HDQLIFE: development and assessment of health-related quality of life in Huntington disease (HD).

PURPOSE: Huntington disease (HD) is a chronic, debilitating genetic disease that affects physical, emotional, cognitive, and social health. Existing patient-reported outcomes (PROs) of health-related quality of life (HRQOL) used in HD are neither comprehensive, nor do they adequately account for clinically meaningful changes in function. While new PROs examining HRQOL (i.e., Neuro-QoL-Quality of Life in Neurological Disorders and PROMIS-Patient-Reported Outcomes Measurement Information System) offer solutions to many of these shortcomings, they do not include HD-specific content, nor have they been validated in HD. HDQLIFE addresses this by validating 12 PROMIS/Neuro-QoL domains in individuals with HD and by using established PROMIS methodology to develop new, HD-specific content. METHODS: New item pools were developed using cognitive debriefing with individuals with HD, and expert, literacy, and translatability reviews. Existing item banks and new item pools were field tested in 536 individuals with prodromal, early-, or late-stage HD. RESULTS: Moderate to strong relationships between Neuro-QoL/PROMIS measures and generic self-report measures of HRQOL, and moderate relationships between Neuro-QoL/PROMIS and clinician-rated measures of similar constructs supported the validity of Neuro-QoL/PROMIS in individuals with HD. Exploratory and confirmatory factor analysis, item response theory, and differential item functioning analyses were utilized to develop new item banks for Chorea, Speech Difficulties, Swallowing Difficulties, and Concern with Death and Dying, with corresponding six-item short forms. A four-item short form was developed for Meaning and Purpose. CONCLUSIONS: HDQLIFE encompasses both validated Neuro-QoL/PROMIS measures, as well as five new scales in order to provide a comprehensive assessment of HRQOL in HD.

Age-group differences in facets of positive and negative affect.

OBJECTIVES: The higher order structure of Positive Affect (PA) and Negative Affect (NA) is comparable in self-report affect data from younger and older adults. The current study advances this work by comparing the factor structure of facets of PA and NA in older and younger adults using exploratory and confirmatory factor analyses. METHOD: Older (N = 203; M age = 73.5 years, range 65-92) and younger (N = 349; M age = 19.1 years, range 18-30) adults completed the Positive and Negative Affect Schedule-Expanded Form (PANAS-X) (Watson, D., & Clark, L.A. (1999). Manual for the Positive and Negative Affect Schedule -- Expanded Form. Iowa City, IA: The University of Iowa), which measures General PA and NA as well as three facets of PA (Joviality, Self-Assurance, and Attentiveness) and four facets of NA (Fear, Sadness, Guilt, and Hostility). RESULTS: Item-level exploratory factor analyses of the facet scales revealed structures that were similar in older and younger adults; however, older adult solutions were more diffuse and diverged more from the PANAS-X scale structure. The facet of Sadness exhibited the largest age-group difference, relating more to guilt and anxiety in older than younger adults. CONCLUSION: Older adults may discriminate less amongst specific affect terms or may experience greater affective heterogeneity. Further, Sadness may manifest in age-specific ways. The construct variance of Sadness, and how this issue might be related to the assessment of depression in older adults, is discussed.

Testosterone deficiency and apathy in Parkinson's disease: a pilot study.

BACKGROUND: Low testosterone in men with Parkinson's disease may be associated with non-motor symptoms of the disease, such as apathy. OBJECTIVE: To determine the association between free serum testosterone level and apathy in elderly men with Parkinson's disease. METHODS: Consecutive non-demented patients (n = 49) and knowledgeable informants (n = 40) participated in the study. Patients and informants reported on apathy using the Frontal Systems Behavior Scale and two visual analogue scales. Patients also provided self reported symptoms of depression on the Beck depression inventory-II. Blood samples were drawn at the time of assessment to determine testosterone levels. RESULTS: A low total testosterone concentration was found in 46.9% of the patients, defined as < or = 325 ng/dl. Free testosterone was significantly correlated with both patient reported and informant reported apathy, independent of disease severity. CONCLUSIONS: Apathy is common in Parkinson's disease and is inversely correlated with free testosterone. Testosterone replacement therapy could be considered as a potential treatment for apathy in some men with Parkinson's disease. More research is needed to replicate these findings and to investigate the response to treatment.

Neuropsychiatric aspects of Huntington's disease.

OBJECTIVE: Neuropsychiatric symptoms are common in Huntington's disease and have been considered its presenting manifestation. Research characterising these symptoms in Huntington's disease is variable, however, encumbered by limitations within and across studies. Gaining a better understanding of neuropsychiatric symptoms is essential, as these symptoms have implications for disease management, prognosis, and quality of life for patients and caregivers. METHOD: Fifty two patients with Huntington's disease were administered standardised measures of cognition, psychiatric symptoms, and motor abnormalities. Patient caregivers were administered the neuropsychiatric inventory. RESULTS: Ninety eight per cent of the patients exhibited neuropsychiatric symptoms, the most prevalent being dysphoria, agitation, irritability, apathy, and anxiety. Symptoms ranged from mild to severe and were unrelated to dementia and chorea. CONCLUSIONS: Neuropsychiatric symptoms are prevalent in Huntington's disease and are relatively independent of cognitive and motor aspects of the disease. Hypothesised links between neuropsychiatric symptoms of Huntington's disease and frontal-striatal circuitry were explored. Findings indicate that dimensional measures of neuropsychiatric symptoms are essential to capture the full range of pathology in Huntington's disease and are vital to include in a comprehensive assessment of the disease.

Effects of depression on working memory in presymptomatic Huntington's disease.

Cognitive decline may precede motor symptoms in Huntington's disease (HD). Depression is common in HD and has also been linked with cognitive impairment. The contribution of depression to cognition in individuals presymptomatic for HD (N=15) was investigated. Tests from the Cambridge Automated Neuropsychological Assessment Battery measured visual and working memory. Depression was assessed with the Beck Depression Inventory and the Unified Huntington's Disease Rating Scale. Depressed mood and estimated time to disease onset, calculated by using DNA mutation length, both were significant predictors of working memory performance. Findings are consistent with and contribute to existing research with individuals presymptomatic for HD, identifying a potentially remediable contribution to cognitive decline (i.e., depressed mood).

Ecological validity of neuropsychological and personality measures of executive functions.

Associations between two types of measures of executive functions, namely, neuropsychological and personality, and measures of real-world behavior were investigated. Undergraduate students were administered neuropsychological measures of executive functions and completed a personality questionnaire developed to measure traits central to the construct of executive functions. Participants also reported on their behavior. Hierarchical regressions indicated that neuropsychological and personality measures of executive functions were significant predictors of different types of behavior. Neuropsychological measures predicted work behaviors and personality measures predicted substance use, risk-taking, and aggressive behaviors. Findings highlight the importance of including personality assessment in standard neuropsychological assessment in order to maximize ability to predict real-world behaviors relevant to independent and socially responsible functioning.

Neurobehaviors and psychotic symptoms in Alzheimer's disease.

Psychotic symptoms are common in Alzheimer's disease (AD) and clinicoanatomical and neuropsychological evidence indicate an association between these symptoms and frontal lobe dysfunction. Neuro-behaviors associated with frontal dysfunction were assessed in Alzheimer's disease (AD) patients with (n = 20) and without psychotic symptoms (n = 21) matched for mean age, education, gender, and dementia severity. The Frontal Lobe Personality Scale (FLOPs) was completed by patient caregivers to measure behaviors typically associated with frontal dysfunction. Findings indicated that AD patients with psychotic symptoms exhibited significantly greater neurobehavioral dysfunction (FLOPs M = 130.69, SD = 24.70) than AD patients without psychotic symptoms (FLOPs M = 111.10, SD = 25.83). Subscale analyses indicated that psychotic AD patients were more dis-inhibited (M = 28.28, SD = 7.54) than patients without psychotic symptoms (M = 20.92, SD = 4.9). Findings are consistent with and contribute to previous neuropsychological and clinicoanatomical research suggesting increased frontal dysfunction in AD with psychotic symptoms and lend additional empirical support to subtyping AD based on the presence of psychotic symptoms. Furthermore, findings provide preliminary evidence indicating which specific type of neurobehavioral abnormalities are related to the presence of distressing psychotic symptoms.

Mothers' personality and its interaction with child temperament as predictors of parenting behavior.

In this longitudinal, multimethod investigation, the authors examined mothers' personality and its interaction with infants' negative emotionality as predictors of parenting behavior. When infants were 8-10 months old (N = 112), mothers completed personality self-reports, and the authors observed infants' negative emotionality in both standard procedures and naturalistic daily contexts. When infants were 13-15 months old (N = 108), the authors observed two aspects of parenting, power assertion and maternal responsiveness, in mother-child interactive contexts. Maternal personality alone and also in interaction with child emotionality predicted future parenting behaviors. The longitudinal links established between personality and parenting behaviors indicate the predictive utility of personality. Findings also highlight the bidirectionality of the early parent-child relationship.

Patients' perceptions of their participation in a clinical trial for postoperative Crohn's disease.

OBJECTIVE: To explore patients' perceptions of their participation in a randomized controlled trial. PATIENTS AND METHODS: A 27-item questionnaire was mailed to all patients who participated in a randomized controlled trial that determined the effectiveness of mesalamine in preventing the recurrence of Crohn's disease postoperatively. RESULTS: The response rate was 66% (99 of 149). Fifty-five per cent of the patients felt that they received better medical care than they otherwise would have and 53% liked taking the medication. Sixty-eight per cent of the patients did not feel that annual colonoscopy was too frequent and 81% felt that the time commitment did not significantly interfere with their job or other activities. Seventy-five per cent and 62% of the patients would have liked more information and education, respectively, about Crohn's disease incorporated into the trial. Although 91% of the patients would agree to participate in a future randomized controlled trial comparing medical therapies, only 44% would agree to participate in a future randomized controlled trial comparing medical with surgical therapies. CONCLUSIONS: The majority of patients were satisfied with their participation in the trial. A large proportion of the patients would participate again but would like more information and education incorporated into the trial. Furthermore, post-trial questionnaires may be helpful in the design of future trials.

Risk and significance of endoscopic/radiological evidence of recurrent Crohn's disease.

BACKGROUND & AIMS: The aim of this study was to determine the risk of endoscopic/radiological recurrence of Crohn's disease postoperatively and the long-term outcome. METHODS: A randomized placebo-controlled trial was performed to determine the effectiveness of mesalamine in preventing recurrent Crohn's disease postoperatively. Patients in the control group were examined endoscopically/radiologically before entry into and annually during the trial. Findings were classified as minimal or severe. RESULTS: There were 76 patients (49 men and 37 women; mean age, 37.1 +/- 13.2 years). Fifty (61.7%) had terminal ileal resections. Overall, 55 endoscopic/radiological recurrences were observed in 51 patients (67.1%). Expressed actuarially, the recurrence rate was 27.5% at 1 year (95% confidence interval [CI], 15.8%-37.6%), 60.8% at 2 years (95% CI, 46%-71.3%), and 77.3% at 3 years (95% CI, 62.7%-86.3%). Nineteen (37%) were symptomatic and 12 (24%) were initially asymptomatic but later became symptomatic (mean, 13.0 +/- 8.8 months), whereas 20 (39%) remained asymptomatic (mean, 16.9 +/- 17.4 months). Patients with severe endoscopic/radiological disease were significantly more likely to be or become symptomatic than those with minimal disease (23 of 32 vs. 8 of 19, respectively; P = 0.0437). CONCLUSIONS: This study suggests that postoperative endoscopic/radiological recurrences occur later than previously reported. Furthermore, many of these patients, especially with minimal disease, will remain asymptomatic.

Quality of life after Brooke ileostomy and ileal pouch-anal anastomosis. Comparison of performance status.

Although the clinical results of Brooke ileostomy are good, patients are permanently incontinent of stool and gas. Alternative operations designed to restore enteric continence, such as ileal pouch-anal anastomosis, must not only be as safe and effective as Brooke ileostomy, but should provide an improved quality of life in order to establish long-term acceptability. Ileal pouch-anal anastomosis has been performed safely and good functional results have been reported. The quality of life after ileal pouch-anal anastomosis, however, has not been documented. Two hundred ninety-eight ileal pouch patients and 406 Brooke ileostomy patients who had the operations performed for chronic ulcerative colitis or familial adenomatous polyposis formed the basis of the study. After adjusting for age, diagnosis, and reoperation rate, logistic regression analysis of performance scores in seven different categories was used to discriminate between operations. Median follow-up was longer in Brooke ileostomy patients than in ileal pouch patients (104 months vs. 47 months, respectively), and Brooke ileostomy patients were slightly older (38 years vs. 32 years). A great majority of patients in each group were satisfied (93% Brooke ileostomy; 95% ileal pouch-anal anastomosis). Thirty-nine per cent of Brooke ileostomy patients, however, desired a change in the type of ileostomy they had. At 47 months, ileal pouch patients had a median of 5 stools per day and 1 at night, 77% did not experience any daytime incontinence, while 22% reported occasional spotting. In each performance category, the performance score discriminated between operations, with the probability of having had an ileal pouch-anal anastomosis operation increasing with improvement in performance scores (p less than 0.05). We concluded that after ileal pouch-anal anastomosis, patients experienced significant advantages in performing daily activities compared to patients with Brooke ileostomy and thus may experience a better quality of life. These results help further to establish ileal pouch-anal anastomosis as a safe, attractive, and valid alternative to Brooke ileostomy.

A new bowel preparation for elective colon and rectal surgery. A prospective, randomized clinical trial.

A two-day cathartic/enema preparation with oral administration of erythromycin and neomycin was compared with an orthograde lavage preparation with oral administration of metronidazole and neomycin in a prospective randomized trial in 300 and 293 eligible patients, respectively, who were undergoing elective colon and rectal surgery. Patients were assessed for infections at six weeks after discharge from the hospital. The major infection rate was less than 1% and the minor infection rate was less than 4%. The overall infection rate was 4.2%. The type of bowel preparation used, the type of operation, and the addition of systemic antibiotic therapy did not affect infection rates significantly. We conclude that this one-day lavage technique, as described, is a safe, effective, economical, and preferred method of colonic preparation for elective colon and rectal surgery.

Gastrointestinal anastomoses. Factors affecting early complications.

A retrospective review of gastric and colonic anastomoses during a recent 12-month period was performed at the Mayo Clinic. One hundred sixty-nine patients had gastroduodenal or gastrojejunal anastomoses (Group I). Five hundred nineteen patients had ileocolonic or ileorectal (222) and colocolonic or colorectal (297) anastomoses (Group II). Major anastomotic complication rates for Group I patients were: leaks, 1%; hemorrhage, 2%; and stenosis or obstruction, 2%. Reoperations and deaths secondary to anastomotic complications during the postoperative period were 2% and 0.6%, respectively. Corresponding rates for Group II were 2%, 1%, and 4%, with reoperative and anastomotic death rates of 1% and 0.2%, respectively. In Group I patients, length of operation had a significant effect (p less than 0.01) on anastomotic complications. In Group II patients, a significant increase in complications was related to the presence of obstruction (p less than 0.001), recent weight loss (greater than 10 pounds) (p less than 0.02), malignancy (p less than 0.04), and sepsis (p less than 0.05).

Sphincter repair for fecal incontinence after obstetrical or iatrogenic injury.

Forty patients with fecal incontinence underwent sphincter repair between 1975 and 1984. Divided sphincter musculature resulted from obstetrical injury in 23 and previous anorectal surgery in 17. Eighteen had undergone a previous attempt at repair. Fifteen patients experienced seepage of stool and 25 had gross incontinence. In nine patients, reconstruction of the external sphincter was by overlap of the muscle ends. Twenty-four others underwent accurate approximation of the external sphincter muscle and anterior plication of the levator muscles, and in seven the anal canal was made smaller by narrowing the anal orifice. Follow-up was an average of 67 months after operation (range, 2.4 to 166 months). Continence was objectively improved in 62 percent (P less than .01) when performance criteria were analyzed by Wilcoxon signed-rank test, although 85 percent of the patients reported subjective improvement. Requirements for protective pads were reduced in 57 percent (P less than .01) and fewer social limitations were experienced in 52 percent (P less than .01). There was no significant correlation between outcome and type of operation.

Anorectal Crohn's disease. A long-term perspective.

A group of 86 patients with anorectal Crohn's disease were followed up from ten to 40 years to determine the course of the disease and the number of patients who later required proctectomy. The overall cumulative probability of avoiding proctectomy was 91.6 percent at ten years and 82.5 percent at 20 years. Resection of all proximal Crohn's disease did not ameliorate the anorectal disease, except in patients who had all proximal disease removed and had no recurrence.

Serological comparison of the Escherichia coli prototype strains for the F(Y) and Att 25 adhesions implicated in neonatal diarrhoea in calves.

Slide agglutination tests using single absorbed and double absorbed antisera indicated that the Att 25 prototype Escherichia coli strain 25 KH9 produces the F(Y) adhesion; that this E coli also produces at least one other surface antigen not found on the F(Y) prototype E coli strain 11a; and that F(Y)+ E coli strain 28a produces at least one other surface antigen not produced by the prototype strains for the F(Y) and Att 25 antigens. These antigens were found on E coli isolated from outbreaks of calf diarrhoea in the United Kingdom.

Influence of sigmoid resection on progression of diverticular disease of the colon.

The surgeon is frequently confronted with the problem of how much colon to resect when operating on patients with colonic diverticulosis or diverticulitis. Two questions arise: will diverticulosis progress in the proximal colon if only the sigmoid is removed, and will diverticulitis recur in the more proximal diverticula? To evaluate these potential problems, the histories were reviewed of 61 patients who had elective sigmoid resection for diverticular disease and who had barium enema examinations before operation, early during the postoperative period, and at least five years later. Progression of diverticulosis was noted in only nine (14.7 per cent) patients on repeat barium-enema examination five to nine years after resection; the progression was noted to be minimal in all nine. Seven patients (11.4 per cent) had signs and symptoms of recurrent diverticulitis. Only three patients demonstrated progression of diverticulosis and recurrent diverticulitis. We see no benefit in resecting all of the diverticula-bearing colon after adequate sigmoid resection, as there is minimal progression in the diverticular process and the risk of recurrence is low.