scholarly journals Visual scanning and cognitive performance in prediagnostic and early-stage Huntington's disease

2009 ◽  
Vol 24 (4) ◽  
pp. 533-540 ◽  
Author(s):  
Tanya Blekher ◽  
Marjorie R. Weaver ◽  
Jeanine Marshall ◽  
Siu Hui ◽  
Jacqueline Gray Jackson ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Cognitive Performance ◽  
Early Stage ◽  
Visual Scanning

scholarly journals Fiberoptic endoscopic evaluation of swallowing in early-to-advanced stage Huntington’s disease

2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Antonio Schindler ◽  
Nicole Pizzorni ◽  
Jenny Sassone ◽  
Lorenzo Nanetti ◽  
Anna Castaldo ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Neurodegenerative Disorder ◽  
Early Stage ◽  
Clinical Care ◽  
Self Report ◽  
Advanced Stage ◽  
Endoscopic Evaluation ◽  
Silent Aspiration ◽  
Scale Scores

Abstract Huntington's disease (HD) is a neurodegenerative disorder characterized by motor disturbances, cognitive decline, and behaviour changes. A well-recognized feature of advanced HD is dysphagia, which leads to malnutrition and aspiration pneumonia, the latter being the primary cause of death in HD. Previous studies have underscored the importance of dysphagia in HD patients with moderate-to-advanced stage disease, but it is unclear whether dysphagia affects patients already at an early stage of disease and whether genetic or clinical factors can predict its severity. We performed fiberoptic endoscopic evaluation of swallowing (FEES) in 61 patients with various stages of HD. Dysphagia was found in 35% of early-stage, 94% of moderate-stage, and 100% of advanced-stage HD. Silent aspiration was found in 7.7% of early-stage, 11.8% of moderate-stage, and 27.8% of advanced-stage HD. A strong correlation was observed between disease progression and dysphagia severity: worse dysphagia was associated with worsening of motor symptoms. Dysphagia severity as assessed by FEES correlated with Huntington’s Disease Dysphagia Scale scores (a self-report questionnaire specific for evaluating swallowing in HD). The present findings add to our understanding of dysphagia onset and progression in HD. A better understanding of dysphagia onset and progression in HD may inform guidelines for standard clinical care in dysphagia, its recognition, and management.

H05 Prevalence And Correlates Of Neuropsychiatric Symptoms In Pre-manifested And Early Stage Huntington's Disease

2014 ◽  
Vol 85 (Suppl 1) ◽  
pp. A53-A53
Author(s):  
S. Martinez-Horta ◽  
J. Perez-Perez ◽  
M. Carceller ◽  
R. de Bobadilla ◽  
J. Pagonabarraga ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Early Stage ◽  
Neuropsychiatric Symptoms

Neuropsychological manifestations of the genetic mutation for Huntington's disease in presymptomatic individuals

2002 ◽  
Vol 8 (7) ◽  
pp. 918-924 ◽  
Author(s):  
JASON BRANDT ◽  
BARNETT SHPRITZ ◽  
ANN MARIE CODORI ◽  
RUSSELL MARGOLIS ◽  
ADAM ROSENBLATT
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Early Stage ◽  
Age At Onset ◽  
Disease Onset ◽  
Signs And Symptoms ◽  
Genetic Mutation ◽  
Clinically Normal ◽  
Dementing Illness ◽  
Brain And Behavior

A triplet repeat (CAG) expansion mutation in the huntingtin gene on chromosome 4 is responsible for Huntington's disease (HD). Presymptomatic genetic testing for this mutation has identified clinically normal persons who are virtually certain to develop this dementing illness if they live a normal lifespan. The present study sought to determine whether these “mutation-positive” persons have impairments in cognitive functioning. Seventy-five mutation-positive persons did not differ from 128 mutation-negative persons on tests selected for their sensitivity to early-stage HD. Interestingly, however, those with the mutation viewed themselves as more likely to develop HD than did those without the mutation. Among mutation-positive subjects, having a longer CAG repeat mutation was likewise not associated with cognitive impairment. However, being closer to estimated disease onset (a product of repeat length and parent's age at onset) was associated with selected cognitive impairments. When viewed in light of previous studies showing atrophy of the caudate nucleus and putamen in mutation-carriers who are close to onset but not those far from onset, these results suggest that subtle changes in brain and behavior may be detected shortly before subjects with the HD mutation develop sufficient signs and symptoms for diagnosis. Conceptual and methodological problems associated with the search for presymptomatic cognitive and behavioral indicators of dementing illness are discussed. (JINS, 2002, 8, 918–924.)

scholarly journals Corrigendum to “Neuropsychiatric symptoms are very common in premanifest and early stage Huntington's disease” [Parkinsonism Relat. Disord. 25C (2016) 58–64]

2016 ◽  
Vol 31 ◽  
pp. 161 ◽  
Author(s):  
Saul Martinez-Horta ◽  
Jesus Perez-Perez ◽  
Erik van Duijn ◽  
Ramon Fernandez-Bobadilla ◽  
Mar Carceller ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Early Stage ◽  
Neuropsychiatric Symptoms

scholarly journals Thalamic Atrophy in Huntington's Disease Co-varies with Cognitive Performance: A Morphometric MRI Analysis

2004 ◽  
Vol 15 (6) ◽  
pp. 846-853 ◽  
Author(s):  
Jan Kassubek ◽  
Freimut D. Juengling ◽  
Daniel Ecker ◽  
G. Bernhard Landwehrmeyer
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Cognitive Performance

Pilot Observations from a Multimodal Imaging Study of Dysphagic Patients in Early Stage Huntington'S Disease

2017 ◽  
Vol 152 (5) ◽  
pp. S928
Author(s):  
Emilia Michou ◽  
Iris Trender-Gerhard ◽  
Alexander Gerhard ◽  
David Craufurd ◽  
Karl Herholz ◽  
...  
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Multimodal Imaging ◽  
Early Stage ◽  
Imaging Study ◽  
Dysphagic Patients

scholarly journals Decreased Metabolism in the Cerebral Cortex in Early-Stage Huntington's Disease: A Possible Biomarker of Disease Progression?

2013 ◽  
Vol 9 (1) ◽  
pp. 21 ◽  
Author(s):  
Hyeeun Shin ◽  
Man Ho Kim ◽  
Su Jin Lee ◽  
Kyung-Han Lee ◽  
Mi-Jung Kim ◽  
...  
Keyword(s):  
Cerebral Cortex ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Disease Progression ◽  
Early Stage
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