Uso de overlays controlados electromiográficamente y su combinación con el tratamiento con alineadores / Use of electromyographically controlled overlays and their combination with and its combination with treatment with aligners

La dimensión vertical (DV) usualmente disminuye con el paso del tiempo y es uno de los objetivos más complicados en resolver en los casos de Ortodoncia, ya sea con brackets o con alineadores. Esta disminución de la DV tiene una influencia directa en el funcionamiento de la articulación temporomandibular (ATM). Las patologías de la ATM, que surgen por la pérdida de la DV, son usualmente tratadas en la fase I con placas que restablecen la posición articular y la función muscular. Luego de corregida la patología de la ATM, en la fase II hay que tratar en lo posible la oclusión, devolviendo al paciente un soporte fisiológico posterior. Aquí se presenta un caso clínico atendido con una Placa Neurofisiológica (PN) en la fase I, y en la fase II, el traslado de esta altura a los overlays (O) y su erupción posterior dentaria con alineadores. Controlando cada paso electromiográficamente para el restablecimiento y control de la función muscular.

The vertical dimension usually decreases over time and is one of the most complicated objectives to solve in Orthodontic cases, whether with braces or aligners. This decrease in vertical dimension has a direct influence on the physiology of the temporomandibular joint. TMJ pathologies that arise from the loss of vertical dimension are usually treated in Phase I with splints that restore joint position and muscle function. After correcting the TMJ pathology, in Phase II, the occlusion should be treated as much as possible, returning the patient to a physiological posterior support. A clinical case is presented involving treatment with a Neurophysiological Splint in the first phase, and in the second phase, the transfer of this height to overlays and subsequent dental eruption with aligners. Each step is electromyographically monitored for the restoration and control of muscle function.

[Chronology of Justo Gonzalo's research on brain dynamics]. / Cronología de la investigación sobre la dinámica cerebral de Justo Gonzalo.

INTRODUCTION: The Spanish neuroscientist Justo Gonzalo y Rodriguez-Leal (1910-1986) investigated the functional organisation of the cerebral cortex over more than four decades. His findings led him to formulate a neurophysiological theory based on the laws of nervous excitability, which he called brain dynamics. This paper presents in chronological order how the main ideas on which it is based arose. DEVELOPMENT: In 1939, Gonzalo observed the phenomena of dynamic action: asynchrony or disaggregation, facilitation and cerebral repercussion. This was followed by two principles: the cerebral effect of lesions according to their magnitude and position (1941), and spiral development of the sensory field (1947). At the same time, he characterised what he called the central syndrome of the cerebral cortex. In the 1950s he developed the concepts of the cortical gradient, similarity and allometry. In contrast to modular conceptions of the cerebral cortex, in which one region is responsible for one function, Gonzalo argued that 'cortical gradients provide the location of systems, while similarity and allometry reveal their functional mechanism.' CONCLUSIONS: The theory of brain dynamics was established in two stages. The first (between 1938 and 1950) had an important clinical foundation, involving the observation of new phenomena and the formulation of new concepts. The second (between 1950 and 1960) included the introduction of more far-reaching concepts, such as the functional cortical gradient, and allometry laws based on a change of scale. Today, various authors believe that the concept of the gradient is crucial for understanding how the brain is organised.

TITLE: Cronología de la investigación sobre la dinámica cerebral de Justo Gonzalo.Introducción. El neurocientífico español Justo Gonzalo y Rodríguez-Leal (1910-1986) investiga la organización funcional de la corteza cerebral durante más de cuatro décadas. Sus hallazgos le llevan a formular una teoría neurofisiológica basada en las leyes de la excitabilidad nerviosa, que denomina dinámica cerebral. En el presente trabajo se expone de forma cronológica cómo surgen las principales ideas sobre las que se articula.Desarrollo. En 1939 Gonzalo observa los denominados fenómenos de acción dinámica: desfasamiento, facilitación y repercusión cerebral. Le siguen dos principios: efecto cerebral de la lesión según la magnitud y posición (1941), y organización sensorial, según un desarrollo espiral (1947). Paralelamente, caracteriza lo que llama el síndrome central de la corteza cerebral. En la década de los cincuenta desarrolla los conceptos de gradiente cortical, similitud y alometría. En contraposición a las concepciones modulares de la corteza cerebral, en las que una región es responsable de una función, Gonzalo expresa que 'los gradientes corticales dan la localización de los sistemas mientras la similitud y alometría revelan su trama funcional'. Conclusiones. La teoría de dinámica cerebral se articula en dos etapas. La primera (de 1938 a 1950) se caracteriza por una importante base clínica con observación de nuevos fenómenos y formulación de nuevos conceptos. La segunda (de 1950 a 1960) incluye la introducción de conceptos de mayor alcance, como el gradiente funcional cortical, y leyes de alometría que se basan en un cambio de escala. Actualmente, varios autores consideran que el concepto de gradiente es clave para entender la organización cerebral.

An appraisal to Hans Berger by the time of his 150th birthday: the human EEG and tales of blood flow, heat and brain waves.

More than 100 years of research have passed by and still the human electroencephalogram (EEG) remains a puzzle to be solved. Starting from his studies on plethysmography until his theories on brain thermodynamics, Hans Berger was able to refine his method of recording cortical signs with the apparatus at his disposal in an ordinary neuropsychiatric yard towards an early account of human EEG. This review is an appraisal of his contribution to the field of modern neurophysiology.

Mais de 100 anos se passaram e o eletroencefalograma humano (EEG) continua sendo um enigma a ser desvendado. A partir de seus estudos sobre pletismografia até suas teorias sobre termodinâmica cerebral, Hans Berger conseguiu refinar seu método de registro da atividade elétrica cortical com os equipamentos a sua disposição em uma ala psiquiátrica comum produzindo uma descrição acurada do EEG humano. Esta revisão é um breve resumo de sua contribuição para o campo da neurofisiologia moderna.

Aula invertida y aula virtual en la formación del Técnico Superior en neurofisiología clínica / Inverted Classroom and Virtual Classroom in the Training of Higher-Degree Associates in Clinical Neurophysiology

Introducción: En las ciencias médicas son diversas las investigaciones asociadas a la modalidad virtual, sobre todo durante la pandemia por COVID-19. Sin embargo, en el campo de la neurofisiología clínica existen pocas experiencias descritas. Objetivo: Describir los resultados del desarrollo virtual de las asignaturas Historia de la Neurociencia y la Neurofisiología en Cuba e Introducción a la Metodología de la Investigación Científica, mediante el aula invertida y el aula virtual en la formación del Técnico Superior en neurofisiología clínica en La Habana. Métodos: Estudio descriptivo de corte transversal y no experimental. Se adecuaron las asignaturas en modalidad virtual, al aplicar un modelo constituido por cuatro fases: preparación, análisis y diseño, desarrollo e implementación, y evaluación. Resultados: Se preparó al claustro en materia de educación virtual. Se diseñaron estrategias didácticas para la virtualización del proceso de enseñanza-aprendizaje, mediante la aplicación del aula invertida y el aula virtual. En la fase de evaluación se obtuvieron valores altos al calcular los indicadores de promoción y calidad, y se aplicó una encuesta a los estudiantes. Para ambas asignaturas predominaron los valores de alta satisfacción en los criterios de la encuesta, excepto en Formas de evaluaciones y Distribución del tiempo en el aprendizaje, con mayores valores de satisfacción media. Conclusión: Los resultados obtenidos en el tránsito a la modalidad virtual cumplieron con los objetivos propuestos en el programa de las asignaturas, por lo que fue posible la enseñanza no presencial mediante el aula invertida y el aula virtual(AU)

Introduction: In medical sciences, there are several pieces of research associated with the virtual modality, especially during the COVID-19 pandemic. However, in the field of clinical neurophysiology, few experiences have been described. Objective: To describe the outcomes of virtually developing the subjects History of Neuroscience and Neurophysiology in Cuba and Introduction to the Methodology of Scientific Research, by means of the inverted classroom and the virtual classroom in the training of higher-degree associates in clinical neurophysiology in Havana. Methods: A descriptive, cross-sectional and nonexperimental study was carried out. The subjects were adapted to the virtual modality by applying a model consisting of four phases: preparation, analysis and design, development and implementation, and evaluation. Results: The faculty was trained in virtual education. Didactic strategies were designed for the virtualization of the teaching-learning process, through the application of the inverted classroom and the virtual classroom. In the evaluation phase, high values were obtained when calculating the promotion and quality indicators, and a student survey was applied. For both subjects, high satisfaction values predominated in the survey criteria, except for evaluations forms and distribution of time for learning, with higher values of the medium satisfaction. Conclusions: The outcomes obtained in the transition to the virtual modality complied with the objectives set in the syllabus of the subjects, making possible non-face-to-face teaching by means of the inverted classroom and the virtual classroom(AU)

Visual Field Coordinate Systems in Visual Neurophysiology.

In this work, algorithms have been developed to: 1. Compare visual field coordinate data, presented in different representation systems; 2. Determine the distance in degrees between any two points in the visual field; 3. Predict new coordinates of a given point in the visual field after the rotation of the head, around axes that pass through the nodal point of the eye. Formulas are proposed for the transformation of Polar coordinates into Zenithal Equatorial coordinates and vice versa; of Polar coordinates into Gnomic Equatorial of double meridians and vice versa; and projections of double meridians system into Zenithal Equatorial and vice versa. Using the transformation of polar coordinates into Cartesian coordinates, we can also propose algorithms for rotating the head or the visual field representation system around the dorsoventral, lateral-lateral and anterior-posterior axes, in the mediolateral, dorsoventral and clockwise directions, respectively. In addition, using the concept of the scalar product in linear algebra, we propose new algorithms for calculating the distance between two points and to determine the area of receptive fields in the visual field.

Attentional impairment in Parkinson's disease is modulated by side of onset: Neurophysiological evidence.

OBJECTIVE: Neurophysiological studies exploring involuntary attention have reported that electroencephalographic (EEG) measures can indicate impaired neural processing from initial stages of Parkinson's disease (PD). Since involuntary attention is regulated by right hemisphere networks and PD generally initiates its motor symptomatology unilaterally, whether involuntary attention is impaired depending on the onset side of PD remains unknown. METHODS: We compared the neurophysiological correlates of involuntary attention among a PD group with left-side onset (L-PD), a PD group with right-side onset (R-PD) symptomatology, and a healthy control group (HC). All participants performed an auditory involuntary attention task while a digital EEG was recorded. RESULTS: Our main finding was a reduction both in the P3a amplitude and evoked delta-theta phase alignment in the L-PD group compared to the HC. Further, there was a significant correlation between P3a amplitude and disease duration in the R-PD, but not in the L-PD group. Behaviorally, both clinical groups, and in particular L-PD, showed reduced orientation towards novel stimuli, and no reduction of distraction effects during the experiment. CONCLUSIONS: Our results indicate that involuntary attention is differentially impaired in patients with left side onset of symptoms. Involuntary attention impairment might be present from initial stages of left onset PD and become progressively compromised in patients with right onset PD. SIGNIFICANCE: The onset side of symptomatology should be considered for attentional impairment in PD.

Las huellas del dolor y su implicancia en la construcción de la corporeidad en pacientes con dolor crónico primario desde una perspectiva psicomotriz / The traces of pain and its implication in the construction of corporeality in patients with primary chronic pain from a psychomotor perspective

El presente estudio tiene como objetivo principal describir la relación entre las primeras experiencias de seguridad del niño y el dolor crónico primerio, desde la perspectiva bio-pico-social. La justificación de esta investigación se fundamenta en la necesidad de llenar un vacío en la literatura científica en relación a este tema y la importancia de profundizar en el conocimiento teórico y descriptivo para comprender y abordar de manera más efectiva el dolor crónico en los pacientes desde un enfoque psicomotriz.

Perceived Exertion: Revisiting the History and Updating the Neurophysiology and the Practical Applications.

The perceived exertion construct creation is a landmark in exercise physiology and sport science. Obtaining perceived exertion is relatively easy, but practitioners often neglect some critical methodological issues in its assessment. Furthermore, the perceived exertion definition, neurophysiological basis, and practical applications have evolved since the perceived exertion construct's inception. Therefore, we revisit the careful work devoted by Gunnar Borg with psychophysical methods to develop the perceived exertion construct, which resulted in the creation of two scales: the rating of perceived exertion (RPE) and the category-ratio 10 (CR10). We discuss a contemporary definition that considers perceived exertion as a conscious perception of how hard, heavy, and strenuous the exercise is, according to the sense of effort to command the limbs and the feeling of heavy breathing (respiratory effort). Thus, other exercise-evoked sensations would not hinder the reported perceived exertion. We then describe the neurophysiological mechanisms involved in the perceived exertion genesis during exercise, including the influence of the peripheral feedback from the skeletal muscles and the cardiorespiratory system (i.e., afferent feedback) and the influence of efferent copies from the motor command and respiratory drive (i.e., corollary discharges), as well as the interaction between them. We highlight essential details practitioners should consider when using the RPE and CR10 scales, such as the perceived exertion definition, the original scales utilization, and the descriptors anchoring process. Finally, we present how practitioners can use perceived exertion to assess cardiorespiratory fitness, individualize exercise intensity prescription, predict endurance exercise performance, and monitor athletes' responses to physical training.

Dysautonomia in RFC1-related disorder: Clinical and neurophysiological evaluation.

OBJECTIVE: To characterize and quantify autonomic involvement in patients with RFC1-related disorder of adult-onset cerebellar ataxia and idiopathic sensory neuropathy. METHODS: We enrolled 16 subjects with biallelic RFC1 (AAGGG)n expansions and 16 age and sex-matched healthy controls that underwent comprehensive clinical and neurophysiological evaluation. Scales for Outcomes in Parkinson's Disease Autonomic Dysfunction (SCOPA-AUT) score was used to assess autonomic symptoms. Electrophysiological testing included assessment of heart rate variability and quantitative sudomotor axon reflex test (QSART). Between-group comparisons were assessed using non-parametric tests. RESULTS: In the patient group, there were 9 men/7 women and the median age was 60.5 years. SCOPA-AUT scores were significantly higher in the RFC1 group compared to controls (22 vs 10, p < 0.001). Half of patients had cardiac autonomic neuropathy. In neurophysiology, there was resting tachycardia combined with abnormal responses during Valsalva maneuver and deep breathing among patients. QSART responses were also significantly reduced in the RFC1 group, especially in the lower limbs. CONCLUSIONS: Autonomic dysfunction is frequent, clinically relevant and involves multiple domains in RFC1-related disorder. Patients have both sympathetic and parasympathetic involvement. From a topographical perspective, this condition is characterized by a small fiber autonomic axonopathy. SIGNIFICANCE: Dysautonomia is frequent, severe and related to peripheral damage in RFC1-related disorder.

Seventy years since the invention of the averaging technique in Neurophysiology: Tribute to George Duncan Dawson / 70 anos da invenção da técnica de promediação na Neurofisiologia: Tributo a George Duncan Dawson

ABSTRACT In 1951, the physiologist George Duncan Dawson presented his work with the averaging of the signal in the evoked potentials (EPs), opening a new stage in the development of clinical neurophysiology. The authors present aspects of Professor Dawson's biography and a review of his work on the EPs and, mainly, the article reveals the new technique in detail that would allow the growth of the clinical application of the visual, auditory, and somatosensory EPs.

RESUMO Em 1951 o fisiologista George Duncan Dawson apresentou seu trabalho com a promediação de sinal nos potenciais evocados, abrindo uma nova etapa no desenvolvimento da neurofisiologia clínica. Os autores apresentam aspectos da biografia do professor Dawson e uma revisão de seus trabalhos sobre os potenciais evocados, principalmente do artigo que mostrava a nova técnica, que viria a permitir o crescimento da aplicação clínica dos potenciais evocados visual, auditivo e somatossensitivo.

Peripheral polyneuropathy from electrodiagnostic tests: a 10-year etiology and neurophysiology overview.

BACKGROUND: Polyneuropathies are characterized by a symmetrical impairment of the peripheral nervous system, resulting in sensory, motor and/or autonomic deficits. Due to the heterogeneity of causes, an etiological diagnosis for polyneuropathy is challenging. OBJECTIVE: The aim of this study was to determine the main causes of polyneuropathy confirmed by electrodiagnostic (EDX) tests in a tertiary service and its neurophysiological aspects. METHODS: This observational cross-sectional study from a neuromuscular disorders center included individuals whose electrodiagnostic tests performed between 2008 and 2017 confirmed a diagnosis of polyneuropathy. Through analysis of medical records, polyneuropathies were classified according to etiology and neurophysiological aspect. RESULTS: Of the 380 included patients, 59.5% were male, with a median age of 43 years. The main etiologies were: inflammatory (23.7%), hereditary (18.9%), idiopathic (13.7%), multifactorial (11.1%), and diabetes (10.8%). The main electrophysiological patterns were axonal sensorimotor polyneuropathy (36.1%) and "demyelinating and axonal" sensorimotor polyneuropathy (27.9%). Axonal patterns showed greater etiological heterogeneity, with a predominance of idiopathic and multifactorial polyneuropathy, while demyelinating and "demyelinating and axonal" polyneuropathies had a significantly fewer etiologies, with a predominance of hereditary and inflammatory polyneuropathies. CONCLUSION: The main causes of polyneuropathy confirmed by EDX test in this study were those that presented a severe, atypical and/or rapidly progressing pattern. Other causes were hereditary and those that defy clinical reasoning, such as multiple risk factors; some polyneuropathies did not have a specific etiology. EDX tests are useful for etiological diagnosis of rare polyneuropathies, because neurophysiological patterns are correlated with specific etiologies.

Seventy years since the invention of the averaging technique in Neurophysiology: Tribute to George Duncan Dawson.

OBJECTIVE: In 1951, the physiologist George Duncan Dawson presented his work with the averaging of the signal in the evoked potentials (EPs), opening a new stage in the development of clinical neurophysiology. The authors present aspects of Professor Dawson's biography and a review of his work on the EPs and, mainly, the article reveals the new technique in detail that would allow the growth of the clinical application of the visual, auditory, and somatosensory EPs.

Neuroauriculotherapy: Framework and Remarks for a possible use in Voice Medicine / Neuroauriculoterapia: marco y observaciones para un posible uso en medicina de la voz

Aim. Neuroauriculotherapy (NAT) is a branch of medicine, which, thanks to its diagnostic and therapeutic value, is a powerful tool at the service of both physician and patient. In our experience, as it is discussed in this article, neuroauriculotherapy can have successful applications in voice science and in phoniatrics. The aim of this article is to open a discussion about possible applications of neuroauriculotherapy in voice medicine. Introduction. From the diagnostic point of view, it is possible to explore, with a palpeur (i.e., a tool that provides a constant pressure) or a "spot-hunter", the presence of a perturbation into a particular organ or area, and its return to normal during treatment. Indeed, at the level of the ear auricle, representations of an organ, its in-nervation, its muscular components, etc., are fixed. These spots or voxels correspond absolutely to the respective sensory, motor, visceral, among other spots. Therefore, if any abnormal potential comes from the periphery, it will illuminate the spots both at the cortical level and in the pavilion ­a real display with a constantly active touch screen. The spot can be treated with needles in the context of a neurophysiological strategy to send a message to the brain.Reflection. NAT appears to be a good method to improve the treatment of voice problems, enhancing the results of other therapies based on drugs or rehab and in-ducing relaxation. In neuroauriculotherapy, the ear is used to give the brain orders in a process which has a logical basis in neurophysiology.Conclusion. Diseases of the vocal tract can be dysfunctional or organic. According to our clinical experience, we can say that neuroauriculotherapy can be used in both cases. Neuroauriculotherapy is also extremely effective in voice therapy, both alone and in combination with other therapies, as there is no conflict among them

Objetivo. La neuroauriculoterapia (NAT) es una rama de la medicina que, gracias a su valor diagnóstico y terapéutico, constituye una poderosa herramienta al servicio del médico y del paciente. En nuestra experiencia, como se comenta en este artículo, la neuroauriculoterapia puede tener aplicaciones exitosas en la ciencia de la voz y en foniatría. El objetivo de este artículo es abrir una discusión sobre las posibles aplica-ciones de la neuroauriculoterapia en la medicina de la voz.Introducción. Desde el punto de vista del diagnóstico, es posible explorar, con un palpeur (i.e., una herramienta que proporciona una presión constante) o un "spot-hunter", la presencia de una perturbación en un órgano o área en particular, y su retorno a la normalidad durante el tratamiento. En efecto, a nivel del pabellón auricular se fijan representaciones de un órgano, su inervación, sus componentes musculares, etc. Estos puntos o vóxeles corresponden absolutamente a los respectivos puntos sensoriales, motores, viscerales, entre otros. Por lo tanto, si algún potencial anormal proviene de la periferia, iluminará los puntos tanto a nivel cortical como en el pabellón ­una pantalla real con una pantalla táctil constantemente activa. El punto se puede tratar con agujas en el contexto de una estrategia neurofisiológica para enviar un mensaje al cerebro.Reflexión. NAT parece ser un buen método para mejorar el tratamiento de los problemas de voz, potenciando los resultados de otras terapias basadas en fármacos o rehabilitación e induciendo a la relajación. En neuroauriculoterapia se utiliza el oído para dar órdenes al cerebro en un proceso que tiene una base lógica en la neurofisiología.Conclusión. Las enfermedades del tracto vocal pueden ser disfuncionales u orgáni-cas. Según nuestra experiencia clínica, podemos decir que la neuroauriculoterapia se puede utilizar en ambos casos. La neuroauriculoterapia también es extremadamente eficaz en la terapia de la voz, tanto sola como en combinación con otras terapias, ya que no existe conflicto entre ellas

Constantin von Economo´s 90th death anniversary.

The year of 2021 marks 90 year since the death of the neuroscientist Constantin von Economo, whose research in various areas was extremely relevant for the field of neurology. He described lethargic epidemic encephalitis, published an atlas of the cytoarchitecture of the human cerebral cortex, and conducted multiple studies in neuroanatomy, neurophysiology, and clinical neurology. Von Economo's genius extended into other nonmedical fields such as aeronautics, and he had renowned artistic skills.

Constantin von Economo´s 90th death anniversary / O 90º aniversário da morte de Constantin von Economo

ABSTRACT The year of 2021 marks 90 year since the death of the neuroscientist Constantin von Economo, whose research in various areas was extremely relevant for the field of neurology. He described lethargic epidemic encephalitis, published an atlas of the cytoarchitecture of the human cerebral cortex, and conducted multiple studies in neuroanatomy, neurophysiology, and clinical neurology. Von Economo's genius extended into other nonmedical fields such as aeronautics, and he had renowned artistic skills.

RESUMO O ano de 2021 marca o 90° aniversário da morte do neurocientista Constantin von Economo, ou Constantin Freiherr (Barão) von Economo. Von Economo realizou várias pesquisas de grande relevo na área da neurologia, com a descrição da encefalite letárgica epidêmica, a publicação do atlas sobre a citoarquitetura do córtex cerebral humano, além dos seus múltiplos estudos em neuroanatomia, neurofisiologia, bem como na neurologia clínica. A genialidade de von Economo se estendeu para outras áreas não médicas, com o seu grande interesse em aeronáutica, como piloto de avião, além dos seus reconhecidos dotes artísticos.

Neurophysiological assessment of brachioradial pruritus patients.

BACKGROUND: Pruritus is a common complaint in dermatology. Wartenberg, in 1943, associated pruritus with neuropathy, relating it to the "posterior antebrachial cutaneous nerve neuropathy". In 1968, Waisman described patients with frequent pruritus complaints in the upper limb during the summer, which he named "brachioradial summer pruritus". Currently, this pruritus is named brachioradial pruritus (BRP). BRP is characterized by a chronic pruritus, usually localized, with a long duration, and without apparent cutaneous abnormalities. Neurological disorders both from the central and peripheral nervous systems, including multiple sclerosis, are associated with pruritus. OBJECTIVE: To investigate correlations between symptomatic dermatomes and alterations in the myotomes, as evidenced by electroneuromyography (ENMG). METHODS: Forty-six patients with BRP dermatological diagnoses were subjected to upper limb ENMG. RESULTS: Among 46 patients with C5 to C8 dermatomal pruritus, we evaluated 113 symptomatic dermatomal areas. Overall, 39 (85%) patients had radicular involvement and 28 (60%) had agreement between complaint and the ENMG findings (p=0.015). A total of 80% of the patients with complaints at C7 and 47% at C6 had radicular involvement at the same level. CONCLUSIONS: Among the patients who presented complaints, 47 and 80%, respectively, had ENMG alterations in the C6 and C7 myotomes. We conclude that peripheral nervous system involvement is associated with BRP.

Adult-onset non-5q proximal spinal muscular atrophy: a comprehensive review.

BACKGROUND: Adult-onset spinal muscular atrophy (SMA) represents an expanding group of inherited neurodegenerative disorders in clinical practice. OBJECTIVE: This review aims to synthesize the main clinical, genetic, radiological, biochemical, and neurophysiological aspects related to the classical and recently described forms of proximal SMA. METHODS: The authors performed a non-systematic critical review summarizing adult-onset proximal SMA presentations. RESULTS: Previously limited to cases of SMN1-related SMA type 4 (adult form), this group has now more than 15 different clinical conditions that have in common the symmetrical and progressive compromise of lower motor neurons starting in adulthood or elderly stage. New clinical and genetic subtypes of adult-onset proximal SMA have been recognized and are currently target of wide neuroradiological, pathological, and genetic studies. CONCLUSIONS: This new complex group of rare disorders typically present with lower motor neuron disease in association with other neurological or systemic signs of impairment, which are relatively specific and typical for each genetic subtype.

Adult-onset non-5q proximal spinal muscular atrophy: a comprehensive review / Atrofia muscular espinhal não-5q proximal de início no adulto: uma revisão abrangente

Abstract Background: Adult-onset spinal muscular atrophy (SMA) represents an expanding group of inherited neurodegenerative disorders in clinical practice. Objective: This review aims to synthesize the main clinical, genetic, radiological, biochemical, and neurophysiological aspects related to the classical and recently described forms of proximal SMA. Methods: The authors performed a non-systematic critical review summarizing adult-onset proximal SMA presentations. Results: Previously limited to cases of SMN1-related SMA type 4 (adult form), this group has now more than 15 different clinical conditions that have in common the symmetrical and progressive compromise of lower motor neurons starting in adulthood or elderly stage. New clinical and genetic subtypes of adult-onset proximal SMA have been recognized and are currently target of wide neuroradiological, pathological, and genetic studies. Conclusions: This new complex group of rare disorders typically present with lower motor neuron disease in association with other neurological or systemic signs of impairment, which are relatively specific and typical for each genetic subtype.

RESUMO Antecedentes: Atrofia muscular espinhal (AME) de início no adulto representa um grupo de doenças neurodegenerativas hereditárias em expansão na prática clínica. Objetivo: Este artigo de revisão sintetiza os principais aspectos clínicos, genéticos, radiológicos, bioquímicos e neurofisiológicos relacionados às formas clássicas e recentemente descritas de AME proximal do adulto. Métodos: Os autores realizaram uma revisão crítica não sistemática descrevendo as principais apresentações de AME proximal de início no adulto. Resultados: Previamente restrito às apresentações de AME tipo 4 associada ao gene SMN1, este grupo atualmente envolve mais de 15 diferentes condições clínicas que compartilham entre si a presença de comprometimento progressivo e simétrico do neurônio motor inferior se iniciando no adulto ou no idoso. Novos subtipos clínicos e genéticos de AME proximal de início no adulto foram reconhecidas e são alvos atuais de estudos direcionados a aspectos neurorradiológicos, patológicos e genéticos. Conclusões: Este novo grupo complexo de doenças raras tipicamente se apresenta com doença do neurônio motor inferior em associação com outros sinais de comprometimento neurológico ou sistêmico, os quais apresentam padrões relativamente específicos para cada subtipo genético.

Evaluación del desarrollo de la asignatura virtual Fisiología II en la especialidad Neurofisiología Clínica / Assessment of developing Physiology II as a virtual subject in the specialty of Clinical Neurophysiology

Introducción: La enseñanza mediante la modalidad virtual se adoptó como medida preventiva ante la propagación de la COVID-19. Para la educación médica cubana ha implicado un reto en cuanto a la reorganización de los programas de estudio. Con este propósito, se rediseño e implementó la asignatura Fisiología II en modalidad virtual, para garantizar la continuidad en la formación de residentes en Neurofisiología Clínica. Objetivo: Evaluar el desarrollo de la asignatura Fisiología II en su modalidad virtual desde la perspectiva de los estudiantes de la especialidad Neurofisiología Clínica. Métodos: Estudio exploratorio, no experimental y transversal. Mediante un cuestionario de preguntas cerradas y una abierta (aspectos positivos y negativos), se evaluó el desarrollo de la asignatura en modalidad virtual en relación con las variables: programa de la asignatura, desempeño de los profesores, escenario de las plataformas y soporte tecnológico. Resultados: La asignatura Fisiología II en modalidad virtual tuvo una alta aceptación por parte de los estudiantes. Los aspectos identificados como positivos fueron los relacionados con la gestión personal del tiempo, y la aplicabilidad y estructura de la asignatura; mientras que los negativos se orientaron hacia problemas de conectividad de internet y el entorno de estudio. Conclusiones: Las exitosas experiencias obtenidas en el desarrollo de la asignatura virtual Fisiología II sientan las bases para el empleo de este tipo de diseño en la especialidad de Neurofisiología Clínica y la posibilidad de extenderlo a otras asignaturas del plan de estudio(AU)

Introduction: Teaching through virtual modality was adopted as a preventive measure against the COVID-19 spread. For Cuban medical education, it has implied a challenge regarding the reorganization of study programs. For this purpose, the subject Physiology II was redesigned and implemented in virtual modality, in order to guarantee continuity in the training of Clinical Neurophysiology residents. Objective: To assess the development of the subject Physiology II in its virtual modality from the perspective of the students of the Clinical Neurophysiology specialty. Methods: Exploratory, nonexperimental and cross-sectional study. Through a questionnaire made up of closed questions and one open question (positive and negative aspects), the development of the subject in virtual modality was assessed in relation to the variables subject syllabus, professors' performance, platform scenario, and technological support. Results: The subject Physiology II in virtual modality had a high acceptance by the students. The aspects identified as positive were those related to individual time management, as well as the subject's applicability and structure; while the negative ones were oriented towards internet connectivity problems and the study environment. Conclusions: The successful experiences obtained in the development of Physiology II as a virtual subject lay the foundations for using this type of design in the specialty of Clinical Neurophysiology and the possibility of extending it to other subjects of the study plan(AU)

Report on the online course "Basic Concepts in Neurophysiology": a course promoted during the COVID-19 pandemic quarantine.

"Basic Concepts in Neurophysiology" was a 3-wk online course developed during six synchronous meetings combined with asynchronous activities. We proposed an active learning course that used free online platforms to teach physiology during a period in which undergraduates were not in classrooms or taking online classes due to the COVID-19 pandemic. Herein, we report the course organization and the students' involvement in, acceptance of, and evaluation of the course. To address the students' perceptions about these points, we sent a questionnaire to 49 participants who finished the course. We found that although most students (52.5%) had never taken a course with similar methods before, almost all of them (95%) liked the flipped class model. Additionally, a majority of the students (92.5%) said that the method increased their study frequency during the social distancing period, which is an important aspect to consider during this challenging time for both students and professors.