Strategies used by teens growing up in families with Huntington disease.

The purpose of this study was to identify helpfulness of strategies used by teens growing up in families with Huntington disease (HD). Forty-four participants responded to a mailed HD Family Survey-Teens Strategies. Strategies were those with strong positive correlation between use and perceived helpfulness, and those with negative or inverse relationships. Obtaining information, thinking about or doing something else, and actions on behalf of the parent with HD were rated as highest use and perceived helpfulness. Emotional suppression had high use but low helpfulness. Participants reported using numerous helpful strategies. Social support was often unavailable to help manage teen concerns.

Couples' coping in prodromal Huntington disease: a mixed methods study.

Huntington disease (HD) includes a prodromal phase with behavioral, cognitive, and motor function decline occurring up to 15 years prior to diagnosis. This study used mixed methods to examine how people in the prodromal phase and their companions coped with noticed changes. Twenty-three couples completed a semi-structured interview and Brief COPE. Participants with prodromal HD used acceptance, emotional support, and planning most frequently; companions used acceptance, planning, and active coping. Least frequently used coping strategies for each were denial, behavioral disengagement, and substance use. Qualitative interviews revealed coping strategies not included in the Brief COPE. Participants with prodromal HD used prescription medications, coping as a couple, hope, and self-monitoring; companions used hope and helping their partners. Many of the coping procedures were rated as effective, especially when changes were not severe. Couples may benefit from counseling that emphasizes using active coping strategies for changes that can be compensated for and acceptance for changes that cannot in prodromal HD. Findings from this study may be helpful for counseling patients and significant others facing other neurodegenerative conditions with prodromal or early phases, such as Alzheimer disease and Parkinson disease.

Cognitive change in patients with Huntington disease on the Repeatable Battery for the Assessment of Neuropsychological Status.

Huntington disease (HD) is a neurodegenerative disease associated with cognitive, motor, and psychiatric deterioration over time. Although there is currently no cure for HD, there has been a surge of clinical trials available to patients with HD over the past 5 years. However, cognitive measures have generally been lacking from these trials. A brief, repeatable neuropsychological battery is needed to assess cognitive endpoints. The Repeatable Battery for the Assessment of Neuropsychological Status (RBANS) may be useful for assessing change in interventional studies or for clinical monitoring. A total of 38 patients with HD were assessed using the RBANS, other cognitive tests, and the standardized HD battery (Unified Huntington's Disease Rating Scale, UHDRS) at two clinic visits approximately 16 months apart. The RBANS Attention Index, as well as individual subtest scores on Coding, Digit Span, List Recognition, Figure Copy, and Figure Recall all declined significantly over this interval. Performance on the UHDRS cognitive tests (Symbol Digit Modalities; Stroop Color, and Stroop Word) also declined, as did functional capacity. Results suggest that cognitive changes were detected both on established cognitive tasks used in HD research and on the RBANS in patients with measurable functional decline. The RBANS provided additional information about other cognitive domains affected (e.g., memory) and may be a useful measure for tracking longitudinal change.

Randomized controlled trial of atomoxetine for cognitive dysfunction in early Huntington disease.

BACKGROUND: Cognitive symptoms are associated with functional disability in Huntington disease; yet, few controlled trials have examined cognitive treatments that could improve patient independence and quality of life. Atomoxetine is a norepinephrine reuptake inhibitor approved for treatment of attention-deficit/hyperactivity disorder. METHODS: Twenty participants with mild Huntington disease who complained of inattention were randomized to receive atomoxetine (80 mg/d) or placebo in a 10-week double-blind crossover study. Primary outcome measures were self-reported attention and attention and executive neuropsychological composite scores. Secondary outcomes were psychiatric and motor symptom scores. RESULTS: The rate of reported adverse effects while on atomoxetine was 56% (vs 35% on placebo), which most commonly included dry mouth (39%), loss of appetite (22%), insomnia (22%), and dizziness (17%). There were no serious adverse events related to atomoxetine. There were statistically significant, although mild, increases in heart rate and diastolic blood pressure on atomoxetine, consistent with other studies and not requiring medical referral. There were no significant improvements while on atomoxetine compared with placebo on primary outcomes. However, there was evidence of significant placebo effects on self-reported attention and psychiatric functions. There were no group differences on the Unified Huntington's Disease Rating total motor score. CONCLUSIONS: Atomoxetine demonstrated no advantages over placebo for primary or secondary outcomes. Although atomoxetine was not effective at improving attention at this dose, its safety and tolerability were similar to other studies.

Patient and caregiver quality of life in Huntington's disease.

Little is known about subjective perceptions of quality of life (QOL) in Huntington's disease (HD). The current study determined correlates of patient and caregiver QOL and assessed change over time. Participants were 22 patient-caregiver dyads, who rated QOL at baseline and 6 months later. Overall, patients' functional and cognitive impairment were significantly correlated with patient and caregiver QOL. Neuropsychiatric symptoms had differential impact on patient and caregiver QOL. Furthermore, when patients recalled their QOL about a previous time, their recall may have been negatively biased. Clinical implications of results are discussed. Future work is needed because subjective QOL is an important outcome measure in therapeutic trials.