Neurosonological Findings Related to Non-Motor Features of Parkinson's Disease: A Systematic Review.

Non-motor symptoms (NMS) in Parkinson's disease (PD), including neuropsychiatric or dysautonomic complaints, fatigue, or pain, are frequent and have a high impact on the patient's quality of life. They are often poorly recognized and inadequately treated. In the recent years, the growing awareness of NMS has favored the development of techniques that complement the clinician's diagnosis. This review provides an overview of the most important ultrasonographic findings related to the presence of various NMS. Literature research was conducted in PubMed, Scopus, and Web of Science from inception until January 2021, retrieving 23 prospective observational studies evaluating transcranial and cervical ultrasound in depression, dementia, dysautonomic symptoms, psychosis, and restless leg syndrome. Overall, the eligible articles showed good or fair quality according to the QUADAS-2 assessment. Brainstem raphe hypoechogenicity was related to the presence of depression in PD and also in depressed patients without PD, as well as to overactive bladder. Substantia nigra hyperechogenicity was frequent in patients with visual hallucinations, and larger intracranial ventricles correlated with dementia. Evaluation of the vagus nerve showed contradictory findings. The results of this systematic review demonstrated that transcranial ultrasound can be a useful complementary tool in the evaluation of NMS in PD.

Effectiveness and safety of PCSK9 inhibitors in real-world clinical practice. An observational multicentre study. The IRIS-PCSK9I study.

Background and aims: The benefits of the PCSK9 inhibitors, alirocumab and evolocumab, in lowering LDL-cholesterol and preventing major adverse cardiac events (MACE) have been demonstrated in pivotal clinical trials. However, few studies of routine clinical practice have been conducted to analyse and compare the efficacy and safety of the two drugs. Methods: Retrospective observational study of patients treated with a PCSK9 inhibitor in five hospitals in Andalusia (southern Spain). Baseline demographic and clinical data, LDL-cholesterol levels and the occurrence of MACEs during the follow-up period were recorded. Results: A total of 141 patients were included in the study: 90 were treated with alirocumab and 51 with evolocumab. The patients' mean age (IQR) was 58 (11) years and 58 (41%) were women. The most frequent concomitant medications were statins, 94 (66.7%), followed by antiplatelet therapy (66%) and ezetimibe (65.2%). The median (IQR) follow-up period was 18 (18) months, with 18 (24) for alirocumab and 11 (18) for evolocumab. At the six-month follow-up visit, LDL-cholesterol values had decreased to pre-treatment levels and remained significantly decreased (p < 0.05) over time, for both drugs, and a greater reduction was achieved in patients with established cardiovascular disease and concomitant treatment with statins. With respect to adverse effects, there were nine MACEs (6.4%), of which seven were with alirocumab (7.8%) and two with evolocumab (3.9%) (p NS). Other adverse effects (9.2%) included local erythema (3.5%), muscle cramps (2.1%), respiratory symptoms (2.1%) and asthaenia (1.4%). Conclusions: The efficacy and safety of alirocumab and evolocumab in routine clinical practice are consistent with the findings of the pivotal clinical trials.

Eslicarbazepine acetate and carotid intima-media thickness in epileptic patients.

OBJECTIVE: Evaluate if eslicarbazepine acetate (ESL) in combination with other non-inducer antiepileptic drugs (AEDs) in the treatment of epilepsy may represent a positive impact in the cardiovascular risk profile. METHODS: multicentre, retrospective, observational, non-interventional, real-life study comparing patients treated with cytochrome P450 (CYP) inducer vs. ESL plus non-inducer AEDs. Primary endpoint: Carotid intima-media thickness (CIMT) measured following the Manheim Consensus criteria. RESULTS: Patients included: 163. The main demographic, clinical and vascular risk parameters were comparable between the two groups except for duration of the disease, prevalence of dyslipidemia and use of lipid-lowering drugs (significantly higher in the inducers group) and number of previous antiepileptic drugs (significantly higher in the non-inducers group). Bivariate analysis of the main endpoint showed almost significant differences (p=0.05) in CIMT measures favourable to non-inducers (average 0.617mm+SD=0.148) vs. inducers (average 0.663mm+SD=0.147). Other variables reaching statistical significance were: age >50 years (p<0.001), high blood pressure (p<0.01) and dyslipidemia (p<0.05). A multivariate analysis including these variables and biochemical vascular risk factors showed a predictor model including two variables: inducers group (p=0.031; Coefficient ß=0.234) and age >50 years (p=0.001; Coefficient ß=0.387). Regarding gender, the mean CIMT in males was significantly higher in the inducers (0.693mm; SD=0.139) than in the non- inducers groups (0.628mm; SD=0.151; p<0.05). In females the differences were not significant. SIGNIFICANCE: The use of CYP inducer AEDs is associated with a significant increase in CIMT as compared with ESL and other non-inducer AEDs. The study shows a decrease in the vascular risk measured by ultrasound criteria in male patients treated with ESL compared with patients treated with inducer AEDs.

[Eslicarbazepine acetate in clinical practice. Efficacy and safety results]. / Acetato de eslicarbazepina en la practica clinica. Resultados de eficacia y seguridad.

INTRODUCTION. Eslicarbazepine acetate (ESL) is a new antiepileptic drug (AED) licensed in Spain in February 2011 as an adjunctive therapy in adults with partial seizures with or without secondary generalization. Clinical trials with ESL have demonstrated acceptable efficacy and safety. AIM. To evaluate the results of ESL in our epilepsy unit during its first year of clinical experience with this AED. PATIENTS AND METHODS. We included all patients who started treatment with ESL at our epilepsy unit from March 2011 to May 2012. We collected the following variables: gender, aetiology of epilepsy, epileptogenic area, reason for switch to ESL, clinical response after initiation of ESL, adverse effects of ESL, refractoriness criteria and treatment discontinuation. A bivariate factor-to-factor correlation study was carried out to establish associations between the independent variables and the clinical response. RESULTS. We recruited 105 patients (51.4% male). 20,7% of patients remained seizure-free and 58.4% showed > 50% improvement after introduction of ESL. At 6 months, 18.1% had experienced some type of side effect, with cognitive disorders being the most common, and 11.5% had discontinued treatment. Combination with lacosamide proved to be significantly less effective in the control of seizures. Combination of ESL with the rest of sodium channel inhibitors was similar in efficacy to others combinations. CONCLUSIONS. ESL is a well-tolerated and effective AED when is used as adjunctive treatment with most of other AED in clinical practice.

Characteristics of temporal lobe epilepsy with no ictal impairment of consciousness.

INTRODUCTION: The predominant manifestations of temporal lobe epilepsy (TLE) are partial seizures with impairment of consciousness (type I.B of ILAE classification), although consciousness impairment is not necessary in all seizures of patients with TLE. Nevertheless, there have been very few reports of TLE patients with exclusive seizures with no impairment of consciousness (i.e. isolated auras). The objective of this study was to determine any differential characteristics of this subgroup of TLE patients. MATERIAL AND METHODS: Retrospective case-control study in 163 consecutive TLE patients from our hospital database. The patients were divided between those with and without ictal impairment of consciousness, based on directed semi-structured questionnaire to the patient and relatives and on video-EEG records. Ten independent variables (8 clinical and 2 paraclinical) were compared between the groups. RESULTS: 14 patients (8.5%) formed the "TLE without ictal impairment of consciousness" group. This group was less refractory to medical treatment [Odds Ratio: 0.14 (0.03-0.64); p<0.01] and had frequent ictal motor behaviour [Odds ratio: 5.33 (1.65-17.14); p=0.008] and less frequent presence of automatisms [p<0.001]. Non-significant tendencies were observed for a higher frequency of lesional substrate and fewer generalization episodes. DISCUSSION: TLE without ictal impairment of consciousness appears to be more frequent than previously thought. This subgroup of TLE patients shows differential characteristics that may possibly result from a differential propagation of the original epileptic activity towards frontal areas rather than towards neocortical and diencephalic structures, which may be related to the more frequent presence of structural lesions.

Predictive model for refractoriness in temporal lobe epilepsy based on clinical and diagnostic test data.

INTRODUCTION: Temporal Lobe Epilepsy (TLE) is frequently resistant to drug treatment, but a high percentage of these patients can be free of seizures after epilepsy surgery. Delay in the surgical decision has been related to quality of life impairment, social and work limitations, and increased mortality risk. A predictive model for refractoriness based on clinical and diagnostic factors may allow its earlier detection and a shorter delay before surgery. MATERIAL AND METHODS: A case-control study was conducted in TLE patients over 16 years old. The dependent variable was resistance to medical treatment according to ILAE 2010 criteria. Independent variables were clinical, semiological, therapeutic, neurophysiological, radiological, and neuropsychological variables. A multivariate study was conducted to identify the variables associated with refractoriness, calculating the positive and negative predictive values and positive likelihood ratios of these variables individually and in combination. These data were used to construct a refractoriness predictive model. RESULTS: ILAE refractoriness criteria were met by 83 patients (50.9%). In the multivariate analysis, refractoriness was significantly associated with one semiological variable, one neuroradiological variable, one neurophysiological variable, and two therapeutic variables but not with neuropsychological test outcomes. These significant variables were used to construct a predictive model. CONCLUSION: Assessment of semiological, neurophysiological, and neuroradiological data can serve to stratify the risk of refractory epilepsy in TLE patients.

[Transient topographical amnesia: a description of a series of eight cases]. / Amnesia topografica transitoria: descripcion de una serie de ocho casos.

INTRODUCTION: Impaired memory, and more particularly spatial orientation, occurs in pathologies such as dementia, cerebrovascular accidents or traumatic brain injuries. Less frequently it also appears as a transient disorder in healthy people with no apparent brain damage, in which case it is known as transient topographical amnesia (TTA). The aim of this work is to report on a series of eight cases of TTA that were evaluated in a neurology unit. CASE REPORTS: We study the cases of eight patients diagnosed with TTA over the period 2002-2008. Patients were considered to fulfil eligibility criteria if they had presented at least one episode of spatial disorientation, with no loss of memory or consciousness, and were able to describe the events that had taken place, without any previous cognitive impairment and with a normal neurological examination. The demographic characteristics taken into account in the study were: predominance of females (75%) and a mean age of 69.13 +/- 8.79 years. The mean number of episodes was 1.75 (range: 1-3), which lasted an average of 24.5 minutes. Three of the eight patients had associated vascular risk factors. Neuroimaging studies did not reveal any relevant findings in any of the eight patients, except one case of a bilateral frontal porencephalic area resulting from a traumatic brain injury suffered in the past. Simple single-photon emission tomography and Doppler ultrasound imaging scans of the brain, as well as an electroencephalogram, were performed on two patients, the results being normal in all cases. Follow-ups were performed on all the patients, without any kind clinical change being observed, except for one patient who developed dementia at six years after the episode of amnesia. CONCLUSIONS: TTA is possibly an underdiagnosed condition which we believe should be included in the differential diagnosis of patients who are referred owing to suspected cognitive impairment.

Vohwinkel Syndrome secondary to missense mutation D66H in GJB2 gene (connexin 26) can include epileptic manifestations.

Vohwinkel Syndrome (VS) is a type of diffuse hereditary palmoplantar keratodermas (DHPPK) accompanied by skeletal dimorphisms and sensorineural deafness. The most frequently reported genetic substrate in VS is a point mutation of GJB2 gene, responsible for encoding connexin 26, a gap-junction protein with a crucial role in neuronal migration in rats. We report the case of a 21-year-old male who is a second-generation member of a family with VS and developed cryptogenic focal epilepsy. Genetic study showed a nucleotide change (c.196G>C) in exon 1 of GJB2 gene, producing a missense mutation, D66H. It is plausible that a functional alteration of connexin 26, such as that resulting of the mutation of our case, can produce an alteration in cortical development with epileptogenic potential. The present case and experimental evidence that connexin 26 is related to animal epileptogenesis suggest that the phenotypic spectrum of VS could be expanded to include epileptic manifestations.

Acetato de eslicarbazepina en la práctica clínica. Resultados de eficacia y seguridad / Eslicarbazepine acetate in clinical practice. Efficacy and safety results

Introducción. El acetato de eslicarbazepina (ESL) es un nuevo fármaco antiepiléptico aprobado en España en febrero de 2011 como tratamiento adyuvante en adultos con crisis parciales, con o sin generalización secundaria. Los ensayos clínicos con ESL han demostrado una aceptable eficacia y seguridad. Objetivo. Evaluar los resultados de la ESL tras el primer año de experiencia en la práctica clínica. Pacientes y métodos. Incluimos a todos los pacientes que, habiendo iniciado tratamiento con ESL entre marzo de 2011 y mayo de 2012 en nuestra unidad, habían completado los seis meses de tratamiento continuado. Se recogieron siete variables independientes clínicas y demográficas. Se realizó un análisis de correlación bivariante factor a factor para establecer las posibles asociaciones entre las variables independientes recogidas y la respuesta clínica. Resultados. Incluimos un total de 105 pacientes (el 51,4%, hombres). El 58,4% mostró una mejoría superior al 50% en el número de crisis tras la introducción de la ESL (el 20,7% quedó libre de crisis). Al cabo de seis meses, un 18,1% experimentó algún tipo de efecto adverso, siendo los más frecuentes las quejas cognitivas, y un 11,5% abandonó el tratamiento. La combinación con lacosamida mostró menos eficacia estadística en el control de las crisis que el resto de las combinaciones. La combinación de ESL con el resto de los inhibidores de los canales del sodio no mostró diferencias significativas de eficacia. Conclusiones. La ESL constituye un fármaco antiepiléptico con buena tolerancia, y con una tasa de efectos adversos y de retención aceptables cuando se usa en combinación con la mayoría de los antiepilépticos disponibles (AU)

Introduction. Eslicarbazepine acetate (ESL) is a new antiepileptic drug (AED) licensed in Spain in February 2011 as an adjunctive therapy in adults with partial seizures with or without secondary generalization. Clinical trials with ESL have demonstrated acceptable efficacy and safety. Aim. To evaluate the results of ESL in our epilepsy unit during its first year of clinical experience with this AED. Patients and methods. We included all patients who started treatment with ESL at our epilepsy unit from March 2011 to May 2012. We collected the following variables: gender, aetiology of epilepsy, epileptogenic area, reason for switch to ESL, clinical response after initiation of ESL, adverse effects of ESL, refractoriness criteria and treatment discontinuation. A bivariate factor-to-factor correlation study was carried out to establish associations between the independent variables and the clinical response. Results. We recruited 105 patients (51.4% male). 20,7% of patients remained seizure-free and 58.4% showed > 50% improvement after introduction of ESL. At 6 months, 18.1% had experienced some type of side effect, with cognitive disorders being the most common, and 11.5% had discontinued treatment. Combination with lacosamide proved to be significantly less effective in the control of seizures. Combination of ESL with the rest of sodium channel inhibitors was similar in efficacy to others combinations. Conclusions. ESL is a well-tolerated and effective AED when is used as adjunctive treatment with most of other AED in clinical practice (AU)

Infarto cortical que simula una parálisis del nervio accesorio / Cortical infarction mimicking paralysis of the accessory nerve

No disponible

No disponible

Amnesia topográfica transitoria: descripción de una serie de ocho casos / Transient topographical amnesia: a description of a series of eight cases

Introducción. La afectación de la memoria, en concreto la orientación espacial, está presente en patologías como la demencia, accidentes cerebrovasculares o traumatismos craneoencefálicos. Más raramente ocurre de forma transitoria en personas sanas sin daño cerebral evidente, denominándose amnesia topográfica transitoria (ATT). El objetivo de este trabajo es describir una serie de ocho casos de ATT valorados en una consulta de neurología. Casos clínicos. Serie de ocho pacientes diagnosticados de ATT entre los años 2002-2008. Como criterios de inclusión, seconsideraron aquéllos que habían presentado al menos un episodio de desorientación espacial episódica sin pérdida de memoria ni conciencia, pudiendo describir los eventos ocurridos, en ausencia de deterioro cognitivo previo y con exploración neurológica normal. Las características demográficas fueron: predominio de sexo femenino (75%) y edad media de 69,13 ± 8,79 años. El número medio de episodios fue de 1,75 (rango: 1-3), con una duración media de 24,5 minutos. Tres de los ocho pacientes asociaban factores de riesgo vascular. Los ocho pacientes tenían estudios de neuroimagen donde no se observaron hallazgos de interés, salvo un caso con un área porencefálica frontal bilateral antigua a un traumatismo craneoencefálico. Se realizó tomografía simple por emisión de fotón único cerebral y ecografía Doppler a dos pacientes, con resultados normales, así como electroencefalograma, que fue también normal. Hubo un seguimiento a todos los pacientes, sin observarse ningún cambio clínico, salvo un paciente que desarrolló demencia a los seis años del episodio de amnesia. Conclusiones. La ATT se trata posiblemente de una entidad infradiagnosticada, que creemos que debe incluirse en el diagnóstico diferencial de pacientes remitidos por sospecha de deterioro cognitivo (AU)

Introduction. Impaired memory, and more particularly spatial orientation, occurs in pathologies such as dementia, cerebrovascular accidents or traumatic brain injuries. Less frequently it also appears as a transient disorder in healthy people with no apparent brain damage, in which case it is known as transient topographical amnesia (TTA). The aim of this work is to report on a series of eight cases of TTA that were evaluated in a neurology unit. Case reports. We study the cases of eight patients diagnosed with TTA over the period 2002-2008. Patients were considered to fulfil eligibility criteria if they had presented at least one episode of spatial disorientation, with no loss of memory or consciousness, and were able to describe the events that had taken place, without any previous cognitive impairment and with a normal neurological examination. The demographic characteristics taken into account in the study were: predominance of females (75%) and a mean age of 69.13 ± 8.79 years. The mean number of episodes was 1.75 (range: 1-3), which lasted an average of 24.5 minutes. Three of the eight patients had associated vascular risk factors. Neuroimaging studies did not reveal any relevant findings in any of the eight patients, except one case of a bilateral frontal porencephalic area resulting from a traumatic brain injury suffered in the past. Simple single-photon emission tomography and Doppler ultrasound imaging scans of the brain, as well as an electroencephalogram, were performed on two patients, the results being normal in all cases. Follow-ups were performed on all the patients, without any kind clinical change being observed, except for one patient who developed dementia at six years after the episode of amnesia. Conclusions. TTA is possibly an underdiagnosed condition which we believe should be included in the differential diagnosis of patients who are referred owing to suspected cognitive impairment (AU)