Visually-Induced Motor Imagery Effects on Motor Adaptation to Reverse Steering Cycling. A Randomized Controlled Trial.

Purpose: First, testing an intervention of neuromodulation based on motor imagery and action observation as a promoter of motor adaptation of a complex motor task involving balance. Second, determining what prior balance factors can affect the motor adaptation task. Methods: A double-blind randomized controlled trial was performed. Forty-eight healthy subjects were recruited. The balance of all participants during gait and standing was assessed before adapting to the complex, multi-limb motor task of riding an inverse steering bicycle (ISB). Two interventions were carried out interleaved among trials of adaptation to the motor task: the experimental group (n = 24) was asked to perform neuromodulation (EN) by watching first-person ISB riding through immersive VR glasses and, simultaneously, mentally mimicking the movements. The control group (CG) was asked to watch a slideshow video of steady landscape images. Results: The results showed that the EN group did not improve the motor adaptation rate and induced higher adaptation times with respect to the CG. However, while the motor adaptation success showed a significant dependence on the prior proprioceptive participation in balance in the CG, the EN group did not present any relationship between the prior balance profile and motor adaptation outcome. Conclusions: Results point to a benefit of the visually guided neuromodulation for the motor adaptation of the subjects with low participation of proprioception in balance. Moreover, the results from the control group would allow to disclose prognostic factors about the success of the motor adaptation, and also prescription criteria for the proposed neuromodulation based on the balance profile.

[Incidence, circumstances and consequences of falls in subjects with stroke: One year of follow-up]. / Incidencia, circunstancias y consecuencias de caídas en sujetos con accidente cerebrovascular: un año de seguimiento.

INTRODUCTION: Falls are among the most frequent complications following stroke (CVA), and have a negative impact on rehabilitation. OBJECTIVES: To study the incidence, circumstances, and consequences of falls in stroke patients up to 12months after starting outpatient kinetic treatment. MATERIALS AND METHODS: Prospective design, case series. Consecutive sampling. Patients admitted to the day hospital between June 2019 and May 2020. Included: adults with a diagnosis of first supratentorial stroke and functional ambulatory category score ≥3. EXCLUSION CRITERIA: other condition affecting locomotion. MAIN VARIABLES: number of falls, circumstances, and consequences. Clinical, demographic, and functional characteristics were measured. RESULTS: Twenty-one subjects were included, 13 suffered at least one fall. The subjects reported 41 falls: 15 were to the most affected side, 35 inside the home, 28 without the indicated equipment, they were alone when the event occurred on 29 occasions, and in two situations medical assistance was required. There were statistically significant differences (P<.05) in functional performance (balance, gait velocity) between those who fell and those who did not. No significant differences were found between gait endurance and falls. CONCLUSION: More than half suffered a fall, alone, to the weaker side, and without the appropriate equipment. With this information the incidence could be reduced by preventive measures.

Abordaje neuropsicológico en una unidad pediátrica de daño cerebral adquirido del sistema público de salud / A neuropsychological approach in a paediatric acquired brain injury unit under the public health system

Introducción: El daño cerebral adquirido (DCA) pediátrico provoca dificultades cognitivo/conductuales y altera el curso del desarrollo. La unidad de DCA del Hospital Infantil Universitario Niño Jesús es la primera dentro del sistema público de salud en dar cobertura integral a pacientes y familias.ObjetivoSe pretende mostrar la metodología de trabajo con los niños y sus familias, describir las características clínicas de los pacientes atendidos y los resultados en cuanto a los tratamientos aplicados.Sujetos53 niños entre los tres meses y los 16 años y medio recibieron tratamiento. Las patologías atendidas son tumores cerebrales, accidentes cerebrovasculares, traumatismos craneoencefálicos, daño tras cirugía de la epilepsia e hipoxia.MétodoA todos los pacientes se le realizó una evaluación al ingreso y otra al alta. Los tratamientos se modulan en función de las dificultades y su gravedad, así como de la edad del niño. Las familias son atendidas tanto individualmente como en formato grupal.ResultadosUna mayor edad del niño se asocia con mejor recuperación del nivel cognitivo y menor duración del tratamiento. Las patologías tienen un impacto diferencial en el CI/CD evaluado al inicio de tratamiento, la hipoxia y las encefalitis son las que asocian mayor gravedad. Las puntuaciones al alta del CI/CD, así como las de memoria verbal y atención, mejoraron significativamente respecto a las del ingreso tras el tratamiento neuropsicológico multicomponente.ConclusionesLa atención al DCA debe incluir programas de rehabilitación neuropsicológica y proporcionar soporte emocional a la familia para que pueda participar activamente en la recuperación del niño o adolescente. (AU)

Introduction: Paediatric acquired brain injury (ABI) causes cognitive and behavioural difficulties and alters the course of child development. The ABI unit at Hospital Infantil Universitario Niño Jesús is the first within the public Spanish health system to provide comprehensive coverage to these patients and their families.ObjectiveThis study aims to show the working methodology followed with patients and their families, and to describe the clinical characteristics of the patients treated and the outcomes of treatment.PatientsFifty-three patients aged between three months and 16 and a half years received treatment. The conditions treated were brain tumours, stroke, traumatic brain injury, damage secondary to epilepsy surgery, and hypoxia.MethodsAll patients were evaluated at admission and at discharge. Treatments were adapted to each patient's difficulties and their severity, as well as to the patient's age. Families received individual and group therapy.ResultsOlder age was associated with better cognitive recovery and shorter duration of treatment. Different conditions show differential impact on intelligence quotient and developmental quotient scores at the beginning of treatment, with hypoxia and encephalitis being associated with greatest severity. Intelligence quotient and developmental quotient scores and visual memory and attention scores at discharge improved significantly after the faceted neuropsychological treatment with respect to scores registered at admission.ConclusionsThe care of patients with ABI should include neuropsychological rehabilitation programmes and provide emotional support to the family so that they may actively participate in the recovery of the child or adolescent. (AU)

A neuropsychological approach in a paediatric acquired brain injury unit under the public health system.

INTRODUCTION: Paediatric acquired brain injury (ABI) causes cognitive and behavioural difficulties and alters the course of child development. The ABI unit at Hospital Infantil Universitario Niño Jesús is the first within the public Spanish health system to provide comprehensive coverage to these patients and their families. OBJECTIVE: This study aims to show the working methodology followed with patients and their families, and to describe the clinical characteristics of the patients treated and the outcomes of treatment. PATIENTS: Fifty-three patients aged between 3 months and 16 and a half years received treatment. The conditions treated were brain tumours, stroke, traumatic brain injury, damage secondary to epilepsy surgery, and hypoxia. METHODS: All patients were evaluated at admission and at discharge. Treatments were adapted to each patient's difficulties and their severity, as well as to the patient's age. Families received individual and group therapy. RESULTS: Older age was associated with better cognitive recovery and shorter duration of treatment. Different conditions show differential impact on intelligence quotient and developmental quotient scores at the beginning of treatment, with hypoxia and encephalitis being associated with greatest severity. Intelligence quotient and developmental quotient scores and visual memory and attention scores at discharge improved significantly after the faceted neuropsychological treatment with respect to scores registered at admission. CONCLUSIONS: The care of patients with ABI should include neuropsychological rehabilitation programmes and provide emotional support to the family so that they may actively participate in the recovery of the child or adolescent.

Interacción con pantalla táctil de smartphone en pacientes con temblor esencial y sujetos sanos / Interaction with touchscreen smartphones in patients with essential tremor and healthy individuals

Introducción: El uso de smartphones en investigación biomédica está creciendo rápidamente en diferentes entornos clínicos. Realizamos un estudio piloto para obtener información sobre el uso de smartphones en pacientes con temblor esencial (TE) y en sujetos sanos, con el objetivo de evaluar si la realización de diversas tareas con las pantallas táctiles difiere entre grupos y describir factores de esta interacción.MétodoSe administró un cuestionario sobre el uso de smartphones a 31 pacientes con TE y 40 sujetos control apareados por edad y sexo. Acto seguido, los participantes interactuaron con una aplicación Android en desarrollo y realizaron 4 test basados en diferentes modos de interacción típicos con pantallas táctiles, con 5 repeticiones de cada tarea.ResultadoLos tipos de uso de smartphones así como su interacción no fueron significativamente diferentes entre pacientes y controles. La edad y el número de usos del smartphone son factores clave en esta interacción con pantallas táctiles.ConclusiónEstas observaciones apoyan el uso de las pantallas táctiles de los smartphones para investigación en TE, pero se requieren más estudios. (AU)

Introduction: Smartphones use in biomedical research is becoming more prevalent in different clinical settings. We performed a pilot study to obtain information on smartphone use by patients with essential tremor (ET) and healthy controls, with a view to determining whether performance of touchscreen tasks is different between these groups and describing touchscreen interaction factors.MethodA total of 31 patients with ET and 40 sex- and age-matched healthy controls completed a descriptive questionnaire about the use of smartphones. Participants subsequently interacted with an under-development Android application, and performed 4 tests evaluating typical touchscreen interaction gestures; each test was performed 5 times.ResultThe type of smartphone use and touchscreen interaction were not significantly different between patients and controls. Age and frequency of smartphone use are key factors in touchscreen interaction.ConclusionOur results support the use of smartphone touchscreens for research into ET, although further studies are required. (AU)

Interaction with touchscreen smartphones in patients with essential tremor and healthy individuals.

INTRODUCTION: Smartphone use in biomedical research is becoming more prevalent in different clinical settings. We performed a pilot study to obtain information on smartphone use by patients with essential tremor (ET) and healthy controls, with a view to determining whether performance of touchscreen tasks is different between these groups and describing touchscreen interaction factors. METHOD: A total of 31 patients with ET and 40 sex- and age-matched healthy controls completed a descriptive questionnaire about the use of smartphones. Participants subsequently interacted with an under-development Android application, and performed 4 tests evaluating typical touchscreen interaction gestures; each test was performed 5 times. RESULT: The type of smartphone use and touchscreen interaction were not significantly different between patients and controls. Age and frequency of smartphone use are key factors in touchscreen interaction. CONCLUSION: Our results support the use of smartphone touchscreens for research into ET, although further studies are required.

Prior cortical activity differences during an action observation plus motor imagery task related to motor adaptation performance of a coordinated multi-limb complex task.

Motor adaptation is the ability to develop new motor skills that makes performing a consolidated motor task under different psychophysical conditions possible. There exists a proven relationship between prior brain activity at rest and motor adaptation. However, the brain activity at rest is highly variable both between and within subjects. Here we hypothesize that the cortical activity during the original task to be later adapted is a more reliable and stronger determinant of motor adaptation. Consequently, we present a study to find cortical areas whose activity, both at rest and during first-person virtual reality simulation of bicycle riding, characterizes the subjects who did and did not adapt to ride a reverse steering bicycle, a complex motor adaptation task involving all limbs and balance. The results showed that cortical activity differences during the simulated task were higher, more significant, spatially larger, and spectrally wider than at rest for good performers. In this sense, the activity of the left anterior insula, left dorsolateral and ventrolateral inferior prefrontal areas, and left inferior premotor cortex (action understanding hub of the mirror neuron circuit) during simulated bicycle riding are the areas with the most descriptive power for the ability of adapting the motor task. Trials registration Trial was registered with the NIH Clinical Trials Registry (clinicaltrials.gov), with the registration number NCT02999516 (21/12/2016).

A neuropsychological approach in a paediatric acquired brain injury unit under the public health system. / Abordaje neuropsicológico en una unidad pediátrica de daño cerebral adquirido del sistema público de salud.

INTRODUCTION: Paediatric acquired brain injury (ABI) causes cognitive and behavioural difficulties and alters the course of child development. The ABI unit at Hospital Infantil Universitario Niño Jesús is the first within the public Spanish health system to provide comprehensive coverage to these patients and their families. OBJECTIVE: This study aims to show the working methodology followed with patients and their families, and to describe the clinical characteristics of the patients treated and the outcomes of treatment. PATIENTS: Fifty-three patients aged between three months and 16 and a half years received treatment. The conditions treated were brain tumours, stroke, traumatic brain injury, damage secondary to epilepsy surgery, and hypoxia. METHODS: All patients were evaluated at admission and at discharge. Treatments were adapted to each patient's difficulties and their severity, as well as to the patient's age. Families received individual and group therapy. RESULTS: Older age was associated with better cognitive recovery and shorter duration of treatment. Different conditions show differential impact on intelligence quotient and developmental quotient scores at the beginning of treatment, with hypoxia and encephalitis being associated with greatest severity. Intelligence quotient and developmental quotient scores and visual memory and attention scores at discharge improved significantly after the faceted neuropsychological treatment with respect to scores registered at admission. CONCLUSIONS: The care of patients with ABI should include neuropsychological rehabilitation programmes and provide emotional support to the family so that they may actively participate in the recovery of the child or adolescent.

Identification of clusters in multifocal electrophysiology recordings to maximize discriminant capacity (patients vs. control subjects).

PURPOSE: To propose a new method of identifying clusters in multifocal electrophysiology (multifocal electroretinogram: mfERG; multifocal visual-evoked potential: mfVEP) that conserve the maximum capacity to discriminate between patients and control subjects. METHODS: The theoretical framework proposed creates arbitrary N-size clusters of sectors. The capacity to discriminate between patients and control subjects is assessed by analysing the area under the receiver operator characteristic curve (AUC). As proof of concept, the method is validated using mfERG recordings taken from both eyes of control subjects (n = 6) and from patients with multiple sclerosis (n = 15). RESULTS: Considering the amplitude of wave P1 as the analysis parameter, the maximum value of AUC = 0.7042 is obtained with N = 9 sectors. Taking into account the AUC of the amplitudes and latencies of waves N1 and P1, the maximum value of the AUC = 0.6917 with N = 8 clustered sectors. The greatest discriminant capacity is obtained by analysing the latency of wave P1: AUC = 0.8854 with a cluster of N = 12 sectors. CONCLUSION: This paper demonstrates the effectiveness of a method able to determine the arbitrary clustering of multifocal responses that possesses the greatest capacity to discriminate between control subjects and patients when applied to the visual field of mfERG or mfVEP recordings. The method may prove helpful in diagnosing any disease that is identifiable in patients' mfERG or mfVEP recordings and is extensible to other clinical tests, such as optical coherence tomography.

Empirical Mode Decomposition-Based Filter Applied to Multifocal Electroretinograms in Multiple Sclerosis Diagnosis.

As multiple sclerosis (MS) usually affects the visual pathway, visual electrophysiological tests can be used to diagnose it. The objective of this paper is to research methods for processing multifocal electroretinogram (mfERG) recordings to improve the capacity to diagnose MS. MfERG recordings from 15 early-stage MS patients without a history of optic neuritis and from 6 control subjects were examined. A normative database was built from the control subject signals. The mfERG recordings were filtered using empirical mode decomposition (EMD). The correlation with the signals in a normative database was used as the classification feature. Using EMD-based filtering and performance correlation, the mean area under the curve (AUC) value was 0.90. The greatest discriminant capacity was obtained in ring 4 and in the inferior nasal quadrant (AUC values of 0.96 and 0.94, respectively). Our results suggest that the combination of filtering mfERG recordings using EMD and calculating the correlation with a normative database would make mfERG waveform analysis applicable to assessment of multiple sclerosis in early-stage patients.

Diagnostic ability of multifocal electroretinogram in early multiple sclerosis using a new signal analysis method.

PURPOSE: To determine if a novel analysis method will increase the diagnostic value of the multifocal electroretinogram (mfERG) in diagnosing early-stage multiple sclerosis (MS). METHODS: We studied the mfERG signals of OD (Oculus Dexter) eyes of fifteen patients diagnosed with early-stage MS (in all cases < 12 months) and without a history of optic neuritis (ON) (F:M = 11:4), and those of six controls (F:M = 3:3). We obtained values of amplitude and latency of N1 and P1 waves, and a method to assess normalized root-mean-square error (FNRMSE) between model signals and mfERG recordings was used. Responses of each eye were analysed at a global level, and by rings, quadrants and hemispheres. AUC (area under the ROC curve) is used as discriminant factor. RESULTS: The standard method of analysis obtains further discrimination between controls and MS in ring R3 (AUC = 0.82), analysing N1 waves amplitudes. In all of the retina analysis regions, FNRMSE value shows a greater discriminating power than the standard method. The highest AUC value (AUC = 0.91) was in the superior temporal quadrant. CONCLUSION: By analysing mfERG recordings and contrasting them with those of healthy controls it is possible to detect early-stage MS in patients without a previous history of ON.

[The clinical spectrum of progressive multifocal leukoencephalopathy: differences and similarities in patients with and without human immunodeficiency virus]. / Espectro clinico de la leucoencefalopatia multifocal progresiva: diferencias y similitudes en pacientes con y sin virus de la inmunodeficiencia humana.

AIM: To analyse the clinical findings, complementary examinations and prognosis of patients with progressive multifocal leukoencephalopathy (PML) treated in our institution, comparing populations with and without associated human immunodeficiency virus (HIV). PATIENTS AND METHODS: A retrospective study of the medical records of patients with probable or definite PML was carried out. Clinical variables, complementary studies (cerebrospinal fluid, magnetic resonance imaging of the brain) and prognostic variables were analysed. Non-parametric statistical tests were used to compare HIV-positive and HIV non-positive populations. RESULTS: Fourteen patients with definite and one probable diagnosis of PML were included. Nine patients had PML associated with HIV; five had other immunosuppressive conditions (two, chronic lymphatic leukaemia; one, multiple sclerosis; one, neuromyelitis optica; and one, neurosarcoidosis); and one, no obvious immunosuppressive condition. The population with HIV presented heterogeneous dirty-appearing white matter lesions more frequently (77.7% versus 16.67%; p = 0.0247) in the cerebral MRI. No other significant differences were identified in the remaining variables analysed. CONCLUSION: HIV/AIDS is the pathology most frequently associated with PML. With the use of immunomodulator drugs its appearance is reported in a variety of other diseases. Heterogeneous dirty-appearing white matter lesions were significantly more common in HIV patients.

TITLE: Espectro clinico de la leucoencefalopatia multifocal progresiva: diferencias y similitudes en pacientes con y sin virus de la inmunodeficiencia humana.Objetivo. Analizar los hallazgos clinicos, examenes complementarios y pronostico de los pacientes con leucoencefalopatia multifocal progresiva (LMP) atendidos en nuestra institucion, comparando las poblaciones con y sin virus de la inmunodeficiencia humana (VIH) asociado. Pacientes y metodos. Estudio retrospectivo de historias clinicas de pacientes con LMP probable o definitiva. Se analizaron variables clinicas, estudios complementarios (liquido cefalorraquideo, resonancia magnetica cerebral) y variables pronosticas. Mediante pruebas estadisticas no parametricas se realizo la comparacion entre las poblaciones con y sin VIH. Resultados. Se incluyo a 14 pacientes con diagnostico de LMP definitiva y uno probable. Nueve pacientes presentaron LMP asociada a VIH; cinco, otras condiciones de inmunoafectacion (dos, leucemia linfatica cronica; uno, esclerosis multiple; uno, neuromielitis optica; y uno, neurosarcoidosis); y uno, sin condicion inmunosupresora evidente. La poblacion con VIH presento con mayor frecuencia lesiones de la sustancia blanca heterogeneas de aspecto «sucio¼ (77,7% frente a 16,67%; p = 0,0247) en la resonancia magnetica cerebral. No se identificaron otras diferencias significativas en las restantes variables analizadas. Conclusion. El VIH/sida es la patologia mas frecuente asociada a LMP. Con el uso de farmacos inmunomoduladores se describe su aparicion en una variedad de otras enfermedades. Las lesiones de la sustancia blanca heterogeneas de aspecto «sucio¼ fueron significativamente mas frecuentes en pacientes con VIH.

[Towards excellence in hospital management. A description of strategical management model]. / Hacia la excelencia en gestión hospitalaria. Un modelo de gestión estratégica.

BACKGROUND AND OBJECTIVE: Health services are evolving from the traditional view of paternalistic medicine towards a more informed and participatory patient-provider relationship. This situation includes the empowerment and participation of patients and their families in health management, including their joint responsibility in the development of health services. The objective of this article is to describe the experience in a large teaching hospital in Spain on the application of a model that focuses on a three-fold approach to healthcare management that includes the experience of the patient. MATERIAL AND METHODS: This approach is identified by the initials EMC2 transforming the E of excellence into 3main areas of improvement: methodology, clinical knowledge and client, or patient, experience. the model has been implemented in different areas: childbirth, asthma care, outpatient clinics, and operating theatre. RESULTS: In the case of childbirth, there was a reduction in caesarean sections, episiotomies, induction of delivery, mean hospital stay, and number of deliveries with instrumentation. The Net Promoter Score went from 82 to 86. There was also an improvement in the areas of patient experience, hospital procedures, and clinical knowledge. Qualitative improvement results were obtained in other areas such as, asthma management, outpatient visits, and the operating room. CONCLUSIONS: This model can be applied to different areas. It promotes patient-focused practice, as well as system sustainability, efficiency, effectiveness, and level of satisfaction with the health care process.

Interaction with touchscreen smartphones in patients with essential tremor and healthy individuals. / Interacción con pantalla táctil de smartphone en pacientes con temblor esencial y sujetos sanos.

INTRODUCTION: Smartphones use in biomedical research is becoming more prevalent in different clinical settings. We performed a pilot study to obtain information on smartphone use by patients with essential tremor (ET) and healthy controls, with a view to determining whether performance of touchscreen tasks is different between these groups and describing touchscreen interaction factors. METHOD: A total of 31 patients with ET and 40 sex- and age-matched healthy controls completed a descriptive questionnaire about the use of smartphones. Participants subsequently interacted with an under-development Android application, and performed 4 tests evaluating typical touchscreen interaction gestures; each test was performed 5 times. RESULT: The type of smartphone use and touchscreen interaction were not significantly different between patients and controls. Age and frequency of smartphone use are key factors in touchscreen interaction. CONCLUSION: Our results support the use of smartphone touchscreens for research into ET, although further studies are required.

Aspectos clínicos y neuropsicológicos del síndrome de hipoventilación central congénita / Clinical and neuropsychological characteristics in congenital central hypoventilation syndrome

Introducción. El síndrome de hipoventilación central congénita (SHCC) es una enfermedad rara producida por mutaciones en el gen PHOX2B. Los pacientes muestran una reducida respuesta a la hipercapnia e hipoxia acompañada de alteraciones difusas del sistema nervioso autónomo y ocasionalmente alteraciones en neuroimagen. No se ha descrito un perfil neuropsicológico específico en los niños y adolescentes con SHCC. Casos clínicos. Se presentan tres casos (de edades comprendidas entre 4 y 19 años) con diferente perfil de afectación cognitiva y funcional. Se comparan los perfiles de los tres casos con los hallazgos descritos en la bibliografía sobre neuropsicología en el SHCC. Conclusiones. El perfil de afectación funcional en el SHCC es variable: en el caso 1 se describe un grave retraso global en el desarrollo con rasgos autistas y acusadas implicaciones funcionales. En el caso 2, la atrofia bilateral del hipocampo se asocia a déficit en cognición social y alteraciones en funciones ejecutivas con moderada repercusión funcional. El caso 3 muestra dificultades en algunas funciones ejecutivas cognitivas (planificación y fluidez no verbal), pero sin repercusión funcional. La evaluación neuropsicológica puede ayudar en el manejo clínico de estos pacientes determinando y orientando la necesidad de tratamientos rehabilitadores

Introduction. Congenital central hypoventilation syndrome (CCHS) syndrome is a rare disease caused by mutations in the PHOX2B gene. Patients show a reduced response to hypercapnia and hypoxia accompanied by diffuse disturbances of the autonomic nervous system and occasionaly also disturbances in neuroimaging. A specific neuropsychological profile has not been described in children and adolescents with CCHS. Case reports. We describe three cases (aged between 4 and 19 years) with different profiles of affectation in cognitive and functionality. These profiles are compared with the features described in the literature about neuropsychology in CCHS. Conclusions. The profile of functional impairment in the CCHS is variable: in case 1, a severe global developmental delay with autistic features and marked functional involvement is described. In case 2, bilateral atrophy of the hippocampus is associated with involvement in social cognition and in executive functions with moderate functional repercussion. Case 3 shows difficulties in some cognitive executive functions (planning and non-verbal fluency), but without functional repercussion. Neuropsychological assessment can help in the clinical management of these patients by determining and guiding the need for rehabilitation treatments

Tremor severity in Parkinson's disease and cortical changes of areas controlling movement sequencing: A preliminary study.

There remains much to learn about the changes in cortical anatomy that are associated with tremor severity in Parkinson's disease (PD). For this reason, we used a combination of structural neuroimaging to measure cortical thickness and neurophysiological studies to analyze whether PD tremor was associated with cortex integrity. Magnetic resonance imaging and neurophysiological assessment were performed in 13 nondemented PD patients (9 women, 69.2%) with a clearly tremor-dominant phenotype. Cortical reconstruction and volumetric segmentation were performed with the Freesurfer image analysis software. Assessment of tremor was performed by means of high-density surface electromyography (hdEMG) and inertial measurement units (IMUs). Individual motor unit discharge patterns were identified from surface hdEMG and tremor metrics quantifying motor unit synchronization from IMUs. Increased motor unit synchronization (i.e., more severe tremor) was associated with cortical changes (i.e., atrophy) in wide-spread cortical areas, including caudal middle frontal regions bilaterally (dorsal premotor cortices), left inferior parietal lobe (posterior parietal cortex), left lateral orbitofrontal cortex, cingulate cortex bilaterally, left posterior and transverse temporal cortex, and left occipital lobe, as well as reduced left middle temporal volume. Given that the majority of these areas are involved in controlling movement sequencing, our results support Albert's classic hypothesis that PD tremor may be the result of an involuntary activation of a program of motor behavior used in the genesis of rapid voluntary alternating movements.

[Clinical and neuropsychological characteristics in congenital central hypoventilation syndrome]. / Aspectos clinicos y neuropsicologicos del sindrome de hipoventilacion central congenita.

INTRODUCTION: Congenital central hypoventilation syndrome (CCHS) syndrome is a rare disease caused by mutations in the PHOX2B gene. Patients show a reduced response to hypercapnia and hypoxia accompanied by diffuse disturbances of the autonomic nervous system and occasionaly also disturbances in neuroimaging. A specific neuropsychological profile has not been described in children and adolescents with CCHS. CASE REPORTS: We describe three cases (aged between 4 and 19 years) with different profiles of affectation in cognitive and functionality. These profiles are compared with the features described in the literature about neuropsychology in CCHS. CONCLUSIONS: The profile of functional impairment in the CCHS is variable: in case 1, a severe global developmental delay with autistic features and marked functional involvement is described. In case 2, bilateral atrophy of the hippocampus is associated with involvement in social cognition and in executive functions with moderate functional repercussion. Case 3 shows difficulties in some cognitive executive functions (planning and non-verbal fluency), but without functional repercussion. Neuropsychological assessment can help in the clinical management of these patients by determining and guiding the need for rehabilitation treatments.

TITLE: Aspectos clinicos y neuropsicologicos del sindrome de hipoventilacion central congenita.Introduccion. El sindrome de hipoventilacion central congenita (SHCC) es una enfermedad rara producida por mutaciones en el gen PHOX2B. Los pacientes muestran una reducida respuesta a la hipercapnia e hipoxia acompañada de alteraciones difusas del sistema nervioso autonomo y ocasionalmente alteraciones en neuroimagen. No se ha descrito un perfil neuropsicologico especifico en los niños y adolescentes con SHCC. Casos clinicos. Se presentan tres casos (de edades comprendidas entre 4 y 19 años) con diferente perfil de afectacion cognitiva y funcional. Se comparan los perfiles de los tres casos con los hallazgos descritos en la bibliografia sobre neuropsicologia en el SHCC. Conclusiones. El perfil de afectacion funcional en el SHCC es variable: en el caso 1 se describe un grave retraso global en el desarrollo con rasgos autistas y acusadas implicaciones funcionales. En el caso 2, la atrofia bilateral del hipocampo se asocia a deficit en cognicion social y alteraciones en funciones ejecutivas con moderada repercusion funcional. El caso 3 muestra dificultades en algunas funciones ejecutivas cognitivas (planificacion y fluidez no verbal), pero sin repercusion funcional. La evaluacion neuropsicologica puede ayudar en el manejo clinico de estos pacientes determinando y orientando la necesidad de tratamientos rehabilitadores.

Rehabilitación tras rotura completa de los músculos isquiotibiales: a propósito de un caso / Rehabilitation after total rupture of the hamstrings: apropos of a case

Las lesiones de los músculos isquiotibiales ocurren más frecuentemente en actividades en las que se combinan la contracción excéntrica y velocidad. Su biomecánica es compleja debido a su condición biarticular que hace que participen en la estabilización de la pelvis, rodilla de forma individual y de ambas articulaciones durante la marcha. Su lesión puede ir de una simple distensión (que conlleva un buen pronóstico de recuperación a corto plazo) a una rotura total de la inserción proximal (asociada más frecuentemente a una recuperación más compleja). Presentamos el caso clínico de una rotura total de la inserción proximal de los isquiotibiales con recidiva a las dos semanas y realizamos una revisión de la literatura (AU)

Hamstring injuries occur more frequently in activities combining eccentric contraction and speed. The biomechanics of these injuries are complex due to their bi-articular status, as the hamstrings are involved in the stabilization of the pelvis, knee individually, and both joints during gait. Hamstring injuries can range from a simple strain (which has a good prognosis for recovery in the short term) to total rupture of the proximal insertion (most frequently associated with more complex recovery). We present the clinical case of a total rupture of the proximal insertion of the hamstring that relapsed within two weeks and provide a review of the literature (AU)

Functional surgery in pediatric drug-resistant posterior cortex epilepsy: Electro-clinical findings, cognitive and seizure outcome.

PURPOSE: Epilepsies originated from the occipital, parietal and/or the posterior edge of the temporal lobe are grouped together into posterior cortex epilepsy (PCE). Our objective was firstly to describe electro-clinical and imaging findings in the presurgical evaluation of children with PCE, and secondly to identify potential factors associated with surgical and cognitive outcomes. METHOD: From the total of patients referred to the Epilepsy Monitoring Unit of 'Hospital Universitario Niño Jesús' from 2003 to 2016, 55 had drug-resistant PCE. Different variables obtained from the multimodal presurgical work-up were analyzed among patients achieving seizure freedom after surgery (ILAE class 1) and patients with persistent seizures. Categorical variables were compared with Fishers exact test and numeric variables with t-Student for independent samples, and multiple logistic regression were used to analyze predictive values. RESULTS: Median duration of epilepsy until surgery was 5 years [3-10 years]. Fifty patients showed lesions in the MRI, and 62.5% had concordant MRI-PET corregistration. 37 (67%) patients were operated (lesionectomy in 21 subjects, tailored resection based on intracranial studies in 16), and 23 (62,2%) reached ILAE class 1, with a mean follow-up period of 3.51 [1-12] years. A lower number of basal seizures and antiepileptic drugs, a well-defined lesion on the MRI, an epileptogenic zone (EZ) restricted to the posterior quadrant and the normalization of postsurgical EEGs were associated with seizure freedom (p<0.05). Additionally, 65% of patients had a long-term improvement of cognitive performances. CONCLUSIONS: Epilepsy surgery should be considered in children with drug-resistant PCE, especially in those with a restricted EZ.