Informe de la Fundación del Cerebro. Impacto social de la enfermedad de Alzheimer y otras demencias / Report by the Spanish Foundation of the Brain on the social impact of Alzheimer disease and other types of dementia

INTRODUCCIÓN: El conocimiento del alcance socioeconómico de las enfermedades que cursan con demencia es esencial para la planificación de recursos y la concienciación social. DESARROLLO: Se ha realizado una revisión de los datos publicados hasta el momento sobre la epidemiología, morbilidad, mortalidad, discapacidad, dependencia e impacto económico de la demencia y la enfermedad de Alzheimer en España. CONCLUSIONES: La mayoría de estudios en población mayor de 65 años estiman una prevalencia entre el 4% y el 9%. La prevalencia es mayor en mujeres en casi todos los grupos de edad. La enfermedad de Alzheimer es la causa de demencia más frecuente (50-70% del total). La demencia provoca un aumento de la morbilidad, mortalidad, discapacidad y dependencia de los pacientes, con una importante disminución de la calidad de vida y la supervivencia. El 80% de los enfermos es cuidado por sus familias, que asumen de media el 87% del coste total, con la consiguiente sobrecarga y menoscabo de la salud y calidad de vida de los cuidadores. El impacto económico de la demencia es enorme, y de evaluación compleja, por la mezcla de costes sanitarios y no sanitarios, directos e indirectos. Es necesario desarrollar programas globales e incrementar los recursos enfocados a fomentar la investigación, prevención, diagnóstico precoz, tratamiento multidimensional y abordaje multidisciplinario, que permitan reducir la carga sanitaria, social y económica de la demencia

INTRODUCTION: Knowledge of the socioeconomic impact of dementia-related disorders is essential for appropriate management of healthcare resources and for raising social awareness. METHODS: We performed a literature review of the published evidence on the epidemiology, morbidity, mortality, associated disability and dependence, and economic impact of dementia and Alzheimer disease (AD) in Spain. CONCLUSIONS: Most population studies of patients older than 65 report prevalence rates ranging from 4% to 9%. Prevalence of dementia and AD is higher in women for nearly every age group. AD is the most common cause of dementia (50%-70% of all cases). Dementia is associated with increased morbidity, mortality, disability, and dependence, and results in a considerable decrease in quality of life and survival. Around 80% of all patients with dementia are cared for by their families, which cover a mean of 87% of the total economic cost, resulting in considerable economic and health burden on caregivers and loss of quality of life. The economic impact of dementia is huge and difficult to evaluate due to the combination of direct and indirect costs. More comprehensive programmes should be developed and resources dedicated to research, prevention, early diagnosis, multidimensional treatment, and multidisciplinary management of these patients in order to reduce the health, social, and economic burden of dementia

Report by the Spanish Foundation of the Brain on the social impact of Alzheimer disease and other types of dementia. / Informe de la Fundación del Cerebro. Impacto social de la enfermedad de Alzheimer y otras demencias.

INTRODUCTION: Knowledge of the socioeconomic impact of dementia-related disorders is essential for appropriate management of healthcare resources and for raising social awareness. METHODS: We performed a literature review of the published evidence on the epidemiology, morbidity, mortality, associated disability and dependence, and economic impact of dementia and Alzheimer disease (AD) in Spain. CONCLUSIONS: Most population studies of patients older than 65 report prevalence rates ranging from 4% to 9%. Prevalence of dementia and AD is higher in women for nearly every age group. AD is the most common cause of dementia (50%-70% of all cases). Dementia is associated with increased morbidity, mortality, disability, and dependence, and results in a considerable decrease in quality of life and survival. Around 80% of all patients with dementia are cared for by their families, which cover a mean of 87% of the total economic cost, resulting in considerable economic and health burden on caregivers and loss of quality of life. The economic impact of dementia is huge and difficult to evaluate due to the combination of direct and indirect costs. More comprehensive programmes should be developed and resources dedicated to research, prevention, early diagnosis, multidimensional treatment, and multidisciplinary management of these patients in order to reduce the health, social, and economic burden of dementia.

Mood in Parkinson's disease: From early- to late-stage disease.

BACKGROUND: Although depression is known to be frequent in Parkinson's disease (PD), it is unclear how mood can change and/or impact on patient's quality of life (QoL) over time. Our aim was to analyze the frequency of depression, mood related factors and the contribution of mood to a patient's QoL perception in regard to disease duration. METHODS: PD patients recruited from the COPPADIS cohort from January 2016 to November 2017 were included in this cross-sectional study. Three groups were defined: <5 years (Group A); from 5 to <10 years (Group B); ≥10 years (Group C). Analysis with well-planned linear regression models was conducted to determine how different factors contribute to mood (Beck Depression Inventory-II [BDI-II] as dependent variable), to health-related QoL (39-item Parkinson's Disease Questionnaire [PDQ-39SI] as dependent variable) and to global QoL (European Health Interview Survey - Quality of Life Eight-Item Index [EUROHIS-QOL8] as dependent variable). RESULTS: Six hundred and sixty-three PD patients (62.6 ± 8.9 years old, 59.6% males) were included: Group A, 50.1% (n = 332); Group B, 33.3% (n = 221) and Group C, 16.6% (n = 110). There were no differences between the three groups in terms of the frequency of depressive symptoms nor the frequency of depression type (major vs. minor vs. subthreshold) (p = 0.729). However, the unique percent variance of PDQ-39SI and EUROHIS-QOL8 explained by BDI-II total score was 2 (23.7%) and threefold (26.9%), respectively, in Group C compared to the other two groups. EUROHIS-QOL8 total score provided the highest unique contribution to mood (16.8%). CONCLUSIONS: Although depression-type frequency does not appear to change over time in PD; the contribution of mood on QoL perception is greater in patients with longer disease duration.

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.

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.

Eficacia del levetiracetam en pacientes con síndrome de Lennox-Gastaut. Presentación de un caso / Levetiracetam efficacy in patients with Lennox-Gastaut syndrome. Presentation of a case

Introducción: El síndrome de Lennox-Gastaut (SLG) es una de las encefalopatías epilépticas más severas de la infancia, caracterizada por la tríada electroclínica de actividad generalizada de punta onda lenta (POL) en el electroencefalograma (EEG), múltiples tipos de crisis epilépticas y retraso mental. Con este trabajo pretendemos describir el cuadro sindrómico en un paciente con antecedente de encefalopatía hipóxico-isquémica y SLG, y su respuesta al tratamiento con levetiracetam (LEV). Método: Estudio descriptivo evolutivo de un niño de 3 años con antecedentes obstétricos de asfixia intrauterina y repercusión multiorgánica, acidosis metabólica, shock hipovolémico y crisis convulsivas con edema cerebral que a los pocos meses de edad desarrolla un síndrome de West, resistente al tratamiento farmacológico. Progresivamente, la semiología de las crisis cambia a episodios de hipertonía generalizada y mioclonías, con actividad electroencefalográfica de punta-onda lenta. Resultados: Con el diagnóstico de SLG se inicia tratamiento con LEV, observándose una mejoría sustancial en la esfera cognitiva, en el control de las crisis, y en los hallazgos electroencefalográficos. Conclusiones: El SLG es uno de los síndromes epilépticos más graves en los pacientes pediátricos, tanto por su semiología como por su farmacorresistencia. El levetiracetam puede producir una mejoría cognitiva, además de contribuir al control de las crisis en estos pacientes (AU)

Introduction: The Lennox-Gastaut syndrome (LGS) is one of the most severe epileptic encephalopathies of childhood, characterized by electro-clinical triad of generalized spike-wave activity, slow (POL) in the electroencephalogram (EEG), multiple types of seizures and development delay. This paper intends to describe the syndrome in a patient with a history of hypoxic-ischaemic encephalopathy and Lennox-Gastaut syndrome, and a good response to treatment with levetiracetam (LEV). Method: Descriptive study on the development of a 3 year old child with intrauterine asphyxia, multiorgan failure, metabolic acidosis, hypovolemic shock, and seizures with cerebral oedema, who developed a West syndrome, resistant to drug treatment. The semiology of seizures progressively changed to generalized episodes of hypertonia and myoclonus, with slow spike-wave electroencephalographic activity. Results: With the diagnosis of Lennox-Gastaut syndrome the patient was treated with levetiracetam, showing a substantial improvement in the cognitive sphere, in the control of seizures, and electroencephalographic findings. Conclusions: Lennox-Gastaut syndrome is one of the most severe epileptic syndromes in paediatric patients. Levetiracetam can help cognitive improvement, and contribute to seizure control in these patients (AU)

Levetiracetam efficacy in patients with Lennox-Gastaut syndrome. Presentation of a case.

INTRODUCTION: The Lennox-Gastaut syndrome (LGS) is one of the most severe epileptic encephalopathies of childhood, characterized by electro-clinical triad of generalized spike-wave activity, slow (POL) in the electroencephalogram (EEG), multiple types of seizures and development delay. This paper intends to describe the syndrome in a patient with a history of hypoxic-ischaemic encephalopathy and Lennox-Gastaut syndrome, and a good response to treatment with levetiracetam (LEV). METHOD: Descriptive study on the development of a 3 year old child with intrauterine asphyxia, multiorgan failure, metabolic acidosis, hypovolemic shock, and seizures with cerebral oedema, who developed a West syndrome, resistant to drug treatment. The semiology of seizures progressively changed to generalized episodes of hypertonia and myoclonus, with slow spike-wave electroencephalographic activity. RESULTS: With the diagnosis of Lennox-Gastaut syndrome the patient was treated with levetiracetam, showing a substantial improvement in the cognitive sphere, in the control of seizures, and electroencephalographic findings. CONCLUSIONS: Lennox-Gastaut syndrome is one of the most severe epileptic syndromes in paediatric patients. Levetiracetam can help cognitive improvement, and contribute to seizure control in these patients.

[Psychometric attributes of the Parkinson's Disease-Cognitive Rating Scale. An independent validation study]. / Atributos psicométricos de la Parkinson's Disease-Cognitive Rating Scale. Estudio de validación independiente.

AIM: To perform an independent evaluation of the psychometric attributes of the Parkinson's Disease-Cognitive Rating Scale (PD-CRS). PATIENTS AND METHODS: The study involved patients with Parkinson's disease (PD) free of any impediments preventing them from participating in the required evaluation. Sociodemographic and historical data were collected for use in this observational, cross-sectional study and the following evaluations were employed: Scales for Outcomes in Parkinson's Disease-Motor Scale (SCOPA-Motor), Hoehn and Yahr staging (HY), Clinical Impression of Severity Index for Parkinson's Disease (CISI-PD), Minimental State Examination (MMSE), SCOPA-Cognitive (SCOPA-Cog), Non-Motor Symptoms Questionnaire and PD-CRS. Acceptability, internal consistence, construct validity and precision of the PD-CRS were analysed. RESULTS: The sample consisted of 50 patients, with a mean age of 63.6 +/- 9.3 years. In all, 66% were males, with a history of 9 +/- 5.7 years with PD, in HY stages 1 to 4. Twelve patients (24%) presented data suggestive of dementia. The PD-CRS score was: sub-cortical sub-scale: 60.9 +/- 16.5; cortical sub-scale: 27.9 +/- 4.4; and total PD-CRS: 88.7 +/- 19.8. The mean-median difference was < 10% of the maximum scores and the total score showed no ceiling or floor effect. Cronbach's alpha was 0.85; the item-total correlations ranged from 0.57 (naming) to 0.73 (working memory), and the homogeneity index of the items was 0.36. Correlation with the MMSE and the SCOPA-Cog was high (rS = 0.53 and 0.77). The PD-CRS score was significantly lower in patients with a low level of schooling and more severe PD according to levels on the CISI-PD and distinguished between patients with and without dementia (70.3 +/- 26.2 versus 94.5 +/- 13; p < 0.001. The standard error of the measurement was 1.98. CONCLUSIONS: The levels of acceptability, internal consistence, construct validity and precision displayed by the PD-CRS were satisfactory.

Atributos psicométricos de la Parkinson’s Disease-Cognitive Rating Scale. Estudio de validación independiente / Psychometric Attriutes of the Parkinson´s disease-cognitive rating scale. An independent validation study

Objetivo. Realizar una evaluación independiente de los atributos psicométricos de la Parkinson’s Disease-CognitiveRating Scale (PD-CRS). Pacientes y métodos. Pacientes con enfermedad de Parkinson (EP) sin impedimentos para la evaluaciónrequerida. Estudio observacional, transversal, con captación de datos sociodemográficos, históricos y las siguientes evaluaciones:Scales for Outcomes in Parkinson’s Disease-Escala motora (SCOPA-Motor), estadios de Hoehn y Yahr (HY), ClinicalImpression of Severity Index for Parkinson’s Disease (CISI-PD), Minimental State Examination (MMSE), SCOPA-Cognitive(SCOPA-Cog), Non-Motor Symptoms Questionnare y PD-CRS. Se analizaron la aceptabilidad, consistencia interna, validezde constructo y precisión de la PD-CRS. Resultados. Se incluyeron 50 pacientes, con una edad media de 63,6 ± 9,3 años.El 66% eran hombres, con 9 ± 5,7 años de duración de la EP, en estadio 1 a 4 de HY. Doce pacientes (24%) presentaban datossugerentes de demencia. La puntuación de la PD-CRS fue: subescala subcortical: 60,9 ± 16,5; subescala cortical: 27,9 ±4,4; y PD-CRS total: 88,7 ± 19,8. La diferencia media-mediana fue < 10% de las puntuaciones máximas, y la puntuación totalno presentó efecto suelo ni techo. El alfa de Cronbach fue de 0,85; las correlaciones ítem-total, de 0,57 (denominación) a0,73 (memoria de trabajo), y el índice de homogeneidad de los ítems, de 0,36. La correlación con el MMSE y la SCOPA-Cogfue alta (rS = 0,53 y 0,77). La puntuación de la PD-CRS fue significativamente menor en pacientes con bajo nivel educativo ymayor gravedad de la EP según niveles de la CISI-PD, y discriminó entre pacientes dementes y no dementes (70,3 ± 26,2 frentea 94,5 ± 13; p < 0,001). El error estándar de la medida fue de 1,98. Conclusión. La PD-CRS mostró aceptabilidad, consistenciainterna, validez de constructo y precisión satisfactorias (AU)

Aim. To perform an independent evaluation of the psychometric attributes of the Parkinson’s Disease-CognitiveRating Scale (PD-CRS). Patients and methods. The study involved patients with Parkinson’s disease (PD) free of any impedimentspreventing them from participating in the required evaluation. Sociodemographic and historical data were collected for use inthis observational, cross-sectional study and the following evaluations were employed: Scales for Outcomes in Parkinson’sDisease-Motor Scale (SCOPA-Motor), Hoehn and Yahr staging (HY), Clinical Impression of Severity Index for Parkinson’sDisease (CISI-PD), Minimental State Examination (MMSE), SCOPA-Cognitive (SCOPA-Cog), Non-Motor Symptoms Questionnaireand PD-CRS. Acceptability, internal consistence, construct validity and precision of the PD-CRS were analysed. Results. Thesample consisted of 50 patients, with a mean age of 63.6 ± 9.3 years. In all, 66% were males, with a history of 9 ± 5.7 yearswith PD, in HY stages 1 to 4. Twelve patients (24%) presented data suggestive of dementia. The PD-CRS score was: subcorticalsub-scale: 60.9 ± 16.5; cortical sub-scale: 27.9 ± 4.4; and total PD-CRS: 88.7 ± 19.8. The mean-median differencewas < 10% of the maximum scores and the total score showed no ceiling or floor effect. Cronbach’s alpha was 0.85; the itemtotalcorrelations ranged from 0.57 (naming) to 0.73 (working memory), and the homogeneity index of the items was 0.36.Correlation with the MMSE and the SCOPA-Cog was high (rS = 0.53 and 0.77). The PD-CRS score was significantly lower inpatients with a low level of schooling and more severe PD according to levels on the CISI-PD and distinguished betweenpatients with and without dementia (70.3 ± 26.2 versus 94.5 ± 13; p < 0.001. The standard error of the measurement was1.98. Conclusions. The levels of acceptability, internal consistence, construct validity and precision displayed by the PD-CRSwere satisfactory (AU)

Síndrome de Panayiotopoulos: a propósito de dos casos en hermanos gemelos / Panayiotopoulos syndrome: a report of two cases in twin brothers

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Vértigo como síntoma inicial de un mixoma en un varón joven / Vertigo as initial symptom of a mixoma in a young male

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