Long-term outcomes after new onset seizure in children living with HIV: A cohort study.

OBJECTIVE: To determine the long-term outcomes, including mortality and recurrent seizures, among children living with HIV (CLWH) who present with new onset seizure. METHODS: Zambian CLWH and new onset seizure were enrolled prospectively to determine the risk of and risk factors for recurrent seizures. Demographic data, clinical profiles, index seizure etiology, and 30-day mortality outcomes were previously reported. After discharge, children were followed quarterly to identify recurrent seizures and death. Given the high risk of early death, risk factors for recurrent seizure were evaluated using a model that adjusted for mortality. RESULTS: Among 73 children enrolled, 28 died (38%), 22 within 30-days of the index seizure. Median follow-up was 533 days (IQR 18-957) with 5% (4/73) lost to follow-up. Seizure recurrence was 19% among the entire cohort. Among children surviving at least 30-days after the index seizure, 27% had a recurrent seizure. Median time from index seizure to recurrent seizure was 161 days (IQR 86-269). Central nervous system opportunistic infection (CNS OI), as the cause for the index seizure was protective against recurrent seizures and higher functional status was a risk factor for seizure recurrence. SIGNIFICANCE: Among CLWH presenting with new onset seizure, mortality risks remain elevated beyond the acute illness period. Recurrent seizures are common and are more likely in children with higher level of functioning even after adjusting for the outcome of death. Newer antiseizure medications appropriate for co-usage with antiretroviral therapies are needed for the care of these children. CNS OI may represent a potentially reversible provocation for the index seizure, while seizures in high functioning CLWH without a CNS OI may be the result of a prior brain injury or susceptibility to seizures unrelated to HIV and thus represent an ongoing predisposition to seizures. PLAIN LANGUAGE SUMMARY: This study followed CLWH who experienced a new onset seizure to find out how many go on to have more seizures and identify any patient characteristics associated with having more seizures. The study found that mortality rates continue to be high beyond the acute clinical presentation with new onset seizure. Children with a CNS OI causing the new onset seizure had a lower risk of later seizures, possibly because the trigger for the seizure can be treated. In contrast, high functioning children without a CNS OI were at higher risk of future seizures.

Estimating the minimal clinically important difference of upper extremity outcome measures in chronic stroke patients with moderate to severe impairment: a cross-sectional study.

BACKGROUND: Approximately 70% of chronic stroke patients experience upper extremity (UE) functional impairments, and UE outcome measures are often used as quality-of-life indicators. OBJECTIVE: The purpose of this study was to estimate minimal clinically important difference (MCID) values for UE outcome measures in chronic stroke patients with moderate to severe UE hemiplegia. METHODS: This study was a cross-sectional study, conducted as a secondary analysis of data from the ReoGo-J study, a multicenter, prospective, randomized, parallel-group trial of robot-assisted self-training for UE hemiplegia in chronic stroke. The patients were randomized to 1 of 3 treatment groups. Treatment was provided 3 times a week for 10 weeks, and UE outcome measures were evaluated before and after treatment. The anchor-based method was used to estimate MCID values for UE outcome measures, with Stroke Impact Scale (SIS) subscales as anchors. MCID values were estimated by identifying cutoff values in a receiver operating characteristic (ROC) curve. RESULTS: Between-group comparisons of UE outcome measures, based on the clinically important difference (CID) values of SIS subscales, revealed significant differences in both the Amount of Use (AOU) and Quality of Movement (QOM) components of the Motor Activity Log (MAL)-14. The estimated MCID values were 0.89 for the AOU component and 0.77 for the QOM component. CONCLUSIONS: The estimated MCID values for the MAL-14 not only add information regarding the clinical characteristics of the MAL-14 but also facilitate interpretations of changing scores in chronic stroke patients with moderate to severe UE hemiplegia undergoing rehabilitation therapy. STUDY REGISTRATION: https://www.umin.ac.jp/ctr/index.htm (UMIN000022509; 1 July 2016).

Chronic brain damage in HIV-infected individuals under antiretroviral therapy is associated with viral reservoirs, sulfatide release, and compromised cell-to-cell communication.

HIV infection has become a chronic and manageable disease due to the effective use of antiretroviral therapies (ART); however, several chronic aging-related comorbidities, including cognitive impairment, remain a major public health issue. However, these mechanisms are unknown. Here, we identified that glial and myeloid viral reservoirs are associated with local myelin damage and the release of several myelin components, including the lipid sulfatide. Soluble sulfatide compromised gap junctional communication and calcium wave coordination, essential for proper cognition. We propose that soluble sulfatide could be a potential biomarker and contributor to white matter compromise observed in HIV-infected individuals even in the current ART era.

A Comprehensive and Advanced Mouse Model of Post-Traumatic Epilepsy with Robust Spontaneous Recurrent Seizures.

Traumatic brain injury (TBI) is a leading cause of epilepsy in military persons and civilians. Spontaneous recurrent seizures (SRSs) occur in the months or years following the injury, which is commonly referred to as post-traumatic epilepsy (PTE). Currently, there is no effective treatment or cure for PTE; therefore, there is a critical need to develop animal models to help further understand and assess mechanisms and interventions related to TBI-induced epilepsy. Despite many attempts to induce PTE in animals, success has been limited due to a lack of consistent SRSs after TBI. We present a comprehensive protocol to induce PTE after contusion brain injury in mice, which exhibit robust SRSs along with neurodegeneration and neuroinflammation. This article provides a complete set of protocols for injury, outcomes, troubleshooting, and data analysis. Our broad profiling of a TBI mouse reveals features of progressive, long-lasting epileptic activity, hippocampal sclerosis, and comorbid mood and memory deficits. Overall, the PTE mouse shows striking consistency in recapitulating major hallmark features of human PTE. This mouse model will be helpful in assessing mechanisms of and interventions for TBI-induced epileptogenesis, epilepsy, and neuropsychiatric dysfunction. © 2022 Wiley Periodicals LLC. Basic Protocol 1: Inducing controlled cortical impact injuries Support Protocol: Creating the custom domed camp Basic Protocol 2: Recording long-term video-EEG signals Basic Protocol 3: Analyzing video-EEG recordings.

Social and physical environmental factors in daily stepping activity in those with chronic stroke.

BACKGROUND, PURPOSE/OBJECTIVE: Walking behavior in the chronic stroke population is multi-factorial. Previous work focused on the role of physical and biopsychosocial factors in understanding daily stepping post stroke. However, qualitative evidence suggests that social and physical environmental factors also affect daily stepping in those with stroke. The purpose of this study was to understand the role of social and physical environmental factors in daily stepping after stroke. METHODS: A total of 249 individuals ≥6 months post stroke were included in this cross-sectional analysis (129 females, mean age 62.98 years, SD 11.94). The social environment included living situation, work status, and marital status. The physical environment included the Area Deprivation Index (ADI) and Walk Score. At least 3 days of stepping was collected using an accelerometry-based device. Predictors were entered sequentially into a regression model: demographic characteristics, social environmental factors, and physical environmental factors. RESULTS: After adjusting for demographic factors, social environmental factors explained 6.2% (p =.017) of the variance in post stroke daily stepping. The addition of physical environmental factors improved the model (ΔR2 =.029, p =.024). The final model explained 9.2% (p =.003) of the variance in daily stepping. Lower area deprivation (ADI ß = -0.178, p =.015) and working (working vs. retired ß = -0.187, p = .029 and working vs. unemployed ß = -0.227, p =.008) were associated with greater daily stepping. DISCUSSION/CONCLUSION: Social and physical environmental factors predicted daily stepping and should be considered when setting expectations relative to the effects of rehabilitation on daily stepping in individuals poststroke.

[Meta-Cognitive Ability and Brain Damage in Autoimmune Encephalitis: A Case Report]. / Otoimmün Ensefalitte Beyin Hasari ve Üst Bilissel Beceri: Bir Olgu Sunumu.

Autoimmune encephalitis is an important contributor to rapidly progressive cognitive and behavioral decline. The purpose of this work was to evaluate the effects of cognitive rehabilitation in a patient with autoimmune encephalitis. We also wanted to evaluate the effectiveness of rehabilitative treatment by monitoring the cognitive and metacognitive outcomes over a time interval. We reported a case of 22 year-old female patient with autoimmune encephalitis, cognitive behavioral impairments, and severe reduction in metarepresentational capacity. We performed an assessment of personality, neuropsychological, and meta-cognitive functions at the beginning of the rehabilitative training. The last evaluation was performed six months after the discharge from the rehabilitation unit. We applied a combination of remediation, psycho-educational treatment, and psychotherapy to improve the knowledge and the empathy of the patient, to promote the selfcontrol strategies, and to prompt better behavioral management. Our findings revealed an improvement in the performance of the individual tests after rehabilitative training.

Encefalopatía traumática crónica, definición, diagnóstico y prevención: revisión de la literatura / Chronic traumatic encephalopathy, definition, diagnosis and prevention: review of literature

La encefalopatía traumática crónica (ETC) es una enfermedad neurodegenerativa que se produce como consecuencia traumatismos cerebrales repetitivos; concusiones, que son un síndrome clínico que se caracteriza por una alteración de la función cerebral. Una concusión, bajo su estricta definición, no debiese causar cambios estructurales en el cerebro por lo que no sería visible a través de imágenes, sí existen cambios a nivel microscópicos, bioquímicos y biomecánicos. La mayoría de los pacientes tienen completa resolución de sus síntomas dentro de 10 días (90 por ciento), pero existe un pequeño porcentaje que persiste con estos, pudiendo presentarse como un síndrome postconcusional, síndrome de segundo impacto o una encefalopatía traumática crónica. La ETC se caracteriza por la acumulación de prot-tau hiperfosforilada en neuronas y astrocitos. Estas se van a presentar en forma de ovillos o hilos neurofibrilares. En etapas iniciales las encontraremos de forma focalizada en la corteza frontal y en las formas más severas su distribución será más generalizada, distribuyéndose en la mayoría de las regiones del cerebro. Su diagnóstico se realiza a través de histopatología, por lo que hasta el momento sólo se ha logrado post-mortem. Se está trabajando en nuevas tecnologías asociadas a biomarcadores y PET para lograr una diagnostico premortem. El mayor énfasis en el manejo de esta taupatía es la prevención y adecuado manejo de las concusiones.

Chronic Traumatic Encephalopathy (CTE) is a neurodegenerative disease which is produced as a consequence of repeated brain trauma: concussions, which are a clinical syndrome characterized by an alteration in brain functions. A concussion, understrict definition, should not cause structural changes to the brain. Therefore, it would not be possible to see through images if there were changes at a microscopic, biochemical level. Most patients see their symptoms completely resolved within 10 days (90 percent), but there is a small percentage which persists, and these might cause a post-concussional syndrome, second impact syndrome of chronic traumatic encephalopathy. CTE is characterized by the accumulation of hyper-phosphorylated Tau protein in neurons and astrocytes. These appear in the form of neurofibrillary tangles. During the initial stages they are focalized in the frontal cortex and, in more severe cases, their distribution is more generalized, spreading through the majority of the regions in the brain. It is diagnosis is done through histopathology. Thus, it has only been possible to do post mortem. New technologies associated with bio-markers and PET are being worked on to achieve a pre-mortem diagnosis. The greatest emphasis in the handling of this tauopathy lies in the prevention and the adequate handling of concussions.

Percutaneous Needle Tenotomy for the Treatment of Muscle and Tendon Contractures in Adults With Brain Damage: Results and Complications.

OBJECTIVE: To study the results and complications of percutaneous needle tenotomy for superficial retracted tendons in patients with brain damage. DESIGN: Prospective observational study. SETTING: University hospital. PARTICIPANTS: Patients with severe brain damage (N=38; mean age, 60.7y; age range, 24-93y; 21 women) requiring surgical management of contractures and eligible for percutaneous needle tenotomy were enrolled between February 2015 and February 2016. INTERVENTIONS: The percutaneous needle tenotomy gesture was performed by a physical medicine and rehabilitation physician trained by an orthopedic surgeon, under local or locoregional anesthesia. Treated tendons varied among patients. MAIN OUTCOME MEASURES: All patients were evaluated at 1, 3, and 6 months to assess surgical outcomes (joint range of motion [ROM], pain, and functional improvement) while screening for complications. RESULTS: Improvements in ROM (37/38) and contractures-related pain (12/12) were satisfactory. Functional results were satisfactory (Goal Attainment Scale score ≥0) for most patients (37/38): nursing (n=12), putting shoes on (n=8), getting in bed or sitting on a chair (n=6), verticalization (n=7), transfers and gait (n=8), and grip (n=2). Five patients had complications related to the surgical gesture: cast-related complications (n=2), hand hematoma (n=2), and cutaneous necrosis of the Achilles tendon in a patient with previous obliterative arteriopathy of the lower limbs (n=1). CONCLUSIONS: Percutaneous needle tenotomy yields good results in the management of selected superficial muscle and tendon contractures. The complications rate is very low, and this treatment can be an alternative to conventional surgery in frail patients with neurologic diseases.

Increased attentional load moves the left to the right.

INTRODUCTION: Unilateral brain damage can heterogeneously alter spatial processing. Very often brain-lesioned patients fail to report (neglect) items appearing within the contralesional space. Much less often patients mislocalize items' spatial position. We investigated whether a top-down attentional load manipulation (dual-tasking), known to result in contralesional omissions even in apparently unimpaired cases, might also induce spatial mislocalizations. METHOD: Nine right-hemisphere-damaged patients performed three computer-based tasks encompassing different levels of attentional load. The side of appearance of visual targets had to be reported either in isolation or while processing additional information (visual or auditory dual task). Spatial mislocalizations (from the contralesional hemispace towards the ipsilesional unaffected one) were then contrasted with omissions both within and across tasks, at individual as well as at group level. RESULTS: The representation of ipsilesional targets was accurate and not affected by dual-tasking requirements. Contralesional targets were instead often omitted and, under dual-task conditions, also mislocalized by four patients. Three cases reported a significant number of left targets as appearing on the right (alloesthesia). Two of these patients perceived more targets (albeit to a wrong spatial location) under dual- than under single-task load. In a fourth patient, increased visual load resulted in synchiria, the (mis)perception of single, contralesional targets as being two (one on each side). CONCLUSIONS: When the neural circuitry subtending spatial processing is damaged, an increase in task load can lead to either a disregard or a bias in the processing of contralesional hemispace. The spatial bias subtending mislocalizations seems to index a more severe deficit than neglect, as if contralesional space would be completely erased rather than merely ignored.

The Effects of Chronic Right Hemispheric Damage on the Allocation of Spatial Attention: Alterations of Accuracy and Reliability.

Right hemispheric damage (RHD) caused by strokes often induce attentional disorders such as hemispatial neglect. Most patients with neglect over time have a reduction in their ipsilesional spatial attentional bias. Despite this improvement in spatial bias, many patients remain disabled. The cause of this chronic disability is not fully known, but even in the absence of a directional spatial attentional bias, patients with RHD may have an impaired ability to accurately and precisely allocate their spatial attention. This inaccuracy and variable directional allocation of spatial attention may be revealed by repeated performance on a spatial attentional task, such as line bisection (LBT). Participants with strokes of their right versus left (LHD) hemisphere along with healthy controls (HC) performed 24 consecutive trials of 24 cm horizontal line bisections. A vector analysis of the magnitude and direction of deviations from midline, as well as their standard deviations (SD), were calculated. The results demonstrated no significant difference between the LHD, RHD and HC groups in overall spatial bias (mean bisection including magnitude and direction); however, the RHD group had a significantly larger variability of their spatial errors (SD), and made larger errors (from midline) than did the LHD and HC groups. There was a curvilinear relationship between the RHD participants' performance variability and their severity of their inaccuracy. Therefore, when compared to HC and LHD, the RHD subjects' performance on the LBT is more variable and inaccurate.

A missed diagnosis: acute encephalopathy with biphasic seizures and late reduced diffusion.

Acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) is a very rare encephalopathy subtype which is characterized by biphasic seizures and disturbance of consciousness in the acute stage followed in the subacute stage by restricted diffusion in the subcortical white matter and finally resulting in cerebral atrophy on magnetic resonance imaging. Although exact pathogenesis of AESD is uncertain, the etiology of AESD has been attributed to viral and bacterial infection. Here we report a case of AESD in a 15-year-old mentally and motor retarded patient, diagnosed based on clinicoradiological correlation.

Facial self-mutilation: an analysis of published cases.

OBJECTIVES: Facial self-mutilation is rare. It is usually discussed from the psychiatric or psychoanalytic perspectives but has little prominence in general medical literature. Our objective was to describe facial self-mutilation in terms of its comorbidities, and to outline the different types of facial mutilation, as well as the basic approach to the patients with facial self-mutilation. METHODS: We undertook a review of all published cases of facial self-mutilation (1960-2011). RESULTS: We identified 200 published cases in 123 relevant papers. Four major groups of comorbidities emerged: psychiatric, neurological and hereditary disorders, and a group of patients without identified comorbidities. There were three general patterns of facial self-mutilation: (1) major and definitive mutilation, with the ocular globe as primary target--seen in patients with psychotic disorders; (2) stereotypical mutilation involving the oral cavity and of variable degree of severity, most often seen in patients with hereditary neuropathy or encephalopathy; (3) mild chronic self-mutilation, seen in patients with non-psychotic psychiatric disorders, acquired neurological disorders, and patients without comorbidities. About 20% of patients that mutilated their face also mutilated extra-facial structures. Patients with psychiatric conditions, especially those with psychotic disorders, had significantly higher (p<0.05) rates of permanent facial self-mutilation than others. Most treatment plans were very individually based, but some principles, such as prevention of irreversible loss of function and structure, or development of infection are applicable to all patients with facial self-mutilation. CONCLUSIONS: Facial self-mutilation is a potentially severe manifestation of diverse conditions. Several aspects of facial self-mutilation remain to be fully characterised from a clinical perspective.

Consequences of brain damage in the public debate regarding the end of life. The mediatic prism: a reflection of reality?

For the last 20 years or so, conflicts on life-support have become the object of widespread media coverage. By focusing public opinion on the alleged physicians' unreasonable obstinacy, these publicized cases impact social debates on life-support. By these, they justify claims for the legalization of assisted suicide, specifically the practice of termination of life by lethal injection. Via a conducted survey of the various caretakers and families involved in this type of situation, we propose an analysis based on the different forms of unreasonable obstinacy. The reasonable or unreasonable nature of treatments can often be perceived differently by physicians, caretakers and families. At least 6 unreasonable obstinacy cases can be brought to light. Publicized cases always involve a conflict between the physicians in charge and the families who view the situation as unreasonable. Nonetheless, evidence shows that in these situations, the roles are often reversed, and the families are the ones demanding the use of unreasonable care. A typical example of this is a recent case that became the object of legal proceedings in France. As it turns out, the publicized filter does not reflect the true reality of cases involving unreasonable care. Specific procedures could aid in notifying the existence of such situations. The role of health care professionals (excluding physicians) appears to play an essential part in preventing these situations from happening.

[Clinical application of prism adaptation for patients with unilateral spatial neglect].

Unilateral spatial neglect (USN) is defined as a failure to pay attention to the contralesional space due to the brain damage. As this symptom affects on many aspect of activities of daily living, a lots of therapeutic approaches were developed so far. Among them, prism adaptation (PA) technique is one of the most promising methods because it could produce generalized and long lasting after-effect and is effective even for chronic USN patients. It is, however, true that there are USN patients showing no PA effect. Unfortunately, we cannot tell in advance who can adapt to prisms and show improvement of their neglect symptom at this moment. In further studies, to know who is more likely to adapt to prisms we should analyze clinical data from USN patients showing improvement by PA. In addition, to be an effective PA technique for much more patients with USN we should clarify what it the best condition in PA task.

Impact of an educational program on the feeding of neurologically impaired children / Impacto de uma acao educativa na alimentacao de criancas neuropatas

PURPOSE: To verify the impact of an educative program focused on aspects related to feeding developed with a group of caregivers of children with chronic non-progressive encephalopathy. METHODS: Cross-sectional comparative study conducted with 30 children diagnosed with chronic non-progressive encephalopathy and their caregivers with the use of a questionnaire and video recordings of a meal conducted by the main caregiver. In order to verify the impact of an educational program in the knowledge and conduct of caregivers, patients were divided into two groups: study - consisting of caregivers submitted to a questionnaire and a video recording before and after the educational program; control - group in which caregivers underwent the procedures in two occasions, but without access to the educational program. RESULTS: Around 93.33% of caregivers were females, most had low educational level, and only 10% had a professional activity. Previous knowledge of caregivers concerning feeding was restricted, with 66% of caregivers not knowing what aspiration was, 60% being unfamiliar with the complications associated with such occurrence, and 86.66% stating that there is no relation between voice and swallowing. During feeding, only 26.66% of the caregivers used verbal commands related to feeding, and 50% did not realize the difficulties presented by their children. We observed a difference with regard to knowledge and conduct in the study group only. CONCLUSION: The educational program had a positive impact on the knowledge and conduct of caregivers concerning the feeding of their children with chronic non-progressive encephalopathy. .

OBJETIVO: Verificar o impacto de uma ação educativa voltada aos cuidados com a alimentação desenvolvida com um grupo de cuidadores de crianças com encefalopatia crônica não progressiva (ECNP). MÉTODOS: Estudo comparativo transversal realizado com 30 crianças com ECNP e seus cuidadores, tendo-se realizado aplicação de questionário e registro de uma refeição conduzida pelo cuidador principal. A fim de se verificar o impacto da ação educativa no conhecimento e conduta dos cuidadores, estes foram divididos em dois grupos: estudo, composto por cuidadores submetidos à aplicação de questionário e registro em vídeo antes e após realização da ação educativa; e controle, grupo submetido aos procedimentos em dois momentos, porém sem acesso à ação educativa. RESULTADOS: 93,33% dos cuidadores eram do sexo feminino, a maioria apresentou baixo nível de escolaridade e apenas 10% desenvolviam alguma atividade profissional. O conhecimento prévio dos cuidadores quanto aos aspectos relacionados à alimentação foi restrito, tendo 66% destes referido não saber o que era aspiração, 60% afirmado não saber as complicações associadas a esta e 86,66% terem negado existir relação entre voz e deglutição. Durante a alimentação, apenas 26,66% dos pais fizeram uso de comandos verbais relacionados à alimentação e 50% não perceberam as dificuldades apresentadas pelo seu filho. Observou-se diferença quanto ao conhecimento e conduta de cuidadores apenas no grupo estudo. CONCLUSÃO: A ação educativa realizada apresentou impacto no conhecimento e conduta dos cuidadores quanto à alimentação de seus filhos com ECNP. .

[Neurological maturation retardation]. / Retraso madurativo neurológico.

INTRODUCTION: The early years of life, more especially the first three, are dominated by a series of anatomical-functional facts that express the progressive enrichment of behaviour through the process of neurological maturation, which is expressed as reaching the maximum level of functioning at each stage of development as a result of a process of learning. DEVELOPMENT: One's personal experience is used to address the main conceptual aspects that define neurological maturation both in its normal aspects, that is to say, its limits, and its pathological deviations, and there is a need for conceptual limits of the so-called maturation retardation. The concepts of 'risk child' and his or her neurodevelopmental control and prognostic factors are analysed. CONCLUSIONS: The ultimate and primordial aim of any longitudinal study is the early detection of all the neurological anomalies, including maturation retardation, since this makes it possible to establish an early--either curative or palliative--treatment without producing 'false positives'. At the same time, if the neurological pathology is defined as residual, help can also be established for the patient and his or her family so that both of them can adapt to the situation in a suitable manner.

TITLE: Retraso madurativo neurologico.Introduccion. Los primeros años de vida, esencialmente los primeros tres, estan presididos por una serie de hechos anatomofuncionales que expresan el progresivo enriquecimiento de la conducta a traves del proceso de maduracion neurologica, expresado como el alcanzar en cada etapa del desarrollo el maximo nivel funcional a traves del aprendizaje. Desarrollo. A partir de la experiencia personal, se abordan los principales aspectos conceptuales que definen la maduracion neurologica tanto en los aspectos de la normalidad, es decir, sus limites, como sus desviaciones patologicas, precisando limites conceptuales del llamado retraso madurativo. Se analizan los conceptos de 'niño de riesgo', y su control neuroevolutivo y factores de pronostico. Conclusiones. El fin ultimo y primordial de todos los estudios longitudinales es la deteccion precoz de todas las anomalias neurologicas ­incluido el retraso madurativo­ que permita un tratamiento precoz, curativo o paliativo, evitando generar 'falsos positivos' y, si la patologia neurologica queda definida como residual, establecer la ayuda al paciente y su entorno familiar para que ambos se adapten adecuadamente a esta situacion.

[Disorders of executive functions. Diagnosis and treatment]. / Trastornos de las funciones ejecutivas. Diagnóstico y tratamiento.

INTRODUCTION: Different contributions to the scientific literature made over the last few decades have confirmed the prefrontal cortex as the neurobiological basis of the executive functions. Today, both the evaluation protocols for carrying out diagnoses and the structuring of the treatment and neurocognitive stimulation plans must interpret each of the prefrontal syndromes (dorsolateral, medial or of the anterior cingulate, and orbitofrontal) involved in the aetiopathogenesis of the different neurodevelopmental and adult disorders in which the executive functions are affected. AIMS: To report on the latest advances in diagnosis and treatment of the executive functions and to stress the importance of identifying and understanding the three syndromes of prefrontal dysfunction in the phase of diagnosis and in neurocognitive rehabilitation. DEVELOPMENT: The authors carry out a review of the literature on the latest advances in neuroscience as regards the neurobiological and neuropsychological foundations of the executive functions and their diagnosis and treatment. CONCLUSIONS: Every day, the scientific community confirms the importance of identifying and understanding the brain circuits in the diagnostic stage, especially the prefrontal dysfunction syndromes involved in the neuropsychological deficits of the different neurodevelopmental and adult disorders, so as to be able to establish effective neurocognitive stimulation protocols.

TITLE: Trastornos de las funciones ejecutivas. Diagnostico y tratamiento.Introduccion. Los diferentes aportes de la literatura cientifica ratifican, desde hace un par de decadas, a la corteza prefrontal como base neurobiologica de las funciones ejecutivas. En la actualidad, tanto los protocolos de evaluacion para la realizacion del diagnostico como la estructuracion de los planes de tratamiento y estimulacion neurocognitiva deben hacer la lectura de cada uno de los sindromes prefrontales (dorsolateral, medial o del cingulo anterior, y orbitofrontal) implicados en la etiopatogenia de los diferentes trastornos del neurodesarrollo y del adulto en los que se ven afectadas las funciones ejecutivas. Objetivos. Exponer los ultimos avances sobre diagnostico y tratamiento de las funciones ejecutivas y resaltar la importancia de identificar y comprender en la fase diagnostica y en la rehabilitacion neurocognitiva los tres sindromes de disfuncion prefrontal. Desarrollo. Se realiza una revision bibliografica sobre los ultimos avances neurocientificos alrededor de las bases neurobiologicas, neuropsicologicas, diagnostico y tratamiento de las funciones ejecutivas. Conclusion. Cada dia la comunidad neurocientifica ratifica la importancia de identificar y comprender en la etapa diagnostica los circuitos cerebrales, especificamente los sindromes de disfuncion prefrontal implicados en los deficits neuropsicologicos de los diferentes trastornos del neurodesarrollo y del adulto para poder establecer protocolos eficaces de estimulacion neurocognitiva.

Impact of an educational program on the feeding of neurologically impaired children.

PURPOSE: To verify the impact of an educative program focused on aspects related to feeding developed with a group of caregivers of children with chronic non-progressive encephalopathy. METHODS: Cross-sectional comparative study conducted with 30 children diagnosed with chronic non-progressive encephalopathy and their caregivers with the use of a questionnaire and video recordings of a meal conducted by the main caregiver. In order to verify the impact of an educational program in the knowledge and conduct of caregivers, patients were divided into two groups: study--consisting of caregivers submitted to a questionnaire and a video recording before and after the educational program; control--group in which caregivers underwent the procedures in two occasions, but without access to the educational program. RESULTS: Around 93.33% of caregivers were females, most had low educational level, and only 10% had a professional activity. Previous knowledge of caregivers concerning feeding was restricted, with 66% of caregivers not knowing what aspiration was, 60% being unfamiliar with the complications associated with such occurrence, and 86.66% stating that there is no relation between voice and swallowing. During feeding, only 26.66% of the caregivers used verbal commands related to feeding, and 50% did not realize the difficulties presented by their children. We observed a difference with regard to knowledge and conduct in the study group only. CONCLUSION: The educational program had a positive impact on the knowledge and conduct of caregivers concerning the feeding of their children with chronic non-progressive encephalopathy.

Severity of disability in patients with cerebral palsy secondary to symptomatic congenital cytomegalovirus encephalopathy.

PURPOSE: Cytomegalovirus (CMV) is a leading cause of congenital encephalopathy and cerebral palsy (CP). In this study we report the severity of disability in individuals who developed CP secondary to symptomatic congenital CMV encephalopathy. METHODS: The medical records of patients with CP secondary to symptomatic congenital CMV encephalopathy diagnosed from 1995 to 2011 were retrospectively reviewed. Gross Motor Functional Classification Scale (GMFCS) level, language function, and swallowing function were collected. RESULTS: Twenty-three patients were found. Of those 23 patients, 83% (19/23) were at a GMFCS level IV or V, 9% (2/23) each GMFCS level II or III and none (0%) at GMFCS I. Eighteen patients were non-verbal, 3 had minimal to moderate verbal skills and 2 had no verbal impairment. Eighteen patients also had severe dysphagia requiring gastrostomy tube (GT) feedings, and 5 ate orally. There was a strong correlation between the severity of GMFCS and having a gastrostomy tube (p< 0.0006) and GMFCS and verbal skills (p< 0.0023). CONCLUSION: This study shows that patients with CP secondary to symptomatic congenital cytomegalovirus encephalopathy have a very high risk of having severe physical and cognitive disabilities. This information can help healthcare providers and caregivers plan for the potential long-term medical, rehabilitation, and financial needs of this group of patients.

Longitudinal neurodevelopmental evolution in children with severe non-progressive encephalopathy.

AIM: The aim of this study was to evaluate the longitudinal neurodevelopmental evolution in children with severe non-progressive encephalopathy. METHODS: Between 1984 and 2005, 17 patients diagnosed with severe non-progressive encephalopathy under the care of the Institute Provvida Madre underwent neurodevelopmental evaluation on an annual basis for at least five consecutive years using the Munich Functional Developmental Diagnostics (MFDD). The severity of each patient's encephalopathy was assessed using the Capacity Profile (CAP). Longitudinal development trends were assessed by means of linear regression analysis, while the degree of discontinuity of the development trajectories was quantified using the Mean Absolute Deviation from Perfect Linear Development (MADPLD). Spearman's rank correlation coefficient and the Mann-Whitney test have been used to investigate the statistical significance of the relationships among the various parameters. RESULTS: We found that patients with severe non-progressive encephalopathy showed, on average, a linear maturation of 1.5-2.5months per year, irrespective of the neurodevelopmental area considered. Nevertheless, we also discovered that the development trajectories could be discontinuous. Indeed, a given child can show no development sign at all for many years and then suddenly encounter a "development jump", especially in the active language and autonomy areas. However, the long-term development linearity hypothesis seemed to hold true in our study. We also found evidences suggesting that faster development in a given domain could be linked to faster development in other domains, that higher discontinuity in a given area could be associated with higher discontinuity in other areas and that higher degrees of discontinuity could be related to lower developmental evolutions. CONCLUSIONS: The main findings of this study are important for physicians to form prognoses, counsel effectively and appropriately target therapeutic interventions. In this perspective, there is a strong need to collect long-term repeated follow-up data concerning this group of infants in order to reinforce the findings presented. In fact, these results should be considered as a starting point for further research because they are based on a limited number of patients and more data are needed to confirm the findings.