Search

Our purpose of this study was to investigate whether clinical rapid eye movement sleep behavior disorder (RBD) is indicative of more widespread degenerative changes in Parkinson’s disease (PD), using a longitudinal cohort. In 2005 and 2007, we prospectively collected clinical and treatment characteristics of 61 consecutive patients with PD. The presence of RBD was assessed by spouse interview. Sixty-four percent of patients had clinical RBD in 2005, versus 52% of patients in 2007. The yearly incidence rate of clinical RBD onset was 9%, while clinical RBD disappeared in 14% of patients per year. The motor disability scores (when treated) were worse in patients with than without clinical RBD in 2005, but not in 2007. There was no difference between groups for frequency of freezing, falls, and hallucinations, or for scores on the depression scale, sleepiness scale, mini-mental state examination and frontal assessment battery at the beginning versus the end of the study. Patients with clinical RBD were disabled earlier than patients without RBD, but there was no specific worsening in the RBD group with time, either for motor or non-motor symptoms. The fluctuation and disappearance of clinical RBD in some patients may be due to functional abnormalities rather than lesions. [ABSTRACT FROM AUTHOR]

The article presents a study to determine the surgical morbidity and mortality rates from 2002-2004. One thousand one hundred eighty-six 18-65-year-old patients undergoing colorectal surgery were examined through their Physiological and Operative Severity Score for the enUmeration of Mortality and morbidity (POSSUM), Surgical Risk Scale (SRS), and Association Française de Chirurgie (AFC) records. The study shows that morbidity and mortality rates are 41% and nine percent, respectively.

Objective: To assess the long-term renal tolerance of a low-dose cyclosporine regimen in uveitis patients with previously healthy kidneys on clinical, biological, and pathological criteria. Methodology: Forty-one patients treated with 4.3 ± 1.6 mg/kg body weight per day cyclosporine for 44.9 ± 3.6 months were included. Mean follow-up was 55.4 ± 0.2 months. Blood pressure, cyclosporine trough level, and renal function were prospectively monitored together with blood urea, creatinine clearance, glomerular filtration rate (GFR), and effective renal plasma flow. Eleven patients underwent serial kidney biopsies before and after 2 years of a 4 ± 0.9 mg/kg daily cyclosporine treatment. Results: Cyclosporine treatment induced a significant increase in plasma creatirtlne (p < .0601), significant decrease in creatinine clearance (p < .0001). and isotopic GFR (p < .0001) over time. The highest dose induced more severe alterations in the renal parameters than did the lowest dose. Prevalence of hypertension was particularly high. Histopathological data showed significant interstitial fibrosis (p < .003) and tubular atrophy (p < .003) after 2 years. Conclusion: Low.lose long-term cyclosporine treatment induces significant renal impairment and a high incidence of hypertension. [ABSTRACT FROM AUTHOR]

Objective: To compare six online evaluation methods for auditing routine second-trimester four-chamber view still images., Methods: We evaluated three different scoring grids (subjective, five-item score and seven-item score), which were applied with or without access to online help, resulting in a total of six evaluation methods. For the subjective scoring grid, images were rated as excellent, good, fair, poor or very poor. For the five-item score, 1 point was allocated for visualization (vs non-visualization or non-evaluable) of each of: heart crux, atria, ventricles, apex and aorta, yielding a score of 0-5. For the seven-item score, 1 point was allocated for clear (vs unclear) visualization of each of: moderator band at the apex, interventricular septum, atrioventricular valves, non-linear insertion of atrioventricular valves (normal offset), septum primum, aorta and pulmonary vein. Each evaluation method was used via the Internet by three randomly selected reviewers, who evaluated the same set of 80 images. Reviewers were experienced in fetal ultrasound, but were not involved in the design of the study. Interrater agreement was the main outcome., Results: The five-item scoring grid with online help achieved the best interrater agreement (interrater intraclass correlation coefficient = 0.7)., Conclusions: Evaluation of the second-trimester sonographic four-chamber view is apparently best achieved with a simple five-item scoring grid., (Copyright © 2011 ISUOG. Published by John Wiley & Sons, Ltd.)

Background: Several scores have been developed to evaluate surgical unit mortality and morbidity. The Physiological and Operative Severity Score for the enUmeration of Mortality and morbidity (POSSUM) and derivatives use preoperative and intraoperative factors, whereas the Surgical Risk Scale (SRS) and Association Française de Chirurgie (AFC) score use four simple factors. To allow for advanced age in patients undergoing colorectal surgery, a dedicated score-the Elderly (E) POSSUM-has been developed and its accuracy compared with these scores., Methods: From 2002 to 2004, 1186 elderly patients, at least 65 years old, undergoing major colorectal surgery in France were enrolled. Accuracy was assessed by calculating the area under the receiver operating characteristic curve (AUC) (discrimination) and calibration., Results: The mortality and morbidity rates were 9 and 41 per cent respectively. The E-POSSUM had both a good discrimination (AUC = 0.86) and good calibration (P = 0.178) in predicting mortality and a reasonable discrimination (AUC = 0.77) and good calibration (P = 0.166) in predicting morbidity. The E-POSSUM was significantly better at predicting mortality and morbidity than the AFC score (P(c) = 0.014 and P(c) < 0.001 respectively)., Conclusion: The E-POSSUM is a good tool for predicting mortality, and the only efficient scoring system for predicting morbidity after major colorectal surgery in the elderly., ((c) 2010 British Journal of Surgery Society Ltd. Published by John Wiley & Sons, Ltd.)

Objective: To determine the longitudinal metric properties of recently developed clinical assessment tools in spinocerebellar ataxia (SCA)., Methods: A subset of 171 patients from the EUROSCA natural history study cohort (43 SCA1, 61 SCA2, 37 SCA3, and 30 SCA6) were examined after 1 year of follow-up. Score changes and effect size indices were calculated for clinical scales (Scale for the Assessment and Rating of Ataxia [SARA], Inventory of Non-Ataxia Symptoms [INAS]), functional tests (SCA Functional Index [SCAFI] and components), and a patient-based scale for subjective health status (EQ-5D visual analogue scale [EQVAS]). Responsiveness was determined in relation to the patient's global impression (PGI) of change and reproducibility described as retest reliability for the stable groups and smallest detectable change., Results: Within the 1-year follow-up period, SARA, INAS, and SCAFI but not EQVAS indicated worsening in the whole group and in the groups with subjective (PGI) worsening. SCAFI and its 9-hole pegboard (9HPT) component also deteriorated in the stable groups. Standardized response means were highest for 9HPT (-0.67), SARA (0.50), and SCAFI (-0.48) with accordingly lower sample size estimates of 143, 250, or 275 per group for a 2-arm interventional trial that aims to reduce disease progression by 50%. SARA and EQVAS performed best to distinguish groups classified as worse by PGI. All scales except EQVAS reached the criterion for retest reliability., Conclusion: While both the Scale for the Assessment and Rating of Ataxia and the SCA Functional Index (SCAFI) (and its 9-hole pegboard component) had favorable measurement precision, the clinical relevance of SCAFI and 9-hole pegboard score changes warrants further exploration. The EQ-5D visual analogue scale proved insufficient for longitudinal assessment, but validly reflected patients' impression of change.

Objective: To establish phenotype-genotype correlations in early-onset Parkinson disease (EOPD), we performed neurologic, neuropsychological, and psychiatric evaluations in a series of patients with and without parkin mutations., Background: Parkin (PARK2) gene mutations are the major cause of autosomal recessive parkinsonism. The usual clinical features are early-onset typical PD with a slow clinical course, an excellent response to low doses of levodopa, frequent treatment-induced dyskinesias, and the absence of dementia., Methods: A total of 44 patients with EOPD (21 with and 23 without parkin mutations) and 9 unaffected single heterozygous carriers of parkin mutations underwent extensive clinical, neuropsychological, and psychiatric examinations., Results: The neurologic, neuropsychological, and psychiatric features were similar in all patients, except for significantly lower daily doses of dopaminergic treatment and greater delay in the development of levodopa-related fluctuations (p < 0.05) in parkin mutation carriers compared to noncarriers. There was no major difference between the two groups in terms of general cognitive efficiency. Psychiatric manifestations (depression) were more frequent in patients than in healthy single heterozygous parkin carriers but did not differ between the two groups of patients., Conclusion: Carriers of parkin mutations are clinically indistinguishable from other patients with young-onset Parkinson disease (PD) on an individual basis. Severe generalized loss of dopaminergic neurons in the substantia nigra pars compacta in these patients is associated with an excellent response to low doses of dopa-equivalent and delayed fluctuations, but cognitive impairment and special behavioral or psychiatric symptoms were not more severe than in other patients with early-onset PD.

Objective: To identify factors that determine disease severity and clinical phenotype of the most common spinocerebellar ataxias (SCAs), we studied 526 patients with SCA1, SCA2, SCA3. or SCA6., Methods: To measure the severity of ataxia we used the Scale for the Assessment and Rating of Ataxia (SARA). In addition, nonataxia symptoms were assessed with the Inventory of Non-Ataxia Symptoms (INAS). The INAS count denotes the number of nonataxia symptoms in each patient., Results: An analysis of covariance with SARA score as dependent variable and repeat lengths of the expanded and normal allele, age at onset, and disease duration as independent variables led to multivariate models that explained 60.4% of the SARA score variance in SCA1, 45.4% in SCA2, 46.8% in SCA3, and 33.7% in SCA6. In SCA1, SCA2, and SCA3, SARA was mainly determined by repeat length of the expanded allele, age at onset, and disease duration. The only factors determining the SARA score in SCA6 were age at onset and disease duration. The INAS count was 5.0 +/- 2.3 in SCA1, 4.6 +/- 2.2 in SCA2, 5.2 +/- 2.5 in SCA3, and 2.0 +/- 1.7 in SCA6. In SCA1, SCA2, and SCA3, SARA score and disease duration were the strongest predictors of the INAS count. In SCA6, only age at onset and disease duration had an effect on the INAS count., Conclusions: Our study suggests that spinocerebellar ataxia (SCA) 1, SCA2, and SCA3 share a number of common biologic properties, whereas SCA6 is distinct in that its phenotype is more determined by age than by disease-related factors.

Background: Stimulation of the subthalamic nucleus is an effective treatment for advanced Parkinson disease (PD) and is currently performed after a mean disease duration of 14 years, when severe motor complications have resulted in marked loss of quality of life. We examined whether surgery at an early stage would maintain quality of life as well as improve motor function., Methods: Twenty patients with PD of short duration (time elapsed since first symptom +/- SD: 6.8 +/- 1.0 years) with mild to moderate motor signs (Unified Parkinson's Disease Rating Scale III "off" medication: 29 +/- 12) who responded well to levodopa treatment were included in pairs, matched for age, duration and severity of disease, and impairment in socioprofessional functioning. Patients were prospectively randomized to undergo bilateral subthalamic nucleus stimulation (n = 10) or receive optimized medical treatment (n = 10). Parkinsonian motor scores, quality of life, cognition, and psychiatric morbidity were assessed at inclusion and at 6, 12, and 18 months after randomization., Results: Quality of life was improved by 24% in surgical and 0% in nonsurgical patients (p < 0.05). After 18 months, the severity of parkinsonian motor signs "off" medication, levodopa-induced motor complications, and daily levodopa dose were reduced by 69%, 83%, and 57% in operated patients and increased by 29%, 15%, and 12% in the group with medical treatment only (p < 0.001). Adverse events were mild or transient, and overall psychiatric morbidity and anxiety improved in the surgical group., Conclusions: Subthalamic nucleus stimulation should be considered a therapeutic option early in the course of Parkinson disease.

Objective: To develop a reliable and valid clinical scale measuring the severity of ataxia., Methods: The authors devised the Scale for the Assessment and Rating of Ataxia (SARA) and tested it in two trials of 167 and 119 patients with spinocerebellar ataxia., Results: The mean time to administer SARA in patients was 14.2 +/- 7.5 minutes (range 5 to 40). Interrater reliability was high, with an intraclass coefficient (ICC) of 0.98. Test-retest reliability was high with an ICC of 0.90. Internal consistency was high as indicated by Cronbach's alpha of 0.94. Factorial analysis revealed that the rating results were determined by a single factor. SARA ratings showed a linear relation to global assessments using a visual analogue scale, suggesting linearity of the scale (p < 0.0001, r(2) = 0.98). SARA score increased with the disease stage (p < 0.001) and was closely correlated with the Barthel Index (r = -0.80, p < 0.001) and part IV (functional assessment) of the Unified Huntington's Disease Rating Scale (UHDRS-IV) (r = -0.89, p < 0.0001), whereas it had only a weak correlation with disease duration (r = 0.34, p < 0.0002)., Conclusions: The Scale for the Assessment and Rating of Ataxia is a reliable and valid measure of ataxia, making it an appropriate primary outcome measure for clinical trials.

Background: High-frequency stimulation of the subthalamic nucleus (STN) is an effective treatment for advanced Parkinson disease (PD). The clinical and preoperative predictive factors of the best postoperative outcome have been identified. Radiologic predictive factors were investigated., Methods: Forty patients with PD underwent surgery for bilateral STN stimulation. MRI was performed in stereotactic conditions before surgery. Brain parenchyma, caudate nucleus, putamen, pallidum, and red nucleus volumes and the surface of the mesencephalon were measured and normalized as percentages of the intracranial volume. Clinical evaluation was performed 1 month before and 6 months after surgery., Results: The normalized brain parenchyma volume was lower in patients who were older and had a longer disease duration or a lower frontal score and was not predictive of the postoperative outcome. The residual scores for activities of daily living and parkinsonian motor disability were higher in patients with a smaller normalized mesencephalon. The normalized caudate nucleus volume was predictive of the pre- and postoperative levodopa-equivalent dosage., Conclusions: Brain atrophy is not an exclusion criterion for neurosurgery, indicating that patients' neurologic, psychiatric, and neuropsychological characteristics are the best predictive factors for neurosurgery. The fact that a smaller normalized mesencephalon surface was associated with a lower beneficial effect of the subthalamic nucleus stimulation on the parkinsonian motor disability suggests that the normalized mesencephalon surface is a predictive factor of the postoperative outcome.