scholarly journals Abnormal Weight and Body Mass Index in Children with Juvenile Huntington’s Disease

2015 ◽  
Vol 4 (3) ◽  
pp. 231-238 ◽  
Author(s):  
Alexander Tereshchenko ◽  
Michael McHugh ◽  
Jessica K. Lee ◽  
Pedro Gonzalez-Alegre ◽  
Kaitlin Crane ◽  
...  
Keyword(s):  
Body Mass Index ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Body Mass ◽  
Juvenile Huntington’S Disease

scholarly journals Factors Associated with Low Body Mass Index in Huntington's Disease: A Spanish Multicenter Study of the European Huntington's Disease Registry

2016 ◽  
Vol 3 (5) ◽  
pp. 452-459
Author(s):  
Esther Cubo ◽  
Jessica Rivadeneyra ◽  
Natividad Mariscal ◽  
Asunción Martinez ◽  
Diana Armesto ◽  
...  
Keyword(s):  
Body Mass Index ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Body Mass ◽  
Multicenter Study ◽  
Disease Registry ◽  
Factors Associated ◽  
Low Body Mass Index

scholarly journals Developmental Trajectory of Height, Weight, and BMI in Children and Adolescents at Risk for Huntington’s Disease: Effect of mHTT on Growth

2020 ◽  
Vol 9 (3) ◽  
pp. 245-251
Author(s):  
Alexander Tereshchenko ◽  
Ellen van der Plas ◽  
Katherine D. Mathews ◽  
Eric Epping ◽  
Amy L. Conrad ◽  
...  
Keyword(s):  
Body Mass Index ◽  
At Risk ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Body Mass ◽  
Child And Adolescent ◽  
Linear Increase ◽  
Developmental Trajectory ◽  
Mutant Form ◽  
The Impact

Background: The gene (Huntingtin or HTT) causing Huntington’s disease (HD) is vital for development and is expressed throughout the brain and body lifelong. The mutant form (mHTT) may influence growth and development. Objective: To determine the impact of mHTT on human measures of growth, including height, weight, and body mass index (BMI), between child and adolescent carriers of mHTT and control peers. Methods: Children ages 6–18 years of age (n = 186) at risk for HD were enrolled in the KidsHD study. For research purposes only, genetic testing was performed to classify participants as Gene-Expanded (GE = 78) or as Gene Non-Expanded (GNE = 108). Outcome measures included height, weight, and body mass index (BMI). Mixed models were used to determine if non-linear age trends differed between groups for BMI, height, and weight. Results: Differences were seen in the trajectory of BMI in which the GE group reached a plateau in late adolescence with no further increase, compared with a nearly linear increase in the GNE group. There was a significant sex interaction pattern where GE males were taller than GNE males in adolescence, in the presence of similar weight. In contrast, GE females weighed significantly less than their GNE counterparts in adolescence, in the presence of similar height. Conclusion: Measures of growth are abnormal in child and adolescent carriers of mHTT, decades before HD onset. Although further studies are needed for replication, the current findings suggest that developmental aberrations may be systemic and a vital part of disease pathology.

scholarly journals A survey-based study identifies common but unrecognized symptoms in a large series of juvenile Huntington's disease

2017 ◽  
Vol 7 (5) ◽  
pp. 307-315 ◽  
Author(s):  
Amelia D Moser ◽  
Eric Epping ◽  
Patricia Espe-Pfeifer ◽  
Erin Martin ◽  
Leah Zhorne ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Large Series ◽  
Juvenile Huntington’S Disease

Juvenile Huntington's Disease Interview Schedule

2008 ◽  
Author(s):  
Helen M. Brewer ◽  
Virginia Eatough ◽  
Jonathan A. Smith ◽  
Cath A. Stanley ◽  
Neil W. Glendinning ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Interview Schedule ◽  
Juvenile Huntington’S Disease

Biological and clinical manifestations of juvenile Huntington's disease: a retrospective analysis

2018 ◽  
Vol 17 (11) ◽  
pp. 986-993 ◽  
Author(s):  
Caterina Fusilli ◽  
Simone Migliore ◽  
Tommaso Mazza ◽  
Federica Consoli ◽  
Alessandro De Luca ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Retrospective Analysis ◽  
Clinical Manifestations ◽  
Juvenile Huntington’S Disease

Drug-Resistant Myoclonic Epilepsy Revealing Juvenile Huntington's Disease: A Case Report

2018 ◽  
Vol 07 (01) ◽  
pp. 021-023
Author(s):  
Fatma Feki ◽  
Chahnez Triki ◽  
Nesrine Amara
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Clinical History ◽  
Myoclonic Epilepsy ◽  
Drug Resistant ◽  
Dna Testing ◽  
History Of ◽  
Progressive Myoclonic Epilepsy ◽  
Juvenile Huntington’S Disease ◽  
Patients Experience

AbstractJuvenile Huntington's disease (JHD) shares many general clinical features with the adult form. One important difference is that JHD patients experience more epileptic manifestations, sometimes difficult to control. We describe an atypical clinical picture of a genetically confirmed JHD patient diagnosed during evaluation for a progressive myoclonic epilepsy. A female patient with a family history of psychiatric disorders developed recurrent drug-resistant myoclonic seizures at the age of 6 years, followed by extrapyramidal symptoms (rigidity and dystonia). Cognitive impairment, akinetic rigidity syndrome, and dystonia were noticed at the age of 10 years. Epileptiform abnormalities were noted in ictal electroencephalography. Magnetic resonance imaging showed brain atrophy. Genetic testing for HD confirmed the diagnosis. JHD can initially manifest as myoclonic epilepsy. A DNA testing should be performed if clinical history is suggestive.

scholarly journals C-59 Sex Differences as Predictors for Executive and Intellectual Functioning in Juvenile Huntington’s Disease Patients

2019 ◽  
Vol 34 (6) ◽  
pp. 1088-1088
Author(s):  
A Zwicker ◽  
C Cederberg ◽  
P Espe-Pfeifer ◽  
P Nopoulos
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Behavioral Problems ◽  
Intellectual Functioning ◽  
Exploratory Research ◽  
General Ability ◽  
Neurodegenerative Process ◽  
Partial Correlations ◽  
Juvenile Huntington’S Disease ◽  
Shifting Attention

Abstract Objective Juvenile Huntington's Disease (JHD) is an extremely rare autosomal dominant neurodegenerative disease with onset in childhood or teenage years. Although there are many similarities with the adult form of the disease, JHD has a clinically distinct presentation. Some common symptoms include behavioral problems and cognitive decline. However, given the rarity of juvenile presentations, limited research exists regarding sex difference performances on executive and intellectual functioning in a JHD population. Thus, exploratory research was conducted to investigate such findings. Method Data from the Kids HD research study was analyzed (N = 58; mean age = 15.5, 50.8% female). Preliminary bivariate partial correlations, independent t-tests, and one way ANOVA tests were used to examine differences in executive and intellectual functioning between male and female participants. Results Performance significantly differed on several aspects of executive and intellectual functioning between sexes, including shifting attention (p = 0.007), verbal intellect (p = 0.019), and general ability intellect (p = 0.041). Significant differences were also observed regarding inhibition (p = 0.003), verbal categorical fluency (p = 0.021), and sorting (p = < 0.001). Conclusions Results suggest that there are significant differences in executive and intellectual functioning between sexes. In particular, males had more difficulty shifting attention, despite higher verbal intellect and higher general ability intellect. Females demonstrated greater inhibition, while males demonstrated stronger verbal categorical fluency and abstract reasoning. Between sexes, results indicate evidence of performance differences across tasks of executive and intellectual functioning. Such findings are consistent with a subcortical neurodegenerative process, such as HD.

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