Underestimated olfactory domains in Huntington's disease: odour discrimination and threshold.

BACKGROUND: Olfaction has recently found clinical value in prediction, discrimination and prognosis of some neurodegenerative disorders. However, data originating from standard tests on olfactory dysfunction in Huntington's disease are limited to odour identification, which is only one domain of olfactory perceptual space. METHOD: Twenty-five patients and 25 age- and gender-matched controls were evaluated by the Sniffin' Sticks test in three domains of odour threshold, odour discrimination, odour identification and the sum score of them. Patients' motor function was assessed based on the Unified Huntington's Disease Rating Scale. RESULTS: Compared with controls, patients' scores of all olfactory domains and their sum were significantly lower. Besides, our patients' odour threshold and odour discrimination impairments were more frequently impaired than odour identification impairment (86 per cent and 81 per cent vs 34 per cent, respectively). CONCLUSION: Olfactory impairment is a common finding in patients with Huntington's disease; it is not limited to odour identification but is more pronounced in odour discrimination and odour threshold.

How to estimate body composition in Huntington's disease. A cross-sectional, observational study using multiple frequencies bioimpedance. / Cómo estimar la composición corporal en la enfermedad de Huntington. Estudio transversal y observacional con bioimpedancia de múltiples frecuencias.

INTRODUCTION: Huntington´s disease (HD) is a rare neurodegenerative disorder. Reliable information about nutritional status, especially body composition from individuals with HD is critical for clinical care and research. The ease of application and portability of multiple frequencies bioelectrical impedance analysis (mfBIA) make it an attractive tool for measuring body composition, but its accuracy in HD is unknown. AIM: To evaluate the accuracy of mfBIA vs. Dual X-ray absorptiometry (DEXA) in HD. PATIENTS AND METHODS: Cross-sectional, observational, and single-center study. HD severity was measured using motor subscale of the unified Huntington´s disease rating scale (m-UHDRS) and the total functional capacity (TFC). Body composition was measured in terms of fat-free mass (FFM), fat mass (FM), fat-free mass index (FFMI), and fat mass index (FMI). Using Bland-Altman plots, we analyzed reliability between DEXA and mfBIA using the Intraclass Correlation Coefficient with 95% confidence intervals (CI) and bias estimates for all. RESULTS: We included 16 patients with HD, 7 men, and 9 women, median age of 58.5 (32;68) years, TFC: 10 (3;13), and m-UHDRS: 31 (7;85). The reliability between mfBIA and DEXA were high for FFMI in men: 0.88 (95% CI 0.17-0.98), and women: 0.90 (95% CI 0.61- 0.98); for FMI, men: 0.97 (95% CI 0.83-0.99), and women: 0.91 (95% CI 0.68-0.98). Compared to DEXA, mfBIA slightly overestimated FFM, FM, FMI and FFMI in men and underestimated FFMI in women. CONCLUSIONS: mfBIA is an easy-to-use, safe, non-invasive, accurate method for measuring body composition and nutritional status in patients with mild-moderate HD.

TITLE: Cómo estimar la composición corporal en la enfermedad de Huntington. Estudio transversal y observacional con bioimpedancia de múltiples frecuencias.Introducción. La enfermedad de Huntington (EH) es un trastorno raro neurodegenerativo. La información fiable del estado nutricional, especialmente de la composición corporal, es crítica en clínica y en investigación. La facilidad de aplicación y portabilidad del análisis de la bioimpedancia de múltiples frecuencias (mfBIA) la convierten en una herramienta atractiva para medirla, pero se desconoce su precisión en la EH. Objetivo. Evaluar la precisión del mfBIA frente a la absorciometría dual de rayos X (DEXA) en la EH. Pacientes y métodos. Estudio transversal, observacional y unicéntrico. La EH se midió con la subescala motora de la escala unificada de valoración de la EH y con la capacidad funcional total. La composición corporal se valoró según la masa libre de grasa (MLG), la masa grasa (MG), el índice de masa libre de grasa (IMLG) y el índice de masa grasa (IMG). Se utilizó el coeficiente de correlación intraclase con intervalos de confianza al 95% y estimaciones de sesgo mediante gráficos de Bland-Altman. Resultados. Se incluyó a 16 pacientes, siete hombres y nueve mujeres, con edad media de 58,5 (32-68) años, capacidad funcional total de 10 (3-13) y escala unificada de valoración de la EH de 31 (7-85). La fiabilidad era alta entre el mfBIA y la DEXA para el IMLG en hombres, 0,88 (intervalo de confianza al 95%: 0,17-0,98), y mujeres, 0,9 (intervalo de confianza al 95%: 0,61-0,98); y para el IMG en hombres, 0,97 (intervalo de confianza al 95%: 0,83-0,99), y mujeres, 0,91 (intervalo de confianza al 95%: 0,68-0,98). El mfBIA sobreestimó ligeramente la MLG, la MG, el IMG y el IMLG en los hombres, pero subestimó el IMLG en las mujeres. Conclusiones. El mfBIA es un método fácil de usar, seguro, no invasivo y preciso para medir la composición corporal y el estado nutricional en pacientes con EH leve-moderada.

Clinical utility of a personalized and long-term monitoring device for Parkinson's disease in a real clinical practice setting: An expert opinion survey on STAT-ON™.

BACKGROUND: STAT-ON™ is an objective tool that registers ON-OFF fluctuations making possible to know the state of the patient at every moment of the day in normal life. Our aim was to analyze the opinion of different Parkinson's disease experts about the STAT-ON™ tool after using the device in a real clinical practice setting (RCPS). METHODS: STAT-ON™ was provided by the Company Sense4Care to Spanish neurologists for using it in a RCPS. Each neurologist had the device for at least three months and could use it in PD patients at his/her own discretion. In February 2020, a survey with 30 questions was sent to all participants. RESULTS: Two thirds of neurologists (53.8% females; mean age 44.9±9 years old) worked in a Movement Disorders Unit, the average experience in PD was 16±6.9 years, and 40.7% of them had previously used other devices. A total of 119 evaluations were performed in 114 patients (range 2-9 by neurologist; mean 4.5±2.3). STAT-ON™ was considered "quite" to "very useful" by 74% of the neurologists with an overall opinion of 6.9±1.7 (0, worst; 10, best). STAT-ON™ was considered better than diaries by 70.3% of neurologists and a useful tool for the identification of patients with advanced PD by 81.5%. Proper identification of freezing of gait episodes and falls were frequent limitations reported. CONCLUSION: STAT-ON™ could be a useful device for using in PD patients in clinical practice.

Impact of new technologies on neurology in Spain. Review by the New Technologies Ad-Hoc Committee of the Spanish Society of Neurology.

INTRODUCTION: New technologies are increasingly widespread in biomedicine. Using the consensus definition of new technologies established by the New Technologies Ad-Hoc Committee of the Spanish Society of Neurology (SEN), we evaluated the impact of these technologies on Spanish neurology, based on communications presented at Annual Meetings of the SEN. MATERIAL AND METHODS: We defined the concept of new technology in neurology as a novel technology or novel application of an existing technology, characterised by a certain degree of coherence persisting over time, with the potential to have an impact on the present and/or future of neurology. We conducted a descriptive study of scientific communications presented at the SEN's annual meetings from 2012 to 2018, analysing the type of technology, the field of neurology, and the geographical provenance of the studies. RESULTS: We identified 299 communications related with new technologies from a total of 8139 (3.7%), including 120 posters and 179 oral communications, ranging from 1.6% of all communications in 2012 to 6.8% in 2018. The technologies most commonly addressed were advanced neuroimaging (24.7%), biosensors (17.1%), electrophysiology and neurostimulation (14.7%), and telemedicine (13.7%). The neurological fields where new technologies were most widely employed were movement disorders (18.4%), cerebrovascular diseases (15.7%), and dementia (13.4%). Madrid was the region presenting the highest number of communications related to new technologies (32.8%), followed by Catalonia (26.8%) and Andalusia (9.0%). CONCLUSIONS: The number of communications addressing new technologies follows an upward trend. The number of technologies used in neurology has increased in parallel with their availability. We found scientific communications in all neurological subspecialties, with a heterogeneous geographical distribution.

Staging Parkinson's Disease Combining Motor and Nonmotor Symptoms Correlates with Disability and Quality of Life.

INTRODUCTION: In a degenerative disorder such as Parkinson's disease (PD), it is important to establish clinical stages that allow to know the course of the disease. Our aim was to analyze whether a scale combining Hoehn and Yahr's motor stage (H&Y) and the nonmotor symptoms burden (NMSB) (assessed by the nonmotor symptoms scale (NMSS)) provides information about the disability and the patient's quality of life (QoL) with regard to a defined clinical stage. MATERIALS AND METHODS: Cross-sectional study in which 603 PD patients from the COPPADIS cohort were classified according to H&Y (1, stage I; 2, stage II; 3, stage III; 4, stage IV/V) and NMSB (A: NMSS = 0-20; B: NMSS = 21-40; C: NMSS = 41-70; D: NMSS ≥ 71) in 16 stages (HY.NMSB, from 1A to 4D). QoL was assessed with the PDQ-39SI, PQ-10, and EUROHIS-QOL8 and disability with the Schwab&England ADL (Activities of Daily Living) scale. RESULTS: A worse QoL and greater disability were observed at a higher stage of H&Y and NMSB (p < 0.0001). Combining both (HY.NMSB), patients in stages 1C and 1D and 2C and 2D had significantly worse QoL and/or less autonomy for ADL than those in stages 2A and 2B and 3A and 3B, respectively (p < 0.005; e.g., PDQ-39SI in 1D [n = 15] vs 2A [n = 101]: 28.6 ± 17.1 vs 7.9 ± 5.8; p < 0.0001). CONCLUSION: The HY.NMSB scale is simple and reflects the degree of patient involvement more accurately than the H&Y. Patients with a lower H&Y stage may be more affected if they have a greater NMS burden.

Sleep Problems Are Related to a Worse Quality of Life and a Greater Non-Motor Symptoms Burden in Parkinson's Disease.

INTRODUCTION: The aim of the present study was to examine the frequency of self-reported sleep problems and their associated factors in a large cohort of PD patients. METHODS: PD patients and controls, recruited from 35 centers of Spain from the COPPADIS cohort were included in this cross-sectional study. Sleep problems were assessed by the Spanish version of the Parkinson's disease Sleep Scale version 1 (PDSS-1). An overall score below 82 or a score below 5 on at least 1 item was defined as sleep problems. RESULTS: The frequency of sleep problems was nearly double in PD patients compared to controls: 65.8% (448/681) vs 33.5% (65/206) (p < 0.0001). Mean total PDSS score was lower in PD patients than controls: 114.9 ± 28.8 vs 132.8 ± 16.3 (p < 0.0001). Quality of life (QoL) was worse in PD patients with sleep problems compared to those without: PDQ-39SI, 19.3 ± 14 vs 13 ± 11.6 (p < 0.0001); EUROHIS-QoL8, 3.7 ± 0.5 vs 3.9 ± 0.5 (p < 0.0001). Non-motor symptoms burden (NMSS; OR = 1.029; 95%CI 1.015-1.043; p < 0.0001) and impulse control behaviors (QUIP-RS; OR = 1.054; 95%CI 1.009-1.101; p = 0.018) were associated with sleep problems after adjustment for age, gender, disease duration, daily equivalent levodopa dose, H&Y, UPDRS-III, UPDRS-IV, PD-CRS, BDI-II, NPI, VAS-Pain, VAFS, FOGQ, and total number of non-antiparkinsonian treatments. CONCLUSION: Sleep problems were frequent in PD patients and were related to both a worse QoL and a greater non-motor symptoms burden in PD. These findings call for increased awareness of sleep problems in PD patients.

Depression is Associated with Impulse-compulsive Behaviors in Parkinson's disease.

BACKGROUND: Depression and impulse control disorders (ICDs) are both common in Parkinson's disease (PD) patients and their coexistence is frequent. Our aim was to determine the relationship between depression and impulsive-compulsive behaviors (ICBs) in a large cohort of PD patients. METHODS: PD patients recruited from 35 centers of Spain from the COPPADIS cohort from January 2016 to November 2017 were included in the study. The QUIP-RS (Questionnaire for Impulsive-Compulsive Disorders in Parkinson's Disease-Rating Scale) was used for screening ICDs (cutoff points: gambling ≥6, buying ≥8, sex≥8, eating≥7) and compulsive behaviors (CBs) (cutoff points: hobbyism-punding ≥7). Mood was assessed with the BDI-II (Beck Depression Inventory - II) and major, minor, and subthreshold depression were defined. RESULTS: Depression was more frequent in PD patients with ICBs than in those without: 66.3% (69/104) vs 47.5% (242/509); p<0.0001. Major depression was more frequent in this group as well: 22.1% [23/104] vs 14.5% [74/509]; p=0.041. Considering types of ICBs individually, depression was more frequent in patients with pathological gambling (88.9% [8/9] vs 50.2% [303/603]; p=0.021), compulsive eating behavior (65.9% [27/41] vs 49.7% [284/572]; p=0.032), and hobbyism-punding (69% [29/42] vs 49.4% [282/571]; p=0.010) than in those without, respectively. The presence of ICBs was also associated with depression (OR=1.831; 95%CI 1.048-3.201; p=0.034) after adjusting for age, sex, civil status, disease duration, equivalent daily levodopa dose, antidepressant treatment, Hoehn&Yahr stage, non-motor symptoms burden, autonomy for activities of daily living, and global perception of QoL. LIMITATIONS: Cross-sectional design. CONCLUSIONS: Depression is associated with ICBs in PD. Specifically, with pathological gambling, compulsive eating behavior, and hobbyism-punding.

Impact of new technologies on neurology in Spain. Review by the New Technologies Ad-Hoc Committee of the Spanish Society of Neurology. / Impacto de las nuevas tecnologías en la neurología en España. Revisión del Comité Ad-Hoc de Nuevas Tecnologías de la Sociedad Española de Neurología.

INTRODUCTION: New technologies (NT) are increasingly widespread in biomedicine. Using the consensus definition of NT established by the New Technologies Ad-Hoc Committee of the Spanish Society of Neurology (SEN), we evaluated the impact of these technologies on Spanish neurology, based on communications presented at Annual Meetings of the SEN. MATERIAL AND METHODS: We defined the concept of NT in neurology as a novel technology or novel application of an existing technology, characterised by a certain degree of coherence persisting over time, with the potential to have an impact on the present and/or future of neurology. We conducted a descriptive study of scientific communications presented at the SEN's annual meetings from 2012 to 2018, analysing the type of NT, the field of neurology, and the geographical provenance of the studies. RESULTS: We identified 299 communications related with NT from a total of 8,139 (3.7%), including 120 posters and 179 oral communications, ranging from 1.6% of all communications in 2012 to 6.8% in 2018. The technologies most commonly addressed were advanced neuroimaging (24.7%), biosensors (17.1%), electrophysiology and neurostimulation (14.7%), and telemedicine (13.7%). The neurological fields where NT were most widely employed were movement disorders (18.4%), cerebrovascular diseases (15.7%), and dementia (13.4%). Madrid was the region presenting the highest number of communications related to NT (32.8%), followed by Catalonia (26.8%) and Andalusia (9.0%). CONCLUSIONS: The number of communications addressing NT follows an upward trend. The number of NT used in neurology has increased in parallel with their availability. We found scientific communications in all neurological subspecialties, with a heterogeneous geographical distribution.

Clinical utility of a personalized and long-term monitoring device for Parkinson's disease in a real clinical practice setting: An expert opinion survey on STAT-ON™.

BACKGROUND: STAT-ON™ is an objective tool that registers ON-OFF fluctuations making possible to know the state of the patient at every moment of the day in normal life. Our aim was to analyze the opinion of different Parkinson's disease experts about the STAT-ON™ tool after using the device in a real clinical practice setting (RCPS). METHODS: STAT-ON™ was provided by the Company Sense4Care to Spanish neurologists for using it in a RCPS. Each neurologist had the device for at least three months and could use it in PD patients at his/her own discretion. In February 2020, a survey with 30 questions was sent to all participants. RESULTS: Two thirds of neurologists (53.8% females; mean age 44.9±9 years old) worked in a Movement Disorders Unit, the average experience in PD was 16±6.9 years, and 40.7% of them had previously used other devices. A total of 119 evaluations were performed in 114 patients (range 2-9 by neurologist; mean 4.5±2.3). STAT-ON™ was considered "quite" to "very useful" by 74% of the neurologists with an overall opinion of 6.9±1.7 (0, worst; 10, best). STAT-ON™ was considered better than diaries by 70.3% of neurologists and a useful tool for the identification of patients with advanced PD by 81.5%. Proper identification of freezing of gait episodes and falls were frequent limitations reported. CONCLUSION: STAT-ON™ could be a useful device for using in PD patients in clinical practice.

Quality of life and non-motor symptoms in Parkinson's disease patients with subthreshold depression.

BACKGROUND: The role of subthreshold depression (subD) in Parkinson's Disease (PD) is not clear. The present study aimed to compare the quality of life (QoL) in PD patients with subD vs patients with no depressive disorder (nonD). Factors related to subD were identified. MATERIAL AND METHODS: PD patients and controls recruited from the COPPADIS cohort were included. SubD was defined as Judd criteria. The 39-item Parkinson's disease Questionnaire (PDQ-39) and the EUROHIS-QOL 8-item index (EUROHIS-QOL8) were used to assess QoL. RESULTS: The frequency of depressive symptoms was higher in PD patients (n = 694) than in controls (n = 207) (p < 0.0001): major depression, 16.1% vs 7.8%; minor depression, 16.7% vs 7.3%; subD, 17.4% vs 5.8%. Both health-related QoL (PDQ-39; 18.1 ±â€¯12.8 vs 11.6 ±â€¯10; p < 0.0001) and global QoL (EUROHIS-QOL8; 3.7 ±â€¯0.5 vs 4 ±â€¯0.5; p < 0.0001) were significantly worse in subD (n = 120) than nonD (n = 348) PD patients. Non-motor Symptoms Scale (NMSS) total score was higher in subD patients (45.9 ±â€¯32 vs 29.1 ±â€¯25.8;p < 0.0001). Non-motor symptoms burden (NMSS;OR = 1.019;95%CI 1.011-1.028; p < 0.0001), neuropsychiatric symptoms (NPI; OR = 1.091; 95%CI 1.045-1.139; p < 0.0001), impulse control behaviors (QUIP-RS; OR = 1.035; 95%CI 1.007-1063; p = 0.013), quality of sleep (PDSS; OR = 0.991; 95%CI 0.983-0.999; p = 0.042), and fatigue (VAFS-physical; OR = 1.185; 95%CI 1.086-1.293; p < 0.0001; VAFS-mental; OR = 1.164; 95%CI 1.058-1.280; p = 0.0001) were related to subD after adjustment to age, disease duration, daily equivalent levodopa dose, motor status (UPDRS-III), and living alone. CONCLUSIONS: SubD is a frequent problem in patients with PD and is more prevalent in these patients than in controls. QoL is worse and non-motor symptoms burden is greater in subD PD patients.

[Validation study of the Spanish version of the Fahn-Tolosa-Marin scale for essential tremor]. / Estudio de validacion de la version española de la escala Fahn-Tolosa-Marin para el temblor esencial.

INTRODUCTION: The Fahn-Tolosa-Marin (FTM) tremor rating scale has been widely used in clinics for the estimation of tremor severity. However, a Spanish language version of this scale has still not been formally validated. AIM: To provide support to the validity and reliability of this version of FTM Scale. PATIENTS AND METHODS: A cross-sectional study was conducted on essential tremor patients. Severity was rated using the FTM scale. Upper limb disability was evaluated by terms of Disabilities of the Arm, Shoulder and Hand Scale (DASH), and to health-related quality of life using the Short Form-36 Health Survey (SF-36). Statistical analysis included sample description, reliability (Cronbach's alpha), convergent validity, and discrimination capacity tests (ROC curves). RESULTS: Forty patients with essential tremor (22 women, 18 men) were included, with a mean age of 65.8 ± 14.7 years (range: 21-90 years). Internal consistency of the FTM was high: Cronbach's alpha: 0,90 (subscale A: 0.85; subscale B: 0.91; subscale C: 0.77), and the floor and ceiling effects were negligible. The FTM (subscale C) showed high correlations with DASH, and acceptable diagnostic capacity, with an area under the curve of 0.86 (95% CI: 0.67-1.00), sensitivity 78% and specificity 75% for a cut-off score > 5.5. CONCLUSION: The Spanish version of FTM the rating scale is a reliable and valid tool to evaluate disability in patients with essential tremor, and a suitable instrument for use in medical research, as well as in clinical practice.

TITLE: Estudio de validacion de la version española de la escala Fahn-Tolosa-Marin para el temblor esencial.Introduccion. Hasta ahora no existe una validacion formal de la version española de la escala Fahn-Tolosa-Marin (FTM), usada ampliamente para valorar la gravedad del temblor. Objetivo. Analizar la validez y la fiabilidad de la version en castellano de la escala FTM. Pacientes y metodos. Estudio observacional transversal en pacientes diagnosticados de temblor esencial. Se evaluo la gravedad del temblor con la escala FTM; la discapacidad en el miembro superior, con la escala Disabilities of the Arm, Shoulder and Hand (DASH), y la calidad de vida relacionada con la salud, con el cuestionario Short Form-36 Health Survey (SF-36). El analisis estadistico incluyo descripcion de la muestra, fiabilidad (alfa de Cronbach), validez convergente y capacidad discriminatoria (curvas ROC). Resultados. Se incluyo a 40 pacientes (22 mujeres y 18 hombres con temblor esencial), con una edad media de 65,8 ± 14,7 años (rango: 21-90 años). La fiabilidad de la FTM fue alta, con un alfa de Cronbach de 0,90 (subescala A: 0,85; subescala B: 0,91; subescala C: 0,77). Para evaluar la discapacidad originada por el temblor esencial, la validez convergente entre las escalas DAHS y FTM (subescala C) fue adecuada, con una capacidad diagnostica aceptable: area bajo la curva de 0,86 (intervalo de confianza al 95%: 0,67-1,00), sensibilidad del 78% y especificidad del 75%, para un punto de corte mayor de 5,5. Conclusion. La escala FTM es un instrumento fiable, valido y preciso para la valoracion del temblor esencial en la poblacion española adulta.

Non-motor symptoms burden, mood, and gait problems are the most significant factors contributing to a poor quality of life in non-demented Parkinson's disease patients: Results from the COPPADIS Study Cohort.

OBJECTIVE: To identify factors related to a poor health-related and global quality of life (QoL) in a cohort of non-demented Parkinson's disease (PD) patients and compare to a control group. METHODS: The data correspond to the baseline evaluation of the COPPADIS-2015 Study, an observational, 5-year follow-up, multicenter, evaluation study. Three instruments were used to assess QoL: (1) the 39-item Parkinson's disease Questionnaire (PDQ-39), (2) a subjective rating of global QoL (PQ-10), and (3) the EUROHIS-QOL 8-item index (EUROHIS-QOL8). Multiple linear regression methods were used to evaluate the direct impact of different variables on these QoL measures. RESULTS: QoL was worse in PD patients (n = 692; 62.6 ±â€¯8.9 years old, 60.3% males) than controls (n = 206; 61 ±â€¯8.3 years old, 49.5% males): PDQ-39, 17.1 ±â€¯13.5 vs 4.4 ±â€¯6.3 (p < 0.0001); PQ-10, 7.3 ±â€¯1.6 vs 8.1 ±â€¯1.2 (p < 0.0001); EUROHIS-QOL8, 3.8 ±â€¯0.6 vs 4.2 ±â€¯0.5 (p < 0.0001). A high correlation was observed between PDQ-39 and Non-Motor Symptoms Scale (NMSS) (r = 0.72; p < 0.0001), and PDQ-39 and Beck Depression Inventory-II (BDI-II) (r = 0.65; p < 0.0001). For health-related QoL (PDQ-39), non-motor symptoms burden (NMSS), mood (BDI-II), and gait problems (Freezing Of Gait Questionnaire [FOGQ]) provided the highest contribution to the model (ß = 0.32, 0.28, and 0.27, respectively; p < 0.0001); whereas mood and gait problems contributed the most to global QoL (PQ-10, ß = -0.46 and -0.21, respectively; EUROHIS-QOL8, ß = -0.44 and -0.23, respectively). CONCLUSIONS: QoL is worse in PD patients than in controls. Mood, non-motor symptoms burden, and gait problems seem to be the most relevant factors affecting health-related and global perceived QoL in non-demented PD patients.

COPPADIS-2015 (COhort of Patients with PArkinson's DIsease in Spain, 2015): an ongoing global Parkinson's disease project about disease progression with more than 1000 subjects included. Results from the baseline evaluation.

BACKGROUND AND PURPOSE: In Parkinson's disease (PD), the course of the disorder is highly variable between patients. Well-designed, prospective studies for identifying PD progression biomarkers are necessary. Our aim was to show the results of baseline evaluations of an ongoing global PD project, COPPADIS-2015 (Cohort of Patients with PArkinson's DIsease in Spain, 2015). METHODS: This was an observational, descriptive, nationwide study (Spain). The recruitment period ended in October 2017. Baseline evaluation included more than 15 validated scales and complementary studies in a subgroup of participants. RESULTS: In total, 1174 subjects from 35 centres were considered valid for baseline analysis: 694 patients (62.6 ± 8.9 years old, 60.3% males), 273 caregivers (58.5 ± 11.9 years old, 31.8% males) and 207 controls (61 ± 8.3 years old, 49.5% males). The mean disease duration was 5.5 ± 4.4 years. Hoehn and Yahr stage was 1 or 2 in 90.7% of the patients whilst 33.9% and 18.1% of them presented motor fluctuations and dyskinesias, respectively. The mean Non-Motor Symptoms Scale total score was 45.4 ± 38.1, and 30.4% of the patients presented cognitive impairment, 16.1% major depression, 12.7% impulse control disorder, 7.2% compulsive behaviour, 57.2% pain and 13.2% falls. Compared to the control group, PD patients presented a significantly higher burden of non-motor symptoms and a worse quality of life. More than 300 subjects conducted complementary studies (serum biomarkers, genetic and neuroimaging). CONCLUSIONS: Parkinson's disease is a complex disorder and different non-motor symptoms are frequently present and are more prevalent than in controls. In real clinical practice it is important to ask for them.

The Promise of Telemedicine for Movement Disorders: an Interdisciplinary Approach.

PURPOSE OF REVIEW: Advances in technology have expanded telemedicine opportunities covering medical practice, research, and education. This is of particular importance in movement disorders (MDs), where the combination of disease progression, mobility limitations, and the sparse distribution of MD specialists increase the difficulty to access. In this review, we discuss the prospects, challenges, and strategies for telemedicine in MDs. RECENT FINDINGS: Telemedicine for MDs has been mainly evaluated in Parkinson's disease (PD) and compared to in-office care is cost-effective with similar clinical care, despite the barriers to engagement. However, particular groups including pediatric patients, rare MDs, and the use of telemedicine in underserved areas need further research. Interdisciplinary telemedicine and tele-education for MDs are feasible, provide similar care, and reduce travel costs and travel time compared to in-person visits. These benefits have been mainly demonstrated for PD but serve as a model for further validation in other movement disorders.

[Quality of life and associated characteristics of restless legs syndrome in the adult population of Burgos, Spain]. / Calidad de vida y caracteristicas asociadas del sindrome de piernas inquietas en la poblacion adulta de Burgos, España.

INTRODUCTION: Restless legs syndrome (RLS) is a neurological disorder with a prevalence of up to 15%, although little is known about its impact upon quality of life. AIM: To analyse the impact of RLS on health-related quality of life. SUBJECTS AND METHODS: A descriptive cross-sectional study. A random sample of 1,275 subjects over 18 years old, stratified by age, was taken from the urban area of Burgos, with an estimated prevalence, 10%; alpha, 5%; accuracy, 3%; and losses, 70%, using a two-phase study (screening and diagnosis of cases of RLS and non-cases confirmed by a doctor). The clinical and sociodemographic data were collected by means of semi-structured questionnaires, the European Quality of Life-5 Dimensions, the Restless Legs Syndrome Quality of Life Questionnaire, the Epworth Scale, the Goldberg Scale, the Sleep Scale and the International Restless Legs Syndrome Rating Scale. RESULTS: The prevalence of RLS was 5.6% (CI 95%: 2.5-8.7%). Of the total number of cases, 79.4% were women and only 7% had been previously diagnosed with RLS. Intense pain, insomnia and depression-anxiety were more frequent among the cases of RLS than in the controls (p < 0.001 in the three cases). The quality of life among the cases of RLS, especially in women, was poorer than among the controls (p < 0.001). For 11.7% of the cases, RLS made it difficult for the patients to work. CONCLUSIONS: RLS is associated with depression-anxiety, with a significant impact on sleep, on social and work relationships, and on the health-related quality of life.

TITLE: Calidad de vida y caracteristicas asociadas del sindrome de piernas inquietas en la poblacion adulta de Burgos, España.Introduccion. El sindrome de piernas inquietas (SPI) es un trastorno neurologico con una prevalencia de hasta un 15%, cuyo impacto sobre la calidad de vida resulta poco conocido. Objetivo. Analizar el impacto del sindrome de piernas inquietas (SPI) en la calidad de vida relacionada con la salud. Sujetos y metodos. Estudio descriptivo transversal. Se incluyo una muestra aleatoria, estratificada por edad, de 1.275 sujetos mayores de 18 años en el area urbana de Burgos, con las estimaciones de prevalencia, 10%; alfa, 5%; precision, 3%; y perdidas, 70%, usando un estudio en dos fases (cribado y confirmacion diagnostica de casos de SPI y no casos por un medico). Los datos clinicos y sociodemograficos se recogieron usando cuestionarios semiestructurados: European Quality of Life-5 Dimensions, Restless Legs Syndrome Quality of Life Questionnaire, escala de Epworth, escala de Goldberg, escala del sueño y cuestionario internacional del sindrome de piernas inquietas. Resultados. La prevalencia del SPI fue del 5,6% (IC 95%: 2,5-8,7%). El 79,4% de los casos eran mujeres; solo el 7% tenia un diagnostico previo de SPI. El dolor intenso, el insomnio y la depresion-ansiedad fueron mas frecuentes en los pacientes con SPI que en los controles (p < 0,001 en los tres casos). La calidad de vida entre los pacientes con SPI, especialmente en las mujeres, fue peor que en los controles (p < 0,001). En el 11,7% de los casos, el SPI produjo dificultades para trabajar. Conclusiones. El SPI esta asociado con ansiedad-depresion, con un significativo impacto en el sueño, en las relaciones sociales y laborales, y en la calidad de vida relacionada con la salud.

[Efficacy of a dynamic orthosis on the upper limbs in the chronic phase of strokes. A longitudinal study]. / Eficacia de la ortesis dinamica del miembro superior en la fase cronica del ictus. Estudio longitudinal.

AIMS: Stroke is the most important medical condition leading to permanent disability in adults. The aim of this study is to evaluate the efficacy of dynamic orthoses in the rehabilitation of the upper limbs in patients who have had a stroke. PATIENTS AND METHODS: We conducted a longitudinal case-control study. The sample used in the study consisted of patients from rehabilitation centres who presented hemiparesis secondary to an ischaemic or haemorrhagic stroke. The patients were randomly distributed into a study group, whose members received a dynamic orthosis on an upper limb for a six-month period, and a control group. Appraisals were performed pre- and post-treatment with the orthosis with Fugl-Meyer Assessment Scale and with Wolf Motor Function to measure their command over body functions and activities. Differences between pre- and post-test were compared using ANCOVA and Student's t. RESULTS: The sample included 40 patients (65% males) who presented chronic hemiparesis secondary to ischaemic (n = 28) or haemorrhagic stroke (n = 12), with a mean age of 58.43 ± 8.67 years. After the six-month follow-up, improved motor function was observed in both groups, according to both scales. The use of a dynamic orthosis was associated with a tendency towards improved strength in the upper limb. CONCLUSIONS: Rehabilitation following a stroke improves strength and body activities in the upper limb. The use of a dynamic orthosis can further improve the strength in this limb, but additional research is needed to confirm our results.

TITLE: Eficacia de la ortesis dinamica del miembro superior en la fase cronica del ictus. Estudio longitudinal.Objetivo. El ictus es la condicion medica mas importante que origina discapacidad permanente en el adulto. El objetivo es valorar la eficacia de ortesis dinamicas en la rehabilitacion del miembro superior en pacientes que han presentado un ictus. Pacientes y metodos. Estudio longitudinal de casos y controles. Se incluyo una muestra de pacientes procedentes de centros de rehabilitacion que presentaban hemiparesia secundaria a ictus isquemico o hemorragico. De forma aleatoria, los pacientes fueron distribuidos en un grupo de estudio, cuyos miembros recibieron una ortesis dinamica en el miembro superior durante seis meses, y un grupo control. Se realizaron valoraciones pre y postratamiento con la ortesis con la Fugl-Meyer Assessment Scale y con la Wolf Motor Function para medir los dominios de las funciones y actividades corporales. Se compararon las diferencias entre pre y postest usando ANCOVA y t de Student. Resultados. Se incluyo a 40 pacientes (65% hombres) que presentaban una hemiparesia cronica secundaria a ictus isquemico (n = 28) o hemorragico (n = 12), con una edad media de 58,43 ± 8,67 años. Tras el seguimiento de seis meses, se observo una mejoria en ambos grupos en la funcion motora segun ambas escalas. El uso de la ortesis dinamica se asocio a una tendencia hacia la mejoria en la fuerza de la extremidad superior. Conclusiones. La rehabilitacion tras el ictus mejora la fuerza y las actividades corporales en el miembro superior. El uso de una ortesis dinamica puede adicionalmente mejorar la fuerza en este miembro, pero se necesitan mas estudios para confirmar nuestros resultados.

Prospective study on cost-effectiveness of home-based motor assessment in Parkinson's disease.

Introduction Treatment adjustments in Parkinson's disease (PD) are in part dependent on motor assessments. The aim of this study was to evaluate the cost-effectiveness of home-based motor monitoring plus standard in-office visits versus in-office visits alone in patients with advanced PD. Methods The procedures consisted of a prospective, one-year follow-up, randomized, case-control study. A total of 40 patients with advanced PD were randomized into two groups: 20 patients underwent home-based motor monitoring by using wireless motion sensor technology, while the other 20 patients had in-office visits. Motor and non-motor symptom severities, quality of life, neuropsychiatric symptoms, and comorbidities were assessed every four months. Direct costs were assessed using a standardized questionnaire. Cost-effectiveness was assessed using the incremental cost-effectiveness ratio (ICER). Results Both groups of PD patients were largely comparable in their clinical and demographic variables at baseline; however, there were more participants using levodopa-carbidopa intestinal gel in the home-based motor monitoring group. There was a trend for lower Unified Parkinson's Disease Rating Scale functional status (UPDRS II) scores in the patients monitored at home compared to the standard clinical follow-up ( p = 0.06). However, UPDRS parts I, III, IV and quality-adjusted life-years scores were similar between both groups. Home-based motor monitoring was cost-effective in terms of improvement of functional status, motor severity, and motor complications (UPDRS II, III; IV subscales), with an ICER/UPDRS ranging from €126.72 to €701.31, respectively. Discussion Home-based motor monitoring is a tool which collects cost-effective clinical information and helps augment health care for patients with advanced PD.

[Psychometric attributes of the Spanish version of A-TAC screening scale for autism spectrum disorders]. / Atributos psicométricos de la versión española de la escala de cribado A-TAC para trastornos del espectro autista.

BACKGROUND: As there are no biological markers for Autism Spectrum Disorders (ASD), screening must focus on behaviour and the presence of a markedly abnormal development or a deficiency in verbal and non-verbal social interaction and communication. OBJECTIVE: To evaluate the psychometric attributes of a Spanish version of the autism domain of the Autism-Tics, AD/HD and other Comorbidities Inventory (A-TAC) scale for ASD screening. MATERIAL AND METHODS: A total of 140 subjects (43% male, 57% female) aged 6-16, with ASD (n=15), Mental Retardation (n=40), Psychiatric Illness (n=22), Tics (n=12) and controls (n=51), were included for ASD screening. The predictive validity, acceptability, scale assumptions, internal consistency, and precision were analysed. RESULTS: The internal consistency was high (α=0.93), and the standard error was adequate (1.13 [95% CI, -1.08 a 3.34]). The mean scores of the Autism module were higher in patients diagnosed with ASD and mental disability compared to the rest of the patients (P<.001). The area under the curve was 0.96 for the ASD group. CONCLUSION: The autism domain of the A-TAC scale seems to be a reliable, valid and precise tool for ASD screening in the Spanish school population.

[Neuroprotection in Parkinson's disease: analysis though group of experts' methodology]. / Neuroprotección en la enfermedad de Parkinson: análisis a través de la metodología de informadores clave.

INTRODUCTION: Currently used antiparkinsonian drugs neither stop nor slow-down the progressive nature of the disease. The final phase of PD is characterized by the presence of symptoms and signs resistant to dopaminergic agents, such as depression, dementia, freezing and falls. Therefore, it is urgent to develop therapies able to positively modify this outcome. Despite neuroprotection is a research priority in PD, no effective strategies have been found so far. METHOD: A key informants study was conducted. A group of experts in PD fulfilled a questionnaire of 10 questions to explore the most important topics related to neuroprotection. Afterwards a consensus about the current situation of neuroprotection in PD was established and future directions of development were suggested. RESULTS: Most of the answers emphasized the need of new concepts, the limitations of animal models and the difficulties in the difficulties in demonstrating a neuroprotective effects in humans owing to a lack of biomarkers. Some of the experts believe that we are already exerting a disease modifying effect. CONCLUSIONS: The concept of neuroprotection should be widened. Animal models should be improved. A reliable biomarker to start neuroprotective therapies long before the appearance of motor symptoms and to evaluate the neuroprotective effect of any therapy should be urgently developed.