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. RESULTS: The probability of meeting the threshold for obsessions and compulsions increased with greater disease severity. Those with no motor abnormalities ("at risk") had a 7% probability of obsessions and a 3.5% probability of compulsions; the peak probability for obsessions (24%) and compulsions (12%) occurred in patients with advanced disease with significant functional disability. CONCLUSIONS: The probability of OC symptoms is more than three times greater by stages 3 and 4 (clearly manifest disease) than in our at-risk group with no apparent motor abnormalities.

Critical periods of suicide risk in Huntington's disease.

Contrary to popular belief, receiving a diagnosis of a devastating fatal disease does not exacerbate, and may even alleviate, the risk of suicide. Suicidal ideation was examined in 4,171 individuals in the Huntington Study Group database. Participants were grouped according to a standardized neurological examination from 0 (i.e., normal examination) to 3 (definite Huntington's disease). Patients with an unequivocal diagnosis of Huntington's disease were further divided by stage of disease, from stage 1 (early) to stage 5 (end stage). Findings showed that the frequency of suicidal ideation doubled from 9.1% in at-risk persons with a normal neurological examination to 19.8% in at-risk persons with soft neurological signs and increased to 23.5% in persons with "possible Huntington's disease." In persons with a diagnosis of Huntington's disease, 16.7% had suicidal ideation in stage 1, and 21.6% had suicidal ideation in stage 2, whereas the proportion of Huntington's disease patients with suicidal ideation diminished thereafter. Findings suggest two critical periods for increased risk of suicide in Huntington's disease. The first critical period is immediately before receiving a formal diagnosis of Huntington's disease, and the second is in stage 2 of the disease, when independence diminishes. Although the underlying mechanisms of suicidal ideation in Huntington's disease are poorly understood, it is critical for health care providers to be aware of periods during which patients may be at an increased risk.

Very early semantic dementia with progressive temporal lobe atrophy: an 8-year longitudinal study.

BACKGROUND: Semantic dementia is a syndrome within the spectrum of frontotemporal lobar degenerations characterized by fluent progressive aphasia (particularly anomia) and loss of word meaning. OBJECTIVE: To report a unique case of very early semantic dementia with a slowly progressive course, allowing insights into the early natural history of this disorder. DESIGN: Case report. SETTING: A tertiary care center. PATIENT: A 62-year-old woman who presented with "memory loss" complaints. MAIN OUTCOME MEASURES: Clinical course, neuropsychological data, and magnetic resonance imaging results. RESULTS: The patient was first evaluated when the results of standard neuropsychological measures were normal but subtle left anterior temporal lobe atrophy was present. During the follow-up period of 8 years, she developed profound anomia and loss of word meaning associated with progressive left anterior temporal lobe atrophy, consistent with semantic dementia. In more recent years, anterograde memory impairment and mild prosopagnosia evolved in association with left hippocampal atrophy and subtle atrophy in the homologous gyri of the right anterior temporal lobe. She remains functionally independent despite her current deficits. CONCLUSIONS: Early identification of patients who will develop semantic dementia is difficult and might be missed with standard clinical, neuropsychological, and structural neuroimaging evaluations. Recognition of this relatively rare syndrome is important for early diagnosis and prognostication and particularly for therapeutic interventions in the future.

The Frontal Systems Behaviour Scale (FrSBe) as a predictor of community integration following a traumatic brain injury.

PRIMARY OBJECTIVE: This study examined the relationships between the Frontal Systems Behaviour Scale (FrSBe), neuropsychological tests and community integration outcomes among individuals with a history of TBI. METHODS: Seventy-six individuals with a history of TBI were consecutively recruited from patients seen in a Neuropsychology clinic in an academic healthcare setting. Participants completed neuropsychological tests, the FrSBe and the Community Integration Questionnaire (CIQ) as part of a standard clinical neuropsychological evaluation. RESULTS: The FrSBe was a significant predictor of community integration (CIQ) outcomes. Specifically, poorer executive functioning (FrSBe Executive Functioning) predicted lower CIQ Total Scores, while increased apathy (FrSBe Apathy) was associated with reduced CIQ Productivity. Regarding neuropsychological tests, only Delayed Memory remained as a predictor: higher scores were associated with enhanced CIQ Total Scores and CIQ Social Integration. Finally, female gender was associated with superior CIQ Total Scores, CIQ Home Integration and CIQ Productivity. CONCLUSIONS: While neuropsychological tests of executive functioning failed to add predictive power to models of community integration following TBI with this sample, the FrSBe, a measure of behavioural manifestations of frontal lobe dysfunction, did predict these important functional outcomes. This suggests that use of the FrSBe may enhance the ecological validity of information gathered during a clinical neuropsychological assessment.

"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. Changes reported by family members included problems in cognition and behavior, which are consistent with prior reports. In addition, family members observed changes in motor functioning, the ability to complete usual activities at work or at home, and interpersonal relationships with family and friends. Family members attempted to manage these changes by taking on new responsibilities and preparing for future caregiving. Lack of information about preclinical HD, difficulty in understanding changes that may represent early stages of HD, and efforts to maintain secrecy of the preclinical HD situation contribute to compromising the abilities of family members to respond to changes in the person with preclinical HD. Findings provide insights into alterations in cognition, behavior, and functioning observed by the family members prior to clinical diagnosis. Findings further support the need for a more comprehensive assessment and management of early HD symptoms as well as support for family members.

Depression and stages of Huntington's disease.

Individuals with manifest Huntington's disease (HD) were interviewed with regard to the presence, frequency, and severity of depression symptoms to better characterize depressed mood across the disease course in HD. Rates of depression were more than twice that found in the general population. One-half reported that they had sought treatment for depression, and more than 10% reported having at least one suicide attempt. The proportion of HD patients endorsing significant depression diminished with disease progression. Despite the public health impact of depression, available treatments are underutilized in HD, and research is needed to document the efficacy and effectiveness of standard depression treatments in this population.