Medication Management Capacity and Its Neurocognitive Correlates in Huntington’s Disease

Abstract Objective Although medication management is a necessary daily activity for individuals with Huntington’s disease (HD), medication management abilities and their relation to cognitive functioning have not been evaluated. Method Twenty individuals with HD and 20 healthy adults (HA) completed the Medication Management Abilities Assessment (MMAA). Individuals with HD also completed a self-report medication management measure and neuropsychological tests assessing executive function, retrospective memory, and prospective memory. Results Individuals with HD performed significantly poorer and made more undertaking errors on the MMAA as compared to HA. No group differences were found in overtaking errors. In the HD group, significant associations were found between undertaking errors and perceived medication management ability as well as between MMAA task performance and measures assessing prospective memory and executive functions. Conclusions Medication management capacity was negatively affected in individuals with HD and may be associated with difficulty remembering to take medications in the future.

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Behavioral Deficits in Juvenile Onset Huntington’s Disease

Brain Sciences ◽

10.3390/brainsci10080543 ◽

2020 ◽

Vol 10 (8) ◽

pp. 543

Author(s):

Kathleen E. Langbehn ◽

Ashley M. Cochran ◽

Ellen van der Plas ◽

Amy L. Conrad ◽

Eric Epping ◽

...

Keyword(s):

Young Adults ◽

Huntington's Disease ◽

Huntington’S Disease ◽

Cag Repeat ◽

Self Report ◽

Cag Repeats ◽

Group Differences ◽

Linear Regression Models ◽

Juvenile Onset ◽

Quantitative Evidence

Reports of behavioral disturbance in Juvenile-Onset Huntington’s Disease (JOHD) have been based primarily on qualitative caregiver reports or retrospective medical record reviews. This study aims to quantify differences in behavior in patients with JOHD using informant- and self-report questionnaires. Informants of 21 children/young adults (12 female) with JOHD and 115 children/young adults (64 female) with a family history of Huntington’s Disease, but who did not inherit the disease themselves (Gene-Non-Expanded; GNE) completed the Behavior Rating Inventory of Executive Function (BRIEF) and the Pediatric Behavior Scale (PBS). Mixed linear regression models (age/sex adjusted) were conducted to assess group differences on these measures. The JOHD group had significantly higher scores, indicating more problems, than the GNE group on all BRIEF subscales, and measures of Aggression/Opposition and Hyperactivity/Inattention of the PBS (all p < 0.05). There were no group differences in Depression/Anxiety. Inhibit, Plan/Organize, Initiate, and Aggression/Opposition had significant negative correlations with Cytosine-Adenine-Guanine (CAG) repeat length (all p < 0.05) meaning that individuals with higher CAG repeats scored lower on these measures. There was greater discrepancy between higher informant-vs. lower self-reported scores in the JOHD group, supporting the notion of lack of insight for the JOHD-affected group. These results provide quantitative evidence of behavioral characteristics of JOHD.

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“Forgetting to Remember” in Huntington's Disease: A Study of Laboratory, Semi-Naturalistic, and Self-Perceptions of Prospective Memory

Journal of the International Neuropsychological Society ◽

10.1017/s1355617713001355 ◽

2013 ◽

Vol 20 (2) ◽

pp. 192-199 ◽

Cited By ~ 11

Author(s):

Diane R. Nicoll ◽

Eva Pirogovsky ◽

Steven Paul Woods ◽

Heather M. Holden ◽

J. Vincent Filoteo ◽

...

Keyword(s):

Huntington's Disease ◽

Huntington’S Disease ◽

Prospective Memory ◽

Memory Deficit ◽

Self Report ◽

Executive Processes ◽

Self Perceptions ◽

Event Based ◽

And Control ◽

Omission Errors

AbstractProspective memory (PM) is dependent on executive processes known to be impaired in Huntington's disease (HD); however, no study to the authors’ knowledge has investigated PM in this group. We examined performance-based, semi-naturalistic, and self-reported PM in 20 individuals diagnosed with mild–moderate HD and 20 demographically similar controls. Relative to controls, HD participants demonstrated significantly lower scores in time-based PM, event-based PM (at a trend level), and the semi-naturalistic PM trial, all of which were marked by omission errors. HD participants demonstrated comparable recognition memory for the PM intentions relative to controls. HD and control participants also showed comparable scores in self-reported PM complaints. The results suggest that HD is associated with deficits in the strategic aspects of PM. HD-associated PM deficits also are evident in real-world situations, which may relate to an apparent meta-memory deficit for PM functioning as indicated by HD participants’ overestimation of their PM performance on self-report. (JINS, 2014, 20, 1–8)

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Everyday Functioning in Huntington’s Disease: A Laboratory-Based Study of Financial Management Capacity

Applied Neuropsychology Adult ◽

10.1080/23279095.2015.1125904 ◽

2016 ◽

Vol 24 (2) ◽

pp. 176-182 ◽

Cited By ~ 2

Author(s):

David P. Sheppard ◽

Eva Pirogovsky-Turk ◽

Steven Paul Woods ◽

Heather M. Holden ◽

Diane R. Nicoll ◽

...

Keyword(s):

Huntington's Disease ◽

Huntington’S Disease ◽

Financial Management ◽

Everyday Functioning ◽

Management Capacity

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Assessment of medication management capacity in a predominantly African American and Caribbean American sample of adults with intractable epilepsy

Epilepsy & Behavior ◽

10.1016/j.yebeh.2018.09.022 ◽

2018 ◽

Vol 88 ◽

pp. 308-314 ◽

Cited By ~ 2

Author(s):

Seth A. Margolis ◽

Jeffrey S. Gonzalez ◽

Jessica Spindell ◽

Maliheh Mohamadpour ◽

Arthur C. Grant ◽

...

Keyword(s):

African American ◽

Medication Management ◽

Intractable Epilepsy ◽

American Sample ◽

Management Capacity ◽

Medication Management Capacity

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Social Cognition, Executive Functions and Self-Report of Psychological Distress in Huntington’s Disease

PLoS Currents ◽

10.1371/currents.hd.bba3a680813122013e6d3e8a144c1da8 ◽

2016 ◽

Cited By ~ 2

Author(s):

Ida Unmack Larsen ◽

Tua Vinther-Jensen ◽

Jørgen Erik Nielsen ◽

Asmus Vogel ◽

Anders Gade

Keyword(s):

Psychological Distress ◽

Social Cognition ◽

Executive Functions ◽

Huntington's Disease ◽

Huntington’S Disease ◽

Self Report

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Social Cognition and Oxytocin in Huntington’s Disease: New Insights

Brain Sciences ◽

10.3390/brainsci8090161 ◽

2018 ◽

Vol 8 (9) ◽

pp. 161 ◽

Cited By ~ 2

Author(s):

Elisa Unti ◽

Sonia Mazzucchi ◽

Daniela Frosini ◽

Cristina Pagni ◽

Gloria Tognoni ◽

...

Keyword(s):

Social Cognition ◽

Huntington's Disease ◽

Huntington’S Disease ◽

Plasma Levels ◽

Neuropsychological Tests ◽

Statistical Significance ◽

Verbal Stimuli ◽

Body Tissues ◽

The Social ◽

State Examination

This study is aimed at relating social cognition in Huntington’s Disease (HD) to plasma levels of the social hormone oxytocin (OT). Indeed, HD patients commonly display reduced social skills and OT is involved in bonding behavior and improved recognition of facial emotions. Twelve mild-symptomatic HD patients (stage II Shoulson & Fahn) and 11 gender/age matched controls (healthy controls, HC), without concurrent psychiatric disorders, were investigated at baseline (T0) for OT plasma levels and social cognition through an extensive battery of neuropsychological tests. Social cognition was also re-examined after two years (T1) in 8 of the 12 patients. Results showed a trend for reduced T0-OT levels in HD vs. HC, mean ± stardard deviation: 6.5 ± 2.4 vs. 9.9 ± 7.2 pg/mL, without reaching statistical significance. At T0, patients showed significantly lower performances than controls at the “Faux-Pas” and “Strange Stories” tests (p < 0.05; p < 0.01); a reduced perception of visual emotions (p < 0.01) and verbal stimuli (p < 0.01) was also reported, involving anger, fear, and sadness (p < 0.05; p < 0.01). Additionally, in the HD population, OT concentrations positively correlated with T1-performances at Neutral\Faux-Pas test (p < 0.05), whereas the cognitive Montreal Cognitive Assessment (MoCA) and Mini Mental State Examination (MMSE) scores positively correlated with psychosocial perception at the “Strange Stories” and Karolinska Directed Emotional Faces (KDEF) tests (p < 0.05). This study, despite its limitations, supports correlations between OT and HD social cognition, suggesting a possible therapeutic use of this hormone. More subjects and additional body tissues/fluids, such as cerebrospinal fluid, should be investigated to confirm this hypothesis.

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F57 Psychiatric symptoms in huntington’s disease: correlations between interview and self-report measures in the capit-hd2 beta-testing study

10.1136/jnnp-2018-ehdn.158 ◽

2018 ◽

Author(s):

Isobel McMillan ◽

Duncan McLauchlan ◽

Monica Busse ◽

Anne-Catherine Bachoud-Lévi ◽

Ralf Reilmann ◽

...

Keyword(s):

Huntington's Disease ◽

Huntington’S Disease ◽

Psychiatric Symptoms ◽

Self Report ◽

Beta Testing

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

Scientific Reports ◽

10.1038/s41598-020-72250-w ◽

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.

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