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Determination of a scale's factor structure requires a two-part process: (1) an initial examination of the factor structure using a sample of individuals with the condition of interest, and (2) repeated examinations of the factor structure using the same analytic methods but applied to independent samples of individuals with the condition of interest who contribute unique variability to the scale measurement. In a previous study, we performed an initial investigation of the factor structure of the Motor Examination section of the Unified Parkinson's Disease Rating Scale (UPDRS). We used a sample of 294 consecutive patients with idiopathic Parkinson's disease (PD) assessed while in the on-state and identified six clinically distinct factors. In the present study, we performed a confirmatory investigation of the factor structure and analysis of the internal consistency of the UPDRS in a new sample of 200 consecutive PD patients who were assessed while in the off-state. Factor analysis again revealed six factors with identical item loadings as those obtained from examinations of patients in the on-state. Estimates of internal consistency were comparable in the off- and on-state examinations. These results indicate that the Motor Examination section of the UPDRS has a stable factor structure and high internal consistency across off- and on-state examinations.

Objective: to determine possible risk factors of a stroke after percutaneous transluminal coronary angioplasty (PTCA). Design: this is a retrospective evaluation of a consecutive group of 4088 patients undergoing PTCA between 1988 and 1995. We have studied the incidence, clinical characteristics, risk factors, and outcome. Results: seven patients, six males and one female (0.17%) developed a stroke after this procedure. In comparison with the control group, the stroke group did not differ regarding age or gender. The existence of a stroke (six located in the brain, and one in the spinal cord), represented 1.24% of all complications (P < 0.001), and 5% of all deaths (P < 0.01) of PTCA. Three patients developed TIA, two patients developed hemorrhagic strokes (in which they received previous thrombolytic therapy), and the other two patients suffered from an ischemic stroke. The statistically significant risk factors of a stroke after PTCA included: intracoronary thrombolytic therapy (P < 0.01), hypercholesterolemia (P < 0.001) and a prior PTCA (P < 0.05). Conclusions: although these procedural complications are infrequent, they are usually serious and important risk factors which could be identified prior to the procedure. These risk factors would allow identification of patients who are prone to a stroke after PTCA. Intracranial hemorrhage occurred only after thrombolytic therapy, and the factors related to hemorrhagic strokes were probably different from those predisposed to ischemic strokes and TIA. Copyright 1998 Lippincott Williams & Wilkins

Travel distance, growing disability, and uneven distribution of doctors limit access to care for most Parkinson's disease (PD) patients worldwide. Telemedicine, the use of telecommunications technology to deliver care at a distance, can help overcome these barriers. In this report, we describe the past, present, and likely future applications of telemedicine to PD. Historically, telemedicine has relied on expensive equipment to connect single patients to a specialist in pilot programs in wealthy nations. As the cost of video conferencing has plummeted, these efforts have expanded in scale and scope, now reaching larger parts of the world and extending the focus from care to training of remote providers. Policy, especially limited reimbursement, currently hinders the growth and adoption of these new care models. As these policies change and technology advances and spreads, the following will likely develop: integrated care networks that connect patients to a wide range of providers; education programs that support patients and health care providers; and new research applications that include remote monitoring and remote visits. Together, these developments will enable more individuals with PD to connect to care, increase access to expertise for patients and providers, and allow more-extensive, less-expensive participation in research.

There are limited data in terms of the clinical profile of Parkinson's disease in sub-Saharan African patients.To compare the clinical profile and access to standard antiparkinsonian therapies of a Cameroonian cohort of patients with an age, sex, and disease duration-matched Spanish cohort (Longitudinal Study of Parkinson's disease, ELEP).Observational, cross-sectional design. Demographic data were collected and the following ELEP assessments were applied: Scales for Outcomes in Parkinson's disease (SCOPA) Motor, Autonomic, Cognition, Sleep and Psychosocial; Hoehn and Yahr staging; modified Parkinson Psychosis Rating Scale; Cumulative Illness Rating Scale-Geriatrics; Hospital Anxiety and Depression Scale; pain and fatigue visual analog scales; Zarit, and EuroQoL.74 patients with idiopathic Parkinson's disease were included (37 from each country) with a mean age of 64.4±10.5 years old, 70.3% males, and mean disease duration of 5.6±5.9 years. Compared to the Spanish cohort, Cameroonians were intermittently treated, less frequently received dopaminergic agonists (p0.001), had a trend for taking lower doses of levodopa (p=0.06), and were more frequently on anticholinergics (p0.0005). Cameroonians were more severely impaired in terms of motor (Hoehn Yahr stage, p=0.03; SCOPA-Motor, p0.001), cognitive status (p0.001), anxiety and depression (p0.001), psychosis (p=0.008), somnolence, fatigue and pain (p0.001, respectively), caregiver burden (p0.0001), and quality of life (p=0.002). Instead, autonomic, comorbidity, and nocturnal sleep problems were similarly found.Limited and intermittent access to dopaminergic drugs has a negative impact on motor symptoms, nonmotor symptoms and quality of life in patients with Parkinson's disease and their caregivers.

Visual symptoms are common among the non-motor symptoms in Parkinson’s disease. The aims of this study were to assess the diagnostic accuracy and relationship of retinal morphologic and functional changes with motor and non-motor symptoms disturbances in Parkinson’s disease. Thirty patients with Parkinson’s disease, with a median Hoehn–Yahr stage of 2 (1–4), were compared to 30 age- and gender-matched controls. Retinal thinning and function were measured using optical coherence tomography (OCT), visual evoked potentials (VEP), and pattern electroretinography. Motor impairment and motor laterality were measured using the Short Parkinson’s Evaluation Scale/Scales for Outcomes in Parkinson’s disease, and non-motor symptoms severity using the non-motor symptoms questionnaire. Only pattern electroretinography, P50 and N95 amplitudes, were lower in patients with Parkinson’s disease, compared to controls (p = 0.01, respectively). Age, disease duration, levodopa dose, motor, and non-motor impairment were not significantly associated with retinal thinning and functional changes. The patients vs. controls area under the curve of OCT, VEP, and pattern electroretinography receiver-operating-characteristic curves were

To assess the residual disability in a sample of patients after suffering a first episode of a stroke and to compare the disability of those patients who live in rural areas with those living in urban areas.An observational, longitudinal study of a cohort of 89 patients from a Neurology Unit, affected by cerebrovascular accident. The following factors were assessed: sociodemographic and environmental factors, co-morbidity, functional status, disability, depression and anxiety, and quality of life. The different clinical and demographic variables were compared after admission to the unit, at hospital discharge, and 3 months afterwards. Regression analyses were also carried out in order to study the association between the clinical and sociodemographic factors, and post-stroke disability.Compared to their previous clinical state, after suffering a stroke patients showed a higher rate of co-morbidity (P.0001), disability (P.0001), depression (P=.002), and a poorer quality of life (P=.013). The difference between patients coming from rural and urban areas was not statistically significant in terms of disability, quality of life, anxiety, depression, or co-morbidity.The level of disability, depression and co-morbidity that patients showed after suffering a stroke was similar to the results obtained in other studies. As a novel feature, there were no differences between patients living in rural areas after suffering a stroke and those living in urban areas, as regards disability, depression, or co-morbidity.

Because migraine has been associated with dopaminergic receptor hypersensitivity, the authors hypothesized that patients with Parkinson disease with current or prior migraine have better dopaminergic response and less motor disability than Parkinson disease patients without migraine. Twenty-eight patients with Parkinson disease were included and matched (10 patients with migraine and 18 patients without migraine). Patients with Parkinson disease and migraine showed greater motor improvement during the ON state than patients without migraine with the same medication exposure. These data support the hypothesis that migraine may be associated with dopaminergic hypersensitivity.

Background Verbal fluency tasks have been identified as important indicators of executive functioning impairment in patients with Huntington’s disease (HD). The F.A.S. test has been widely used to assess phonological fluency in Anglo-Saxon people, because these three letters are the most frequent in English language. In the contrast, normative data have been developed for the letters P, M and R since they are more frequent in the Spanish population. Aims To compare the performance in phonological verbal fluency (F.A.S vs P.M.R) in a sample of HD participants and their correlation with the global cognitive status. Method Pilot, cross sectional study. The F.A.S. and P.M.R fluency tests were administered to a sample of patients with HD (2 pre-manifests, 4 manifests). Scores in terms of correct words for each letter, perseverations and intrusions were collected. The global cognitive status was assessed by the Mini-mental State Examination (maximum score 35). Data were analysed using non parametric tests. Results The F.A.S and P.M.R fluency testes were comparable (total score p = 0.24, intrusions score p = 0.31, perseveration score p = 0.08). However, there was a higher correlation of P.M.R with MMSE ( r s = 0.81, p = 0.05) compared to F.A.S. ( r s = 0.60, p = 0.20). Conclusions F.A.S and P.M.R provided an equivalent measure of phonological verbal fluency in our sample. However, P.M.R seems to be a better predictor of global cognitive status. Based on these preliminary results, the phonological verbal fluency should be analysed based on the patient’s mother tongue. Further studies including a higher sample size and group differences (manifest vs. pre-manifest) are needed to confirm these preliminary results.

Background Patients with HD are at risk of malnutrition due to increased energy consumption; chorea; alteration in chewing, swallowing, mobility and cognitive functions; but on the other hand they are usually treated with antipsychotic drugs implicated as a risk factor for the development of obesity and metabolic syndrome. Bioelectrical Impedance Assessment (BIA) is a good method for body compartments estimates, but on the other hand anthropometric methods could be cheaper. Aims To validate anthropometrics as a cheaper method compared to BIA to predict risk for malnutrition in HD in terms of body fat mass, fat free mass in patients with HD. Methods Multicentric cross-sectional study. Patients with genetically confirmed HD were included. Body composition was measured using the single-frequency (BIA) Tanita® BC418MA, and anthropometric methods (height, calf circumference, and waist circumference) were measured using a soft nonstretchable tape midway. Results Thirty HD patients with 53% women and a mean age of 57 ± 13 years, BMI 24.1 ± 4.2, waist 86.8 ± 14.3 cm, waist to height ratio 0.53 ± 0.08 were included. According to the WHO classification, 10% of patients were underweighted, 50% normal, 27% overweighted, and 13% obese. Fat free mass was highly correlated with calf circumference (rs = 0.78, p Conclusions Anthropometric measures such as calf circumference are highly correlated with fat free mass and can be used as a biomarker. In HD patients, low muscle mass is associated with deterioration of physical function. Further studies are needed to confirm our results. Acknowledgement We gratefully acknowledge to Gerencia Regional de Salud of Junta de Castilla y Leon for financial support to this work.

Understanding the impact of placebo treatment is pivotal to the correct interpretation of clinical trials. The aim of present study was to examine the placebo effect in tic disorders.Raw data were obtained for 6 placebo-controlled parallel and cross-over trials that involved medical interventions for tic disorders. Tic severity was measured using the Yale Global Tic Severity Scale. Placebo effect was defined as an improvement of at least 30% over baseline scores in the total tic score and was considered clinically relevant when at least 10% of patients in the placebo-arm met that benchmark.In total, 91 placebo-treated patients (80% males; mean age, 16.5 years; standard deviation, 10.5 years) were included. Although there was a trend toward improvement in the total tic scores after placebo administration (P=0.057), the magnitude of the placebo effect was small (Cohen's d=0.16) but relevant (19% of the sample). Females were more likely than males to have a placebo effect.The magnitude of the placebo effect in tic disorders appeared to be small. Further longitudinal studies using objective assessments for tic disorders are warranted to confirm the current results. © 2013 Movement Disorder Society.

Background: The feasibility and validity of Web-based assessments in Parkinson's disease is unknown. The objectives of this study were to develop and to compare home Web-based assessments with office-based assessments. Methods: We tested feasibility and validity using a longitudinal, randomized crossover design. Patients were assessed at baseline and after 6 and 12 weeks using both assessments including the Unified Parkinson's Disease Rating Scale, the Unified Dyskinesia Rating scale, timed tests, and quality-of life and Non-Motor Symptoms questionnaires. Results: Forty-two patients were included (22 men, 20 women; mean age, 64.7 ± 9.0 years). Only 2 patients (5%) dropped out. The mean intraclass correlation coefficient between Web- and office-based assessments ranged from 0.67 (first visit) to 0.75 (last visit) and 0.81 and 0.82 for doctor- and patient-administered scales, respectively. No differences in responsiveness (P = 0.63), and data precision (P = 0.11) were found, but Web-based assessments had fewer missing values (P = 0.01). Conclusions: Web-based assessments offer a feasible format for assessing PD-related impairment from home. © 2011 Movement Disorder Society

The impact of apathy on health-related quality of life (HRQOL) in recently diagnosed Parkinson's disease (PD) has not been systematically investigated. The objective of this cross-sectional survey (ANIMO study) was to examine the contribution of apathy to HRQOL in a Spanish sample of recently diagnosed PD patients. PD patients, diagnosed within 2 years of inclusion, were recruited at 102 outpatient clinics in 82 communities throughout Spain. Apathy was quantified using the Lille Apathy Rating Scale and HRQOL with the EuroQol-5D questionnaire. A mean EuroQol-5D index score of 0.89 obtained from population references in Spain was used as the cutoff for this study. The relationship between apathy and the dichotomized EuroQol-5D index score (0.89 [lower HRQOL] vs ≥0.89 [reference]) was examined using multiple logistic regression analysis, adjusting for sociodemographic and clinical variables. We consecutively recruited 557 patients (60.3% men) with a mean age of 68.8 ± 9.7 years. Apathy was diagnosed in 291 (52.2%) and was related to problems in each of the EuroQoL dimensions. Apathetic PD patients showed EuroQol-5D index scores significantly lower than those without apathy (0.64 vs 0.83). In an adjusted model, apathetic PD patients were 2.49 times more likely to have lower HRQOL than nonapathetic patients (odds ratio, 2.49; 95% confidence interval, 1.49-4.15, P0.01). Apathy is very common in those with recently diagnosed PD and is one of the major clinical determinants of HRQOL in this disease. It should be one of the primary concerns among clinicians who provide treatment to individuals affected by PD.

Tic disorders constitute a neurodevelopmental disorder of childhood. This study sought to determine the prevalence of tic disorders in a school-based sample. A randomized sample of 1158 schoolchildren, based on clusters (classrooms) in the province of Burgos (Spain), was identified on a stratified sampling frame combining types of educational center and setting (mainstream schools and special education), using a two-phase approach (screening and diagnosis ascertainment by a neurologist). Tics with/without impairment criterion were diagnosed according to Diagnostic and Statistical Manual of Mental Disorders criteria. In mainstream schools, tics were observed in 125/741 students (16.86%; 95% confidence interval, 14.10-19.63), and were more frequent in boys (87/448, 19.42%; 95% confidence interval, 15.64-23.19) compared with girls (38/293, 12.96%; 95% confidence interval, 8.95-16.98; P = 0.03). In special education centers, tics disorders were observed in 11/54 of children (20.37%; 95% confidence interval, 8.70-32.03). Overall, tics with impairment criteria were less frequent than tics without impairment criteria (4.65% vs 11.85%, P < 0.0001). The most frequent diagnoses involved chronic motor tics (6.07%) and Tourette syndrome (5.26%). Tic disorders are common in childhood, and the use or nonuse of impairment criteria exerts a significant impact on tic prevalence estimates.

Background Little is known about the energy needs in Huntington’s disease (HD). Aims To analyse and compare the total energy expenditure (TEE) and energy balance (EB) in a representative sample of HD patients included in the European Huntington’s Disease Registry with healthy controls, homogeneous in age, gender and body mass index. Methods Observational, case-control single centre study. To estimate the EB, food caloric-energy intake (EI) and TEE were considered. To assess EI, a 24-hour dietary recall questionnaire was collected. TEE was computed as the sum of resting energy expenditure (REE) measured by indirect calorimetry, physical activity (PA) monitored by an actigraph, and dietary induced thermogenesis. The results were adjusted by fat free mass measured by bioelectrical impedance. Results 22 patients (36% males, mean age 50.2 ± 15.6 years, motor UHDRS 27.9 ± 23.7, TFC 11.0 (13.0–7.0), cognitive UHDRS 167.0 (242.5–100.2), CAG 43.0 ± 6.0, EI 38.5 ± 9.9 kcal/kg, PA 5.3 (7.4–3.0) kcal/kg, REE 30.8 ± 6.3 kcal/kg, TEE 40.5 ± 7.9 kcal/kg), and 18 controls (50% males, mean age 47.4 ± 13.7, EI 38.6 ± 10.3 kcal/kg, PA 8.4 (13.7–4.9) kcal/kg, REE 30.7 ± 6.6 kcal/kg, TEE 44.0 ± 7.8 kcal/kg) were included. EB was similar in both groups (patients -2.0 ± 11.7 kcal/kg; controls -5.3 ± 11.2 kcal/kg; p = 0.36). PA was lower in patients compared to controls (p = 0.01). Conclusions Although patients with HD appear to have lower energy expenditure mainly due to decreased voluntary physical activity, they are still able to maintain their energy needs with an adequate food intake.

Background Dysphagia is a common complication of neurogenerative disorders, and a leading cause of death due to aspiration pneumonia. Little is known about the frequency and the characteristics of dysphagia in HD. Well-balanced strategies for treatment and prevention of dysphagia in HD are lacking. Objetives To determine the prevalence of dysphagia in HD. Methods National, multicenter, observational, cross-sectional Study (Spanish EHDN). Dysphagia was assessed using the questionnaire EAT-10 (eating assessment tool), caregiver burden using the SumaCare, functional capacity using the TFC score, disease severity using the Unified HD Rating Scale (UHDRS), psyquiatric status (PBA-s), nutritional status using the Body Mass Index (BMI), and quality of life using the SF36. Results 224 patients were included, 59.2% women, mean age 48.9 + 14.8 years, median TFC score of 9 (IQR 10), median UHDRS motor score of 35 (IQR 52), median cognitive scores 150 (IQR 193), and mean BMI 24.7 + 5.4. One hundred and ninety seven patients (88%) completed the EAT-10. Dysphagia was found in 37% of the simple, most of them had a caregiver, and it was equally frequent in men compared to women. Patients with dysphagia were older (p = 0.02), had higher UHDRS motor scores (p Conclusions Dysphagia is very frequent in HD and associated with more severe disease, lower functional capacity and caregiver burden. Further strategies are warranted to identify dysphagia and develop nursing interventions including mealtime tips, environment accommodation, and food choices to prevent aspiration pneumonia in HD clinics. Funded by EHDN, Nutricia and Fresenius Kabi.

Background The impact of a diet is well known in human health. Intake of food groups (fruits and ‘uts, vegetables, legumes, olive oil, whole grains, fish and wine) in the traditional Mediterranean Diet (MeDi) and high MeDi adherence have been associated with lower incidence of chronic diseases and slower cognitive decline, but the relationship between MeDi adherence and nutritional composition has not been reported. Objectives To describe MeDi and nutritional composition in patients with HD. Methods Spanish multicenter, cross-sectional study (EHDN). To assess MeDi we used a 3-days dietary record. Food groups, macro and micronutrients and energy intake information were obtained using the software AyS, version 2.0. MeDi scores ranged from 0–9, and higher scores indicated higher adherence. Results 98 patients were included, 62% women, age 49 ± 14 years, medians TFC 9.0 (7–12), motor UHDRS 32 (7–45) and cognitive UHDRS 161 (132–203), and mean CAG 44 ± 6, BMI 24.6 ± 4.6, waist circumference (WC) 87.7 ± 14.6, waist-height ratio (WHR) 0.54 ± 0.45. Low MeDi adherence was found in 56% of the sample most of them in the age group of 18–50 years. Percentage calories from: protein 18 ± 4, lipid 36 ± 7, and carbohydrates 46 ± 8. MeDi adherence was similar items of age, sex, BMI, WC, WHR, educational level, type of caregiver, TFC and physical activity. Compared to lower MeDi adherence, HD patient with moderate/high adherence had higher: SF36 score (p = 0.009), AGP+AGM)/AGS ratio (p Conclusions Patients with HD show low MeDi adherence. In this study we found that higher MeDi adherence was associated with higher quality of life and better nutritional composition. Longitudinal studies and warranted to assess if higher MeDi adherence may delay HD progression and comorbidities. We gratefully acknowledge to European Huntington’s Disease Network for financial support to this work.

The main purpose was to validate a multistage protocol to screen tic disorders in mainstream and special education centers and to investigate whether telephone-based interviews were accurate to diagnose tic disorders when compared with in-person neurological interviews.A school-based survey of children and adolescents (6-16 years). A proxy report questionnaire for tic disorders was completed by teachers, observers, and parents. The diagnosis was confirmed by the neurologist based on the Diagnostic and Statistical Manual of Mental disorders (DSM-IV TR) criteria. The sensitivity, specificity, positive and negative predictive values, the likelihood ratio for a positive result (LR+), and the Kappa coefficients (k) for telephone-based interviews reliability were calculated.One hundred twenty subjects, 57 from special education center and 63 from a mainstream school were studied. The sensitivity of the proxy report questionnaire was 58% and 36% when completed by the observers (mainstream and special education center, respectively), 40 and 73% when completed by the teachers, and 58 and 36% when completed by the parents. Using any of these 3 screening sources, the sensitivity was 92%, the positive predictive value was 38%, and negative predictive value was 97% (mainstream schools), whereas the sensitivity was 82%, the positive predictive value was 20%, and negative predictive value was 82% (special education centers). Parents (mainstream schools) and teachers (special education center) produced the highest LR+ (7.25 and 1.26, respectively) and the intraobserver reliability of the telephone-based interviews versus in-person neurological interviews gave a k coefficient of 0.83.The efficiency of different screening instruments for tic disorders seems to vary in different settings. Telephone-based interviews may be a valid and convenient backup to ascertain the diagnosis of tic disorders when face-to-face neurological examination is not possible.

To determine the most significant clinical predictors that influence driving ability in Parkinson disease (PD).National-multi-centre, cross-sectional study covering PD outpatients. Clinical assessment was based on the following questionnaires: cognition (SCOPA-Cog); motor impairment and disabilities (SCOPA motor); depression/anxiety; sleep (SCOPA-Sleep); psychosis and severity/global impairment (HY and CISI-PD). Driving status data was obtained using a standardized questionnaire. Comparisons between drivers and ex-drivers were calculated using chi(2) and Student t-tests as appropriate. Multi-variate logistic regression analysis was performed to identify independent driving ability clinical predictors.Compared with the drivers, the ex-drivers were older (p = 0.00005), had longer disease duration (p = 0.03), had more overall cognitive dysfunction (p = 0.004) and had greater motor impairment, as measured by the CISI (p = 0.02), HY stage (p = 0.034) and by the SCOPA-motor scale (p = 0.002) and difficulty in activities of daily life (p = 0.002). In the regression model analysis, aging and ADL impairment were the principal clinical predictors that differentiated drivers from ex-drivers.Although overall driving impairment in PD is associated with advancing disease severity, driving ability seems to be more strongly influenced by age and ADL impairment. Multi-disciplinary teams are required to assess driving ability in patients with PD and develop rehabilitation measures for safer driving.

Two small studies reported suboptimal therapy adherence in Parkinson's disease. We conducted a larger multicenter European study to assess medicine-taking behavior. Parkinson's disease patients taking dopaminergic therapy were enrolled in 8 centers in 5 countries, and disease severity and demographics recorded. Antiparkinson drug adherence was measured for 4 weeks using electronic monitoring bottles which record the date and time of cap opening (Aardex, Switzerland). One hundred twelve patients, mean age 65 years (standard deviation (SD) 10), with Parkinson's disease for 7.7 (SD 8.2) years completed the study. Total median adherence (doses taken/doses prescribed) was 97.7% (interquartile range [IQ] 90.6-100), days adherence (correct dose days) was 86.2% (IQ 61.1-96.2) and timing adherence (doses taken at correct time intervals) was 24.4% (IQ 5.3-56.5). Fourteen patients (12.5%) took less than 80% of prescribed doses, which was defined as suboptimal adherence. Patients with satisfactory adherence took a median of 8 mg/day (IQ 0-33) less than their prescribed dose of levodopa (P = NS), while suboptimal adherence patients took a median of 481 mg/day (IQ 205-670) less than their prescribed dose (P = 0.0006). The Parkinson motor score was significantly higher in patients with suboptimal adherence at 29 (IQ 20-40), versus those with satisfactory adherence at 19 (IQ 13-26), P = 0.005. Once daily drugs had significantly better adherence when compared with drugs prescribed more frequently (P < 0.0001). Suboptimal therapy adherence is associated with significant deviation from prescribed levodopa doses, despite greater Parkinson's motor severity. Optimizing oral medication intake has a potential role in maximizing the therapy response in Parkinson's disease.

Publisher Summary Parkinsonism is defined as the akinetic-rigid syndrome that mimics Parkinson's disease (PD). Apart from PD itself, the most representative diseases are progressive supranuclear palsy, multiple system atrophy, corticobasal degeneration, and dementia with Lewy bodies. This chapter reviews the scientific background in support of the use of scales as instruments for measurement, as well as the most relevant scales specifically designed for parkinsonism. The Likert model is the most frequently used scale model. It provides composite scores, drawn from the sum of the individual items, to represent the construct. Scales used to assess parkinsonism are classified into two categories: disease-centered and patient-centered measures. The disease-centered scales reflect aspects of interest to clinicians, and assess the severity of disease in terms of evolutionary stage, declared symptoms, signs obtained by means of examination by physicians or technicians, inability to perform activities of daily living (ADL), and complications. Patient-centered measures assess the impact of the disease and its consequences from patients' point of view, in terms of aspects that are of interest to them and are linked to quality of life and psychosocial adjustment.

Several neurochemical, neuropathological, neuroimaging, and experimental data, suggest that iron deficiency plays an important role in the pathophysiology of restless legs syndrome (RLS). Heme-oxygenases (HMOX) are an important defensive mechanism against oxidative stress, mainly through the degradation of heme to biliverdin, free iron, and carbon monoxide. We analyzed whether HMOX1 and HMOX2 genes are related with the risk to develop RLS. We analyzed the distribution of genotypes and allelic frequencies of the HMOX1 rs2071746, HMOX1 rs2071747, HMOX2 rs2270363, and HMOX2 rs1051308 SNPs, as well as the presence of Copy number variations (CNVs) of these genes in 205 subjects RLS and 445 healthy controls. The frequencies of rs2071746TT genotype and rs2071746T allelic variant were significantly lower in RLS patients than that in controls, although the other 3 studied SNPs did not differ between RLS patients and controls. None of the studied polymorphisms influenced the disease onset, severity of RLS, family history of RLS, serum ferritin levels, or response to dopaminergic agonist, clonazepam or GABAergic drugs. The present study suggests a weak association between HMOX1 rs2071746 polymorphism and the risk to develop RLS in the Spanish population.

The Fatigue Impact Scale for Daily Use (D-FIS) was used in a cross-sectional study including 142 consecutive Parkinson's disease (PD) patients. Usual clinical measures for PD, the Montgomery-Asberg Depression Rating Scale and the Parkinson's Disease Questionnaire-8 items were applied. In addition to the D-FIS, patients with fatigue (67.6%, PWF) completed the Multidimensional Fatigue Inventory (MFI), a visual analogue scale for fatigue (VAS-F) and a Global Perception of Fatigue scale (GPF). Relevant psychometric D-FIS results were: floor effect = 4.2%; ceiling effect = 1.1%; skewness = 0.44; item homogeneity = 0.63; Cronbach's alpha = 0.93; item-total correlation = 0.68 (item 1)-0.82 (item 8); standard error of measurement = 2.15; convergent validity with other fatigue measures = 0.54 [GPF]-0.62 [VAS-F] (p0.001). In a multiple linear regression model, fatigue, depression, and disability independently influenced HRQoL, as measured by the PDQ-8. Patients on amantadine had lower prevalence of fatigue. In PD, D-FIS is a consistent and valid measure for fatigue, a frequent symptom previously found to impair patients' HRQoL. Fatigue was also linked to depression and disability in this study.

In Parkinson's disease (PD), the Clinical Global Impression of Severity (CGIS) is often used as an additional outcome in clinical trials. It is hypothesized that this measure summarizes clinical assessment and is mainly influenced by the rating of four domains, namely, motor signs, disability, motor complications, and cognitive impairment. Ratings of these four factors were combined to form a new Clinical Impression of Severity Index for Parkinson's Disease (CISI-PD). A sample of 150 PD patients was evaluated using the following scales: Hoehn and Yahr staging, Schwab and England scale, Unified Parkinson's Disease Rating Scale, Scales for Outcomes in Parkinson's Disease-Motor Scale, CGIS, and CISI-PD. The results show that the CGIS is closely related to the above-mentioned measures (r=0.49-0.89). CISI-PD correlation with these scales was very similar (r=0.55-0.91), to the extent that the difference between corresponding coefficients was systematically less than 0.10. A multiple regression model showed that 92% of the CIGS variance was explained by the four CISI-PD items. Finally, the CISI-PD displayed adequate psychometric properties, with satisfactory internal consistency (alpha=0.90) and convergent (r>0.75) and known-groups validity. The CISI-PD is a valid and reliable measure that expands the information obtained via the CGIS.

We developed and tested the clinimetric properties of a scale for psychogenic movement disorders (PMDs). PMDs are disabling but lack any generally accepted treatment strategies. To develop treatments, means of assessing disease severity must be provided. No scale to assess PMDs existed. The PMD scale developed here rates 10 phenomena (rest tremor, action tremor, dystonia, chorea, bradykinesia, myoclonus, tics, athetosis, ballism, cerebellar incoordination), 2 functions (gait, speech), and 14 body regions. To study interrater agreement, three movement disorder neurologists independently rated 88 videotapes of PMD patients. Data analysis was performed using a kappa coefficient of agreement, Kendall's coefficient of concordance, Spearman correlations, and intraclass correlation coefficients. Validity and scale responsiveness were tested as well. All phenomena and speech and gait dysfunction occurred in the patient sample. A wide range of affected body regions, severity, and incapacitation was captured. Ratings showed excellent interrater reliability for presence or absence of each phenomenon (kappa range, 0.63 to 0.86). Kendall's concordance coefficients for phenomenology, function, and total PMD scores were 0.92, 0.93, and 0.91. Spearman correlations between raters ranged from 0.86 to 0.90. The scale was responsive to changes that occurred as a result of a neuropsychiatric intervention. The PMD scale adequately captures the complex movements of PMDs and can be used to assess PMDs and test the efficacy of intervention strategies.

Prompted by the lack of cross-cultural comparative data, and because a better understanding in the different clinical presentations of psychogenic movement disorders (PMDs) is relevant to neurological assessment and interventions, we compared the phenomenology, anatomical distribution, and functional impairment of PMDs in the United States and Spain. Consecutive patients diagnosed with PMD by a movement disorder specialist from one US site and from eight Spanish university centers were included in the study. The two groups were similar in their movement types, anatomical distribution, and functional impairment. PMDs were more prevalent in women than in men and were most common in upper and lower extremities. Gait and speech dysfunctions were distributed similarly in both countries. We found action tremor to be the most frequent PMD in both countries.

Objectives. Compare the efficacy of two walking assistance devices (wheeled walker and standard walker) to unassisted walking for patients with PD and gait freezing. Background. Although numerous walking devices are used clinically, their relative effects on freezing and walking speed have never been systematically tested. Methods. Nineteen PD patients (14 non-demented) walked under three conditions in randomized order: unassisted walking, standard walker, and wheeled walker. Patients walked up to three times in each condition through a standard course that included rising from a chair, walking through a doorway, straightway walking, pivoting, and return. Total walking time, freezing time and number of freezes were compared for the three conditions using mixed models (walking time) and Friedman's test (freezing). The wheeled walker was further studied by comparing the effect of an attached laser that projected a bar of light on the floor as a visual walking cue. Results. Use of either type of device significantly slowed walking compared to unassisted walking. Neither walker reduced any index of freezing, nor the laser attachment offered any advantage to the wheeled walker. The standard walker increased freezing, and the wheeled walker had no effect on freezing. Among the non-demented subjects (n=14), the same patterns occurred, although the walking speed was less impaired by the wheeled walker than the standard walker in this group. Conclusions. Though walkers may stabilize patients and increase confidence, PD patients walk more slowly when using them, without reducing freezing. Because the wheeled walker was intermediate for walking time and does not aggravate freezing, if walkers are used for these subjects, this type of walker should be favored.

Paraparesis is weakness affecting both lower extremities, and paraplegia is paralysis of the lower extremities. Although lower extremity weakness can be caused by peripheral nerve or muscle disease, the terms paraparesis and paraplegia are usually reserved for manifestations of lesions of the corticospinal tracts, frequently at the spinal cord level. Sudden paraparesis is most often caused by spinal cord trauma, whereas developmental or slowly progressive paraparesis can be caused by cerebral palsy, congenital malformation of the spinal cord, or familial neurodegenerative diseases. Diagnosis is usually confirmed by magnetic resonance imaging scans of the brain and spinal cord and, in selected cases, by electromyography, vitamin or metal levels, antibodies titers, and genetic testing. Treatment depends on the diagnosis and ranges from vitamin replacement to spinal cord surgery. For chronic paraplegia, long-term treatment involves rehabilitation, psychological support, and prevention of complications such as infections.

Friedreich's ataxia is an autosomal recessive genetic disorder. Clinical manifestations are neurological dysfunction, cardiomyopathy, and diabetes mellitus. It is a progressive disorder and patients become wheelchair-bound approximately 10–12 years after onset. Balance difficulties, bone deformities, progressive ataxia, and neuropathy are early signs. Genetic testing can confirm the diagnosis. There are no specific treatments, and management of the disease remains symptomatic and palliative.

Multisystem atrophy is a neurological disorder that includes degeneration in the extrapyramidal, pyramidal, autonomic, and cerebellar systems. The clinical hallmark features of multisystem atrophy are parkinsonism and varying degrees of hypotension, urinary disturbances, sexual impotency, ataxia, and weakness with spasticity.