The Relationship Between Age-of-Onset and the Behavioral Phenotypic Manifestations in Huntington's Disease
| Main Author: | |
|---|---|
| Language: | English |
| Published: |
The Ohio State University / OhioLINK
2018
|
| Subjects: | |
| Online Access: | http://rave.ohiolink.edu/etdc/view?acc_num=osu152409456533713 |
| id |
ndltd-OhioLink-oai-etd.ohiolink.edu-osu152409456533713 |
|---|---|
| record_format |
oai_dc |
| spelling |
ndltd-OhioLink-oai-etd.ohiolink.edu-osu1524094565337132021-08-03T07:06:16Z The Relationship Between Age-of-Onset and the Behavioral Phenotypic Manifestations in Huntington's Disease Ranganathan, Megha Genetics Health Care Neurology Health Sciences Huntingtons disease HD behavioral symptoms behavioral manifestations age of onset Objective: The purpose of this study is to characterize behavioral manifestations of the Huntington's disease (HD) phenotype as associated with age-of-onset (AOO) of clinical presentation. Background: Behavioral, cognitive, and motor symptoms are cardinal features of HD. The relationship between behavioral symptoms and AOO of clinical symptoms in HD has not been fully explored.Methods: Participants were subjects with manifest HD registered in the Enroll-HD database (as of 2016). The major initial symptom at disease onset, motor, cognitive, or behavioral, and severity of behavioral symptoms at disease presentation in individuals with early onset HD (AOO <30 yrs), early-adult onset HD (AOO 30-59 yrs), and late-adult onset HD (AOO >59 yrs) was compared. Information on the Clinical Characteristics form and short version of the Problem Behaviors Assessment (PBA-s) was used to assess symptom presence and severity at disease onset. Descriptive statistics, chi-square tests, and multinomial logistic regression models were utilized for analysis. Results: A total of 4,469 individuals were eligible for the study. Of individuals in the early onset cohort, 126 (26%) had behavioral symptoms as the presenting symptom compared to 678 (19%) individuals in the early-adult onset cohort and 56 (11%) in the late-adult onset cohort (p<.0001). A one year increase in AOO was associated with a 5.6% decrease in the odds of behavioral symptoms being the presenting symptom at disease onset (p<.0001). A one year increase in AOO was associated with a 5.5% decrease in the odds of presentation with severe behavioral symptoms of any type, particularly disorientation, delusions, and obsessive-compulsive disorder. There was no statistically significant relationship between AOO and risk of severe depression, suicidal ideation or hallucinations.Conclusions: Individuals with earlier onset HD may be more likely to present with behavioral symptoms at disease onset than later-onset individuals. A better understanding of the relationship between AOO and the behavioral phenotype of HD will be helpful in developing therapies that aim to treat symptom specific disease presentations. The findings from this study may also influence how risk assessments are made by genetic counselors and other clinicians for individuals at risk for HD. The observations in this study offer important insight into future avenues of research. 2018-08-14 English text The Ohio State University / OhioLINK http://rave.ohiolink.edu/etdc/view?acc_num=osu152409456533713 http://rave.ohiolink.edu/etdc/view?acc_num=osu152409456533713 unrestricted This thesis or dissertation is protected by copyright: all rights reserved. It may not be copied or redistributed beyond the terms of applicable copyright laws. |
| collection |
NDLTD |
| language |
English |
| sources |
NDLTD |
| topic |
Genetics Health Care Neurology Health Sciences Huntingtons disease HD behavioral symptoms behavioral manifestations age of onset |
| spellingShingle |
Genetics Health Care Neurology Health Sciences Huntingtons disease HD behavioral symptoms behavioral manifestations age of onset Ranganathan, Megha The Relationship Between Age-of-Onset and the Behavioral Phenotypic Manifestations in Huntington's Disease |
| author |
Ranganathan, Megha |
| author_facet |
Ranganathan, Megha |
| author_sort |
Ranganathan, Megha |
| title |
The Relationship Between Age-of-Onset and the Behavioral Phenotypic Manifestations in Huntington's Disease |
| title_short |
The Relationship Between Age-of-Onset and the Behavioral Phenotypic Manifestations in Huntington's Disease |
| title_full |
The Relationship Between Age-of-Onset and the Behavioral Phenotypic Manifestations in Huntington's Disease |
| title_fullStr |
The Relationship Between Age-of-Onset and the Behavioral Phenotypic Manifestations in Huntington's Disease |
| title_full_unstemmed |
The Relationship Between Age-of-Onset and the Behavioral Phenotypic Manifestations in Huntington's Disease |
| title_sort |
relationship between age-of-onset and the behavioral phenotypic manifestations in huntington's disease |
| publisher |
The Ohio State University / OhioLINK |
| publishDate |
2018 |
| url |
http://rave.ohiolink.edu/etdc/view?acc_num=osu152409456533713 |
| work_keys_str_mv |
AT ranganathanmegha therelationshipbetweenageofonsetandthebehavioralphenotypicmanifestationsinhuntingtonsdisease AT ranganathanmegha relationshipbetweenageofonsetandthebehavioralphenotypicmanifestationsinhuntingtonsdisease |
| _version_ |
1719453725592911872 |