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CAG repeat length and weight loss in pre-manifest Huntington’s disease

C.F. Kutz, L.J. Adams (Colorado Springs, CO, USA)

Meeting: 2016 International Congress

Abstract Number: 1104

Keywords: Chorea (also see specific diagnoses, etc): Clinical features, Huntingtons disease

Session Information

Date: Wednesday, June 22, 2016

Session Title: Huntington's disease

Session Time: 12:00pm-1:30pm

Location: Exhibit Hall located in Hall B, Level 2

Objective: To explore if CAG repeat length is a predictor of weight loss in pre-manifest Huntington’s disease.

Background: Weight loss is a hallmark finding in Huntington’s disease. It has been previously hypothesized that weight loss is a result of decreased caloric intake, increased motor activity, and /or higher metabolic rate. Prior research indicated a positive correlation between CAG repeat length and weight loss in Huntington’s disease, however, previous analyses involved only individuals with clinically manifest disease.

Methods: A retrospective analysis using the HSG COHORT database was conducted to determine if there is a predictive relationship between CAG repeat length and weight loss in pre-manifest Huntington’s disease. The percentage of weight change was calculated in the control and case groups. An odds-ratio was calculated to provide an estimate for the relationship between CAG length and weight loss.

Results: Data analysis from 180 participants of the COHORT Study was conducted. The pre-manifest group included 73 individuals with 36-52 CAG repeats, the control group consisted of 107 controls with 36 CAG repeats. Of the 12 case groups, 7 had a negative percentage of weight change indicative of weight gain between baseline and visit 1, and 5 had a positive percentage of weight change indicating a small degree of weight loss. The control group had an average weight change of 0.72 from baseline to visit 1. The odds ratio of 0.309, suggests the odds of having 5% weight loss is 0.309 times greater for those individuals with 36 CAG repeats. These findings do not suggest a predictive relationship between CAG repeat length and weight loss in pre-manifest Huntington’s Disease.

Conclusions: Unintended weight loss is a hallmark symptom of Huntington’s disease. The cause of weight loss is most likely multi-factorial and occurs despite adequate or even increased caloric intake. Previous research concluded CAG repeat length is inversely related to weight loss in clinically-manifest HD, although in pre-manifest individuals, there does not appear to be the same predictive relationship. Additional research to include both asymptomatic and symptomatic individuals may provide clarity to the relationship between CAG length and rate of weight loss. We can conclude that Huntington’s disease symptomatology, particularly rate of weight loss, is variable and can not always be predicted by CAG repeat length.

To cite this abstract in AMA style:

C.F. Kutz, L.J. Adams. CAG repeat length and weight loss in pre-manifest Huntington’s disease [abstract]. Mov Disord. 2016; 31 (suppl 2). https://www.mdsabstracts.org/abstract/cag-repeat-length-and-weight-loss-in-pre-manifest-huntingtons-disease/. Accessed May 13, 2025.
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