Session Information
Date: Monday, September 23, 2019
Session Title: Huntington’s Disease
Session Time: 1:45pm-3:15pm
Location: Agora 3 West, Level 3
Objective: The aim of this study to emphasize that three juvenile form of HD cases who have insomnia as clinical onset symptoms.
Background: Huntington’s Disease (HD) is an autosomal dominant progressive neurodegenerative disorder. It is caused by an abnormal expansion of a CAG repeat. The juvenile form of HD is characterised by an onset in childhood or adolescence. Juvenile form also involves changes in behaviour, mental function and sleep disorders. Many HD patients have sleep problems such as insomnia and daytime somnolence. There have been no large-scale systematic studies of in sleep in HD.
Method: The family consist of eight members. The parents are healthy mother and father with HD (number of CAG repeat: 50). There are six children. Three children (ages 11-F, 15-M, 19-F) had HD. Their CAG repeat was found respectively 55 (case-1), 69 (case-2), 52 (case-3). Case-1 is premanifest HD but she has had insomnia one year. Case-2, the patient have had mental disorders, seizures and insomnia for several years. Case-3, the patient has had insomnia and motor control problems for two years.
Results: Sleep dysfunction in HD is likely to be under-diagnosed by clinicians and under-reported by patients because it seems that HD patients rarely complain to their doctors about sleep disturbance, and they do not show excessive daytime sleepiness. Our three cases’ onset syptom was insomnia then other syptoms were seen.
Conclusion: In conclusion, juvenile HD patients may have frequent insomnia before the manifest stage of HD.
References: 1- Douglas I, Evans S, Rawlins MD, Smeeth L, Tabrizi SJ, Wexler NS. Juvenile Huntington’s disease:a population-based study using the General Practice ResearchDatabase. BMJ Open. 2013; 3:3(4). pii: e002085. doi: 10.1136/bmjopen-2012-002085. Print 2013. 2- Quarrell O, O’Donovan KL, Bandmann O, et al. The prevalence of juvenile Huntington’s disease: a review of the literature and meta-analysis. PLoS Curr 2012;4. doi:10.1371/418606b742ef3. 3- Morton AJ. Circadian and sleep disorder in Huntington’s disease. Exp Neurol. 2013 May;243:34-44. doi: 10.1016/j.expneurol.2012.10.014. Epub 2012 Oct 22.
To cite this abstract in AMA style:
M. Gultekin. Insomnia as clinical onset symptom in Juvenile Huntington’s Disease: Evaluation of a Family [abstract]. Mov Disord. 2019; 34 (suppl 2). https://www.mdsabstracts.org/abstract/insomnia-as-clinical-onset-symptom-in-juvenile-huntingtons-disease-evaluation-of-a-family/. Accessed June 15, 2025.« Back to 2019 International Congress
MDS Abstracts - https://www.mdsabstracts.org/abstract/insomnia-as-clinical-onset-symptom-in-juvenile-huntingtons-disease-evaluation-of-a-family/