in Skeletal Muscle but not Heart of Late-Stage R6/2 Mice [0.03%]
R6/2晚期阶段小鼠骨骼肌而非心脏的亨廷顿聚集体和线粒体病理变化
Kerstin Kojer,Tanja Hering,Chantal Bazenet et al.
Kerstin Kojer et al.
Background: Cell or tissue specific background may influence the consequences of expressing the Huntington's disease (HD) mutation. Aggregate formation is known to occur in skeletal muscle, but not heart of the R6/2 fragm...
Filipe B Rodrigues,Lori Quinn,Edward J Wild
Filipe B Rodrigues
In this edition of the Huntington's Disease Clinical Trials Corner we expand on the GENERATION-HD1 and PACE-HD trials, and we list all currently registered and ongoing clinical trials in Huntington's disease. ...