scholarly journals Clinical Presentation and Features of Juvenile-Onset Huntington’s Disease: A Systematic Review

2019 ◽  
Vol 8 (2) ◽  
pp. 171-179 ◽  
Author(s):  
Thomas Cronin ◽  
Anne Rosser ◽  
Thomas Massey
Keyword(s):  
Systematic Review ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Clinical Presentation ◽  
Juvenile Onset

scholarly journals The Association between CAG Repeat Length and Age of Onset of Juvenile-Onset Huntington’s Disease

2020 ◽  
Vol 10 (9) ◽  
pp. 575 ◽  
Author(s):  
Jordan L. Schultz ◽  
Amelia D. Moser ◽  
Peg C. Nopoulos
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Age Of Onset ◽  
Repeat Length ◽  
Cag Repeat ◽  
Linear Regression Models ◽  
Juvenile Onset ◽  
Additional Information ◽  
Using Data ◽  
The Relationship

There is a known negative association between cytosine–adenine–guanine (CAG) repeat length and the age of motor onset (AMO) in adult-onset Huntington’s Disease (AOHD). This relationship is less clear in patients with juvenile-onset Huntington’s disease (JOHD), however, given the rarity of this patient population. The aim of this study was to investigate this relationship amongst a relatively large group of patients with JOHD using data from the Kids-JOHD study. Additionally, we analyzed data from the Enroll-HD platform and the Predict-HD study to compare the relationship between CAG repeat length and AMO amongst patients with AOHD to that amongst patients with JOHD using linear regression models. In line with previous reports, the variance in AMO that was predicted by CAG repeat length was 59% (p < 0.0001) in the Predict-HD study and 57% from the Enroll-HD platform (p < 0.0001). However, CAG repeat length predicted 84% of the variance in AMO amongst participants from the Kids-JOHD study (p < 0.0001). These results indicate that there may be a stronger relationship between CAG repeat length and AMO in patients with JOHD as compared to patients with AOHD. These results provide additional information that may help to model disease progression of JOHD, which is beneficial for the planning and implementation of future clinical trials.

scholarly journals The global prevalence of Huntington's disease: a systematic review and discussion

2016 ◽  
Vol 6 (4) ◽  
pp. 331-343 ◽  
Author(s):  
Sheharyar Sajjad Baig ◽  
Mark Strong ◽  
Oliver WJ Quarrell
Keyword(s):  
Systematic Review ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Global Prevalence

scholarly journals Physical Therapy and Exercise Interventions in Huntington’s Disease: A Mixed Methods Systematic Review

2017 ◽  
Vol 6 (3) ◽  
pp. 217-235 ◽  
Author(s):  
Nora E. Fritz ◽  
Ashwini K. Rao ◽  
Deb Kegelmeyer ◽  
Anne Kloos ◽  
Monica Busse ◽  
...  
Keyword(s):  
Systematic Review ◽  
Mixed Methods ◽  
Physical Therapy ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Exercise Interventions

CAG repeat size and clinical presentation in Huntington's disease

Neurology ◽  
1994 ◽  
Vol 44 (6) ◽  
pp. 1137-1137 ◽  
Author(s):  
T. Ashizawa ◽  
L-J. C. Wong ◽  
C. S. Richards ◽  
C. T. Caskey ◽  
J. Jankovic
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Clinical Presentation ◽  
Cag Repeat ◽  
Repeat Size

The Efficacy and Safety of Pridopidine on Treatment of Huntington's Disease Patients: A Systematic Review and Meta‐Analysis

2021 ◽  
Author(s):  
Moamen Mostafa Asla ◽  
Asmaa Ahmed Nawar ◽  
Alaa Abdelsalam ◽  
Esraa Elsayed ◽  
Marwa Abdelazim Rizk ◽  
...  
Keyword(s):  
Systematic Review ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Meta Analysis ◽  
Efficacy And Safety
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