Engagement with genetic discrimination: concerns and experiences in the context of Huntington disease.

It has been over 20 years since the inception of predictive testing for Huntington disease (HD), yet the social implications of knowing one's genetic risk for HD have not been fully explored. Genetic discrimination (GD) is a potential risk associated with predictive testing. Although anecdotal reports of GD have been documented, there is a paucity of research on the nature and experiences of GD in the context of HD.

Perceptions of discrimination among persons who have undergone predictive testing for Huntington's disease.

Potential discrimination from genetic testing may undermine technological advances for health care. Researching long-term consequences of testing for genetic conditions that may lead to discrimination is a public health priority. The consequences of genetic discrimination generate social, health, and economic burdens for society by diminishing opportunities for at-risk individuals in a range of contexts.

Beyond disgust: impaired recognition of negative emotions prior to diagnosis in Huntington's disease.

Previous studies of emotion recognition suggest that detection of disgust relies on processing within the basal ganglia and insula. Research involving individuals with symptomatic and pre-diagnostic Huntington's disease (HD), a disease with known basal ganglia atrophy, has generally indicated a relative impairment in recognizing disgust. However, some data have suggested that recognition of other emotions (particularly fear and anger) may also be affected in HD, and a recent study found fear recognition deficits in the absence of other emotion-recognition impairments, including disgust.

Verbal episodic memory declines prior to diagnosis in Huntington's disease.

Previous studies of verbal episodic memory in pre-diagnostic Huntington's disease (HD) have yielded mixed results; some evidence suggests that memory decline is evident prior to the onset of pronounced neurological signs of HD, whereas other data indicate that memory function remains normal throughout the pre-diagnostic period. This study examines verbal episodic memory in a sample of CAG expanded individuals who have not yet been clinically diagnosed, and who represent a wide range of points along the continuum from health to disease. The Hopkins Verbal Learning Test-Revised (HVLT-R) was administered to 479 participants (428 with the HD CAG expansion and 51 without), and performance was compared to neurobiological indices of disease progression, including a DNA-based estimate of proximity to clinical diagnosis, magnetic resonance imaging (MRI) measures of striatal volume, and neurologist ratings of motor signs.

"No one else sees the difference: "family members' perceptions of changes in persons with preclinical Huntington disease.

Manifestations of Huntington disease (HD) prior to clinical diagnosis are not well understood. This study documents adult family members' perceptions of changes and their attempts to manage these changes in persons who had received a positive predictive molecular HD test prior to clinical diagnosis. Data were obtained from 19 adult family members in six focus groups in the US and Canada and one individual interview in the US.

Probability of obsessive and compulsive symptoms in Huntington's disease.

Background: The purpose of this study was to examine the probability of obsessive and compulsive (OC) symptoms across stages of Huntington's disease (HD) with both cross sectional and longitudinal data.

Methods: We present the largest sample to date of individuals at risk for HD (N = 3964). Obsessive and compulsive symptoms were assessed with the Unified Huntington's Disease Rating Scale OC items.

Preparing for preventive clinical trials: the Predict-HD study.

Background: The optimal design and outcome measures for preventive clinical trials in neurodegenerative diseases are unknown.

Objective: To examine measures that may be associated with disease in the largest cohort ever recruited of prediagnosed individuals carrying the gene expansion for Huntington disease (HD).

Design: The Predict-HD study is a multicenter observational research study in progress at 17 sites in the United States, 4 in Canada, and 3 in Australia.

Brain structure in preclinical Huntington's disease.

Background: Huntington's disease (HD) is traditionally conceptualized as a degenerative disease of the striatum. Recent scientific advances, however, have suggested neurodevelopmental contributions and extrastriatal brain abnormalities. This study was designed to assess the morphology of the brain in participants who had previously undergone elective DNA analyses for the HD mutation who did not currently have a clinical diagnosis of HD (preclinical HD subjects).