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- Almqvist, Elisabeth W., 1958-
(författare)
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A randomized, placebo-controlled trial of coenzyme Q10 and remacemide in Huntington's disease
- 2001
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Ingår i: Neurology. - : Ovid Technologies (Wolters Kluwer Health). - 0028-3878 .- 1526-632X. ; 57:3, s. 397-404
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Tidskriftsartikel (refereegranskat)abstract
- OBJECTIVES: To determine whether chronic treatment with coenzyme Q10 or remacemide hydrochloride slows the functional decline of early Huntington's disease (HD).METHODS: The authors conducted a multicenter, parallel group, double-blind, 2 x 2 factorial, randomized clinical trial. Research participants with early HD (n = 347) were randomized to receive coenzyme Q10 300 mg twice daily, remacemide hydrochloride 200 mg three times daily, both, or neither treatment, and were evaluated every 4 to 5 months for a total of 30 months on assigned treatment. The prespecified primary measure of efficacy was the change in total functional capacity (TFC) between baseline and 30 months. Safety measures included the frequency of clinical adverse events.RESULTS: Neither intervention significantly altered the decline in TFC. Patients treated with coenzyme Q10 showed a trend toward slowing in TFC decline (13%) over 30 months (2.40- versus 2.74-point decline, p = 0.15), as well as beneficial trends in some secondary measures. There was increased frequency of nausea, vomiting, and dizziness with remacemide and increased frequency of stomach upset with coenzyme Q10.CONCLUSIONS: Neither remacemide nor coenzyme Q10, at the dosages studied, produced significant slowing in functional decline in early HD.
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- Almqvist, Elisabeth W., 1958-
(författare)
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Dosage effects of riluzole in Huntington's disease : a multicenter placebo-controlled study.
- 2003
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Ingår i: Neurology. - : Ovid Technologies (Wolters Kluwer Health). - 0028-3878 .- 1526-632X. ; 61:11, s. 1551-6
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Tidskriftsartikel (refereegranskat)abstract
- BACKGROUND: Riluzole retards striatal glutamate release and pathologic consequences in neurotoxic animal models of Huntington's disease (HD).OBJECTIVE: To determine the dosage-related impact of riluzole on chorea in HD.METHODS: An 8-week double-blind dose-ranging multicenter study of riluzole was conducted in 63 subjects (32 women, 31 men) with HD who were randomized to receive placebo, riluzole 100 mg/day, or riluzole 200 mg/day. The prespecified outcome measure was change in the total maximal chorea score of the Unified Huntington's Disease Rating Scale (UHDRS).RESULTS: Fifty-six (89%) subjects completed the study. A reduction (p < 0.01) in chorea at 8 weeks was found using a linear trend test with dose. Comparing the groups individually, the reduction in chorea for the riluzole 200-mg/day group (-2.2 +/- 3.3) was different (p = 0.01) from placebo (+0.7 +/- 3.4), but the riluzole 100-mg/day group (-0.2 +/- 2.9) was not. Riluzole did not improve other motor, cognitive, behavioral, or functional components of the UHDRS. Alanine aminotransferase was elevated in a dosage-dependent fashion (p = 0.01).CONCLUSIONS: Over 8 weeks of treatment, riluzole 200 mg/day ameliorated chorea intensity in HD without improving functional capacity or other clinical features of illness. Riluzole 200 mg/day was attended by reversible liver transaminase abnormalities that would require monitoring in long-term studies.
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- Almqvist, E W, et al.
(författare)
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High incidence rate and absent family histories in one quarter of patients newly diagnosed with Huntington disease in British Columbia.
- 2001
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Ingår i: Clinical Genetics. - 0009-9163 .- 1399-0004. ; 60:3, s. 198-205
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Tidskriftsartikel (refereegranskat)abstract
- The advent of the direct mutation test for Huntington disease (HD) has made it possible to identify a previously unrecognized symptomatic population of HD, including those with an atypical presentation or patients without a family history of HD. The present study investigated the uptake of this test in the province of British Columbia (BC), Canada and assessed the incidence rate and rate of identification of new mutations for HD. All symptomatic individuals residing in BC who were referred for the genetic test for HD between 1993 and 2000 (n=205) were analyzed for CAG expansion, baseline demographics and clinical data, and a family history of HD. A total of 141 (or 68.8%) had a CAG expansion > or =36. Of these, almost one-quarter (24.1%) did not have a family history of HD. An extensive chart review revealed that 11 patients (or 7.8%) had reliable information on both parents (who lived well into old age) and therefore possibly could represent new mutations for HD. This indicates a three to four times higher new mutation rate than previously reported. Our findings also show that the yearly incidence rate for HD was 6.9 per million, which is two times higher than previous incidence studies performed prior to the identification of the HD mutation. We also identified five persons with a clinical presentation of HD but without CAG expansion (genocopies) (2.4%).
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- Almqvist, E W, et al.
(författare)
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Psychological consequences and predictors of adverse events in the first 5 years after predictive testing for Huntington's disease.
- 2003
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Ingår i: Clinical Genetics. - : Wiley. - 0009-9163 .- 1399-0004. ; 64:4, s. 300-9
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Tidskriftsartikel (refereegranskat)abstract
- The promise of genetic medicine is to provide information, based on genotype, to persons not yet sick about their risk of future illness. However, little is known of the long-term psychological effects for asymptomatic persons learning their risk of having a serious disease. Predictive genetic testing for Huntington's disease (HD) has been offered for the longest time for any disease. In the present study, the psychological consequences of predictive testing were assessed prospectively in individuals at risk for HD during seven visits over 5 years. Questionnaires of standard measures of psychological distress (the General Severity Index of the Symptom Check List-90-Revised), depression (the Beck Depression Inventory), and general well-being (the General Well-Being Scale) were administered to the participants. A significant reduction in psychological distress was observed for both result groups throughout 2 years (p < 0.001) and at 5 years (p = 0.002). Despite the overall improvement of the psychological well-being, 6.9% (14 of 202) of the participants experienced an adverse event during the first 2 years after predictive testing that was clinically significant. The frequency of all defined adverse events in the participants was 21.8%, with higher frequency in the increased risk group (p = 0.03) and most occurring within 12 months of receiving results.
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- Andershed, Birgitta, et al.
(författare)
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Being a close relative of a dying person : development of the concepts "involvement in the light and in the dark"
- 2000
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Ingår i: Cancer Nursing. - : Ovid Technologies (Wolters Kluwer Health). - 0162-220X .- 1538-9804. ; 23:2, s. 151-159
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Tidskriftsartikel (refereegranskat)abstract
- The current study is based on an earlier article in which relatives' involvement in care was described as involvement in the light or involvement in the dark. Involvement in the light was characterized as the relative being well informed and experiencing a meaningful involvement. The relatives involved in the dark felt uninformed, that they were groping around in the dark when they tried to support the patient. The present study analyzed further the meaning of involvement in the light and involvement in the dark, and investigated whether two different care cultures, the relationship with the staff, and a rapid course of illness influence the involvement of relatives. Relatives of 52 patients who died, 30 at a surgical department and 22 in a hospice ward, were interviewed after the patients' deaths. All the relatives of the patients in the hospice ward and 13 of those in the surgical department were judged to be involved in the light. Of the relatives judged to be involved in the dark, 12 either had a sick relative with a rapid course of illness or felt that the sick relative had died unexpectedly. A pattern was clearly observed: The relatives involved in the light described being met with respect, openness, sincerity, confirmation, and connection, whereas the opposite was experienced by those involved in the dark.
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- Andershed, Birgitta
(författare)
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Närståendes behov i palliativ vård
- 2004
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Ingår i: Närståendes behov. - Stockholm : Svensk sjuksköterskeförening. - 9185060097 ; , s. 103-115
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Bokkapitel (populärvet., debatt m.m.)
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