Consideraciones bioéticas en el manejo de la esclerosis lateral amiotrófica: Una aproximación al quehacer fonoaudiológico

Introducción: La esclerosis lateral amiotrófica (ELA) es la forma más común de enfermedad degenerativa de motoneurona en la edad adulta y es considerada una enfermedad terminal. Por lo mismo, el accionar del fonoaudiólogo debe considerar el respeto a los principios bioéticos básicos para garantizar una asistencia adecuada. Objetivo: Conocer aquellas consideraciones bioéticas relacionadas al manejo y estudio de personas con ELA para luego brindar una aproximación hacia el quehacer fonoaudiológico. Método: Se efectuó una búsqueda bibliográfica en las bases de datos PubMed, Scopus y SciELO. Se filtraron artículos publicados desde 2000 hasta junio de 2023 y fueron seleccionados aquellos que abordaban algún componente bioético en población con ELA. Resultados: Aspectos relacionados al uso del consentimiento informado y a la toma de decisiones compartidas destacaron como elementos esenciales para apoyar la autonomía de las personas. Conclusión: Una correcta comunicación y una toma de decisiones compartida son claves para respetar la autonomía de las personas. A su vez, la estandarización de procedimientos mediante la investigación clínica permitirá aportar al cumplimiento de los principios bioéticos de beneficencia y no maleficencia, indispensables para la práctica profesional.

Introduction: Amyotrophic lateral sclerosis (ALS) is the most common form of degenerative motor neuron disease in adulthood and is considered a terminal disease. For this reason, the actions of the speech therapist must consider respect for basic bioethical principles to guarantee adequate assistance. Objective: To know those bioethical considerations related to the management and study of people with ALS to then provide an approach to speech therapy. Methodology: A bibliographic search was carried out in the PubMed, Scopus, and SciELO databases. Articles published from 2000 to June 2023 were filtered and those that addressed a bioethical component in the population with ALS were selected. Results: Aspects related to the use of informed consent and shared decision-making stood out as essential elements to support people's autonomy. Conclusion: Proper communication and shared decision-making are key to respecting people's autonomy. In turn, the standardization of procedures through clinical research will contribute to compliance with the bioethical principles of beneficence and non-maleficence, essential for professional practice.

Molecular identification of Sarcocystis neurona in tissues of wild boars (Sus scrofa) in the border region between Brazil and Uruguay.

Sarcocystis neurona, owing to its clinical importance in domestic animals, is currently one of the most studied agents, presenting a wide range of intermediate hosts that have not yet been described, mainly in wild fauna. Thus, the aim of this study was to describe the detection and molecular detection of S. neurona by amplification of the 18S rRNA region in the tissues of wild boars killed by boar control program in border Brazil Uruguay. A total of 79 samples of DNA from wild boar tissues from the LADOPAR/UFSM sampling bank were used, with Nested-PCR reactions being performed for amplification of the 18S rRNA region and the expected final product of 290 bp. Subsequently, the positive samples were subjected to restriction fragment length polymorphism (RFLP) technique with the restriction enzymes DdeI and HPAII. A second semi-Nested reaction was performed to obtain a larger sequence of nucleotides with amplification of the 18S region and the expected final product of 500 bp for S. neurona and Nested amplification ITS1 with product final of 367 pb. In 32 samples, it was possible to detect S. neurona both by nested Nested-PCR reaction and RFLP, and the presence of the agent was confirmed by sequencing, corresponding to 40.51% of the total tissues evaluated. This is the first report of the occurrence of this species of Sarcocystis in wild boars, and further studies evaluating the role of these animals as intermediate hosts, and in the epidemiology of this protozoan are necessary, as well as verifying the risk factors for infection.

Prescription patterns of riluzole in a population of patients with motor neuron disease / Patrón de prescripción de riluzol en una población de pacientes con enfermedad de neurona motora

SUMMARY OBJECTIVE: To determine the prescription pattern of riluzol and the variables associated to its use in a population of patients with motor neuron disease affiliated to the Colombian General Social Security Health System (SGSSS) in 2017. METHOD: Descriptive cross-sectional study. Through a systemized data base of approximately 3,5 million members to the Colombian SGSSS; patients who had been given riluzol uninterruptedly between April 1 and June 30 of 2017, were selected. Sociodemographic, pharmacological variables and comorbidities were analyzed. Defined daily dose (DDD) was estimated for 1.000 inhabitants/day and its costs. RESULTS: There were found 81 patients with motor neuron disease receiving riluzol, with an average age of 60,8+12,6 years. 48.1% were male. The prevalence of motor neuron disease was 29/100.000 individuals. Patients received riluzol in 50 mg tablets and the doses was estimated in 0,016 DDD for 1.000 inhabitants/day 63% were receiving medicines that reflect comorbidity or could interact with riluzol. The total cost of riluzol dispensed in 2017 was USD 85.348 and per prescribed daily dose on average was USD 2,3. CONCLUSIONS: The use of riluzol in patients with motor neuron disease in Colombia was carried by the recommended doses by the WHO and with a direct cost lower than reported in other countries. Studies are recommended in order to determine the effectiveness of riluzol in real-life conditions.

RESUMEN OBJETIVOS: Determinar el patrón de prescripción de riluzol y las variables asociadas a su utilización en una población de pacientes con enfermedad de neurona motora afiliados al Sistema General de Seguridad Social en Salud de Colombia (SGSSS) en 2017. METODOLOGÍA: Estudio descriptivo de corte transversal. Mediante una base de datos sistematizada de aproximadamente 3,5 millones de afiliados al SGSSS de Colombia; se seleccionaron pacientes a quienes se les haya dispensado riluzol de manera ininterrumpida entre 1 abril y 30 junio de 2017. Se analizaron variables socio-demográficas, farmacológicas y las comorbilidades. Se estimaron la dosis diaria definida (DDD) por 1.000 habitantes/día y los costos. RESULTADOS: Se encontraron 81 pacientes con enfermedad de neurona motora recibiendo riluzol, con edad promedio de 60,8+12,6 años. El 48,1% eran hombres. La prevalencia de enfermedad de neurona motora fue 2,29/100.000 personas. Los pacientes recibieron riluzol en tabletas de 50 mg y se estimó la dosis en 0,016 DDD por 1.000 habitantes/día. El 63% recibían medicamentos que reflejan comorbilidad o pudieran tener interacción con riluzol. El costo total del riluzol dispensado en 2017 fue USD 85.348 y por dosis diaria prescrita en promedio fue USD 2,3. CONCLUSIONES: El uso de riluzol en pacientes con enfermedad de neurona motora en Colombia se realizó a las dosis recomendadas por la OMS y con un costo directo menor al reportado en otros países. Se recomienda realizar estudios que permitan determinar la efectividad del riluzol en condiciones de la vida real.

La enfermedad neurológica de Leonardo da Vinci / The neurological disease of Leonardo da Vinci

Introducción: el año 2019 se conmemoraron los 500 años de la muerte de Leonardo da Vinci en el Chateux du Cloux, Francia. Según registros históricos, Leonardo vivió los últimos años de su vida con una parálisis en su mano derecha, atribuida a un probable accidente cerebrovascular (ACV), lo cual afectó a su capacidad para emprender nuevos proyectos pictóricos. Este trabajo describe como fueron los últimos años de vida de Leonardo, revisa los antecedentes sobre salud y su probable enfermedad neurológica, sus eventuales etio-logías y sus consecuencias. Desarrollo: Leonardo llegó a Francia en 1516 por invitación del rey Francisco I para llevar el Renacimiento y completar su formación personal. En 1517 es visitado por el cardenal Luis de Aragón, y su secretario registra en su diario que Leonardo se encuentra afectado de una parálisis de la mano derecha. Algunos autores sugieren un ACV como causa probable, sobretodo porque según el historiador Vasari, Leonardo vivió sus últimos años en condición de discapacidad. Se llega a plantear que su condición de vege-tariano habría podido influir como factor de riesgo de ACV. Sin embargo, un dibujo no fechado de Figino muestra a Leonardo con una parálisis de tipo periférica de la mano derecha, abriendo el diagnóstico diferencial. Conclusiones: no hay información suficiente para valorar el tipo y la causa de enfermedad neurológica de Leonardo da Vinci, no obstante, está influyó significativamente en su interacción con su discípulo Francisco Melzi y su mecenas Francisco I, hecho que contribuyó a que su legado perdure hasta hoy.

Introduction: the year2019 marked the 500th anniversary of the death of Leonardo da Vinci at the Chateau du Cloux, France. According to historical records, Leonardo lived the last years of his life with palsy on his right hand, attributed to a probable stroke, which affected his ability to undertake new pictorial projects. This study describes how were the last years of Leonardo's life, reviews his medical history and his probable neurological disease, with its possible etiologies and its consequences. Discussion: Leonardo arrived in France in 1516 invited by King Francis I to lead the Renaissance and complete his education. In 1517, cardinal Luis de Aragón visited Leonardo, and his secretary recorded in his diary that he was affected by a palsy on his right hand. Some authors suggest that stroke could have been a probable cause, especially because according to the historian Vasari, Leonardo lived his last years in a condition of disability. His vegetarian food habit could have been a risk factor for his suspected stroke. However, an undated drawing by Figino shows Leonardo with a peripheral nerve palsy on his right hand, opening up the differential diagnosis. Conclusions:there is not enough information to assess the type and cause of Leonardo da Vinci's neurological disease. However, this condition significantly affected his interaction with his discipleFrancisco Melzi and his patron Francisco I, a fact that contributed to his legacy enduring until today.

Natural Occurring Muscular Sarcocysts in Urban Domestic Cats (Felis catus) Without Sarcocystis-Associated Disease.

PURPOSE: Despite of classically acting as definitive hosts of different Sarcocystis species, domestic cats have been pointed out as possible intermediate hosts of S. neurona and S. felis. Nonetheless, details concerning natural sarcocyst development in cats without Sarcocystis-associated disease are scarce. This study aimed to characterize the natural occurrence of muscular sarcocysts in a random group of cats submitted for necropsy. METHODS: One hundred cats necropsied at a Veterinary Pathology Service were included. Nine different muscular tissues from each cat were sampled for histological analysis and Polymerase Chain Reaction (PCR) using multispecies primers for Sarcocystis neurona, Neospora caninum and Toxoplasma gondii. PCR-positive cases were sequenced for genus and species identification. Epidemiologic data was also analyzed. RESULTS: Tissue sarcocysts were identified in hematoxylin and eosin-stained slides from five cats, and S. neurona was the only confirmed species. Multifocal sarcocysts affecting two or more muscles were common among positive cats (4/5). Sarcocysts were identified within vastus lateralis (3/5), intercostal (3/5), subscapular (2/5) and diaphragm (2/5) sections. These cysts were always incidental necropsy findings. All sarcocyst-positive cats were from urban areas, among which two were feral and three were pets. Outdoor access was consistently reported. Two cats were positive for retrovirosis, and treatments with potentially immunosuppressive drugs were never stated. CONCLUSIONS: This study describes the natural occurrence of S. neurona muscular sarcocysts in a random group of cats without Sarcocystis-associated disease. These findings reinforce the participation of feral and pet cats from urban areas as natural intermediate hosts of S. neurona.

Mieloencefalite protozoária equina: relato de caso / Equine protozoal myeloencephalitis: case report

A Mieloencefalite Protozoária Equina (EPM) é uma doença infecciosa que possui como principal agente o protozoário Sarcocystis neurona. O parasita acomete o sistema nervoso central de equinos podendo causar alterações neurológicas como ataxia, paresia, atrofia muscular e alterações de estado mental. Com ampla distribuição no território nacional, é importante que esta afecção faça parte do diagnóstico diferencial em animais da espécie equina, uma vez que equinos portadores desta afecção podem apresentar desde sinais discretos, como ligeira queda no desempenho, a graves sinais neurológicos. Para isso, o médico veterinário deve compreender suas características e os métodos diagnósticos eficazes para sua identificação. Uma égua da raça Brasileiro de Hipismo, de cinco anos de idade, foi atendida na cidade de Belo Horizonte, em Minas Gerais, com histórico de atrofia na região lombar e garupa. Ainda, foi relatada a presença de gambás na fazenda em que vivia o animal. No exame neurológico foi detectada incoordenação motora de grau 2. O teste SAG ELISA foi realizado, e obtido resultado positivo. Todos os achados e o resultado positivo no teste corroboram o diagnóstico da EPM, o que levou ao tratamento preconizado, e a visível melhora clínica. O objetivo desse trabalho é relatar um caso clínico de EPM em uma égua no município de Belo Horizonte, Minas Gerais, Brasil, levando em consideração a importância do conhecimento dos aspectos epidemiológicos, clínicos, diagnósticos e terapêuticos da doença.(AU)

Equine Protozoal Myeloencephalitis (EPM) is an infectious disease whose main agent is the protozoan Sarcocystis neurona. The parasite affects the central nervous system of horses and can cause neurological alterations like ataxia, paresis, muscle atrophy and changes in mental status. With wide distribution in the national territory, it is important that this disease is part of the differential diagnosis in animals of equine species, since equines with this disease can present from discrete signs, such as a slight decrease in performance, to serious neurological signs. That is why the veterinarian must understand its characteristics and effective diagnostic methods for its identification. A five-year-old Brazilian Sport Horse mare was attended in the city of Belo Horizonte, Minas Gerais, with a history of atrophy in the lumbar region and rump. Also, the presence of opossums on the farm where the animal lived was reported. In neurological examination, grade 2 motor incoordination was detected. The SAG ELISA test was performed, and a positive result was obtained. All findings and the positive test result corroborate the diagnosis of EPM, which led to the recommended treatment, and visible clinical improvement. The aim of this paper is to report a clinical case of EPM in a mare in the city of Belo Horizonte, Minas Gerais, Brazil, taking into account the importance of knowledge of the epidemiological, clinical, diagnostic and therapeutic aspects of disease.(AU)

Sarcocystis neurona and related Sarcocystis spp. shed by opossums (Didelphis spp.) in South America / Sarcocystis neurona e Sarcocystis spp. relacionados, excretados por gambás (Didelphis spp.) na América do Sul

Protozoan parasites of the genus Sarcocystis are obligatory heteroxenous cyst-forming coccidia that infect a wide variety of animals and encompass approximately 200 described species. At least four Sarcocystis spp. (S. falcatula, S. neurona, S. lindsayi and S. speeri) use opossums (Didelphis spp.) as definitive hosts, and two of them, S. neurona and S. falcatula, are known to cause disease in horses and birds, respectively. Opossums are restricted to the Americas, but their distribution in the Americas is heterogeneous. Five Didelphis spp. are distributed in South America (D. aurita, D. albiventris, D. marsupialis, D. imperfecta and D. pernigra) whereas just one opossum species (D. virginiana) is found in North America. Studies conducted in the last decades show that Sarcocystis spp., derived from South American Didelphis spp., have biological and genetic differences in relation to Sarcocystis spp. shed by the North American opossum D. virginiana. The aim of this review was to address the peculiar scenario of Sarcocystis species shed by South American opossums, with a special focus on diagnosis, epidemiology, and animal infections, as well as the genetic characteristics of these parasites.(AU)

Parasitos protozoários do gênero Sarcocystis são coccídios heteroxenos formadores de cistos, que infectam variadas espécies animais e compreendem cerca de 200 espécies descritas. Pelo menos quatro Sarcocystis spp. (S. falcatula, S. neurona, S. lindsayi e S. speeri) utilizam gambás (Didelphis spp.) como hospedeiros definitivos; e duas delas, S. neurona and S. falcatula são conhecidas por causarem doença em equinos e aves, respectivamente. Gambás didelfídeos são restritos ao continente americano, contudo são distribuídos de forma heterogênea nas Américas. Cinco Didelphis spp. são distribuídos na América do Sul (D. aurita, D. albiventris, D. marsupialis, D. imperfecta e D. pernigra), enquanto somente uma espécie (D. virginiana) é encontrada na América do Norte. Trabalhos conduzidos, nas últimas décadas, mostram que Sarcocystis spp. derivados de Didelphis spp. sul-americanos possuem diferenças biológicas e genéticas, quando comparados a Sarcocystis spp. excretados pelo gambá norte-americano D. virginiana. O objetivo desta revisão é discutir a situação peculiar das espécies de Sarcocystis na América do Sul com um foco especial em diagnóstico, epidemiologia e infecções animais, assim como nas características genéticas desses parasitos.(AU)

Sarcocystis neurona and related Sarcocystis spp. shed by opossums (Didelphis spp.) in South America / Sarcocystis neurona e Sarcocystis spp. relacionados, excretados por gambás (Didelphis spp.) na América do Sul

Abstract Protozoan parasites of the genus Sarcocystis are obligatory heteroxenous cyst-forming coccidia that infect a wide variety of animals and encompass approximately 200 described species. At least four Sarcocystis spp. (S. falcatula, S. neurona, S. lindsayi and S. speeri) use opossums (Didelphis spp.) as definitive hosts, and two of them, S. neurona and S. falcatula, are known to cause disease in horses and birds, respectively. Opossums are restricted to the Americas, but their distribution in the Americas is heterogeneous. Five Didelphis spp. are distributed in South America (D. aurita, D. albiventris, D. marsupialis, D. imperfecta and D. pernigra) whereas just one opossum species (D. virginiana) is found in North America. Studies conducted in the last decades show that Sarcocystis spp., derived from South American Didelphis spp., have biological and genetic differences in relation to Sarcocystis spp. shed by the North American opossum D. virginiana. The aim of this review was to address the peculiar scenario of Sarcocystis species shed by South American opossums, with a special focus on diagnosis, epidemiology, and animal infections, as well as the genetic characteristics of these parasites.

Resumo Parasitos protozoários do gênero Sarcocystis são coccídios heteroxenos formadores de cistos, que infectam variadas espécies animais e compreendem cerca de 200 espécies descritas. Pelo menos quatro Sarcocystis spp. (S. falcatula, S. neurona, S. lindsayi e S. speeri) utilizam gambás (Didelphis spp.) como hospedeiros definitivos; e duas delas, S. neurona and S. falcatula são conhecidas por causarem doença em equinos e aves, respectivamente. Gambás didelfídeos são restritos ao continente americano, contudo são distribuídos de forma heterogênea nas Américas. Cinco Didelphis spp. são distribuídos na América do Sul (D. aurita, D. albiventris, D. marsupialis, D. imperfecta e D. pernigra), enquanto somente uma espécie (D. virginiana) é encontrada na América do Norte. Trabalhos conduzidos, nas últimas décadas, mostram que Sarcocystis spp. derivados de Didelphis spp. sul-americanos possuem diferenças biológicas e genéticas, quando comparados a Sarcocystis spp. excretados pelo gambá norte-americano D. virginiana. O objetivo desta revisão é discutir a situação peculiar das espécies de Sarcocystis na América do Sul com um foco especial em diagnóstico, epidemiologia e infecções animais, assim como nas características genéticas desses parasitos.

Mieloencefalite protozoária equina: relato de caso / Equine protozoal myeloencephalitis: case report

A Mieloencefalite Protozoária Equina (EPM) é uma doença infecciosa que possui como principal agente o protozoário Sarcocystis neurona. O parasita acomete o sistema nervoso central de equinos podendo causar alterações neurológicas como ataxia, paresia, atrofia muscular e alterações de estado mental. Com ampla distribuição no território nacional, é importante que esta afecção faça parte do diagnóstico diferencial em animais da espécie equina, uma vez que equinos portadores desta afecção podem apresentar desde sinais discretos, como ligeira queda no desempenho, a graves sinais neurológicos. Para isso, o médico veterinário deve compreender suas características e os métodos diagnósticos eficazes para sua identificação. Uma égua da raça Brasileiro de Hipismo, de cinco anos de idade, foi atendida na cidade de Belo Horizonte, em Minas Gerais, com histórico de atrofia na região lombar e garupa. Ainda, foi relatada a presença de gambás na fazenda em que vivia o animal. No exame neurológico foi detectada incoordenação motora de grau 2. O teste SAG ELISA foi realizado, e obtido resultado positivo. Todos os achados e o resultado positivo no teste corroboram o diagnóstico da EPM, o que levou ao tratamento preconizado, e a visível melhora clínica. O objetivo desse trabalho é relatar um caso clínico de EPM em uma égua no município de Belo Horizonte, Minas Gerais, Brasil, levando em consideração a importância do conhecimento dos aspectos epidemiológicos, clínicos, diagnósticos e terapêuticos da doença.

Equine Protozoal Myeloencephalitis (EPM) is an infectious disease whose main agent is the protozoan Sarcocystis neurona. The parasite affects the central nervous system of horses and can cause neurological alterations like ataxia, paresis, muscle atrophy and changes in mental status. With wide distribution in the national territory, it is important that this disease is part of the differential diagnosis in animals of equine species, since equines with this disease can present from discrete signs, such as a slight decrease in performance, to serious neurological signs. That is why the veterinarian must understand its characteristics and effective diagnostic methods for its identification. A five-year-old Brazilian Sport Horse mare was attended in the city of Belo Horizonte, Minas Gerais, with a history of atrophy in the lumbar region and rump. Also, the presence of opossums on the farm where the animal lived was reported. In neurological examination, grade 2 motor incoordination was detected. The SAG ELISA test was performed, and a positive result was obtained. All findings and the positive test result corroborate the diagnosis of EPM, which led to the recommended treatment, and visible clinical improvement. The aim of this paper is to report a clinical case of EPM in a mare in the city of Belo Horizonte, Minas Gerais, Brazil, taking into account the importance of knowledge of the epidemiological, clinical, diagnostic and therapeutic aspects of disease.

Pathological, immunohistochemical, and molecular findings of equine protozoal myeloencephalitis due to Sarcocystis neurona infection in Brazilian horses.

Equine protozoal myeloencephalitis (EPM) is an important neurologic disease of horses in the American continent caused by Sarcocystis neurona and Neospora hughesi infection. This study describes the pathological, immunohistochemical, and molecular findings of fatal cases of EPM in southern Brazil. A review was performed on a total of 13 cases compatible with EPM, which were diagnosed by postmortem examination in the period of 2010-2017. Epidemiological information was obtained from necropsy reports. Gross and histological lesions were characterized, and cases were subjected to immunohistochemistry anti-Sarcocystis neurona, Toxoplasma gondii, and Neospora spp. Molecular search was performed using ITS-1 gene PCRs. Microscopic lesions were multifocal in all cases, and more frequently observed in the spinal cord segments and in the rhombencephalon. Intralesional protozoans were histologically detected in five horses, while a positive immunostaining for S. neurona was observed in eleven cases (11/13). Through molecular techniques, six positive cases for the ITS-1 gene were detected, and obtained sequences presented highest similarity with S. neurona. EPM due to S. neurona infection represents an important neurologic disease of horses in Brazil and this disease should be considered as a main differential diagnosis in horses presenting neurologic signs.

Rehabilitación robótica en espasticidad de mano y muñeca / Robotic rehabilitation in hand and wrist spasticity

Introducción. El presente estudio es una serie de casos clínicos que describe los resultados en el tratamiento de la espasticidad de mano y muñeca mediante órtesis robóticas realizado por el grupo de investigación F-Ciber-Rehabilitación en Manizales (Colombia). Materiales y métodos. Se seleccionaron cinco (5) pacientes con espasticidad de mano y muñeca secundaria a lesión de neurona motora superior de diferente etiología, a quienes se les practicó terapia física y movilización pasiva con la órtesis robótica PRO-DWix®. Resultados. Los pacientes presentaron mejoría de la espasticidad según la escala Ashworth Modificada, disminución de la intensidad del dolor según la escala EVA y recuperación gradual del arco de movilidad articular según goniometría; además, luego de tres meses de rehabilitación robótica presentaron beneficios en su calidad de vida. Discusión. Se requieren ensayos clínicos aleatorizados para determinar las ventajas de la terapia física convencional complementada con terapia robótica, frente a la sola terapia convencional. Conclusiones. La movilización pasiva con órtesis robóticas en pacientes con espasticidad de mano y muñeca por lesiones de neurona motora superior, favorece la modulación de la espasticidad, la disminución del dolor, la recuperación del arco de movilidad articular y la calidad de vida. Nivel de evidencia. El propósito del estudio es generar evidencia clínica de nivel III respecto de las ventajas de la movilización pasiva con órtesis robóticas en la rehabilitación de pacientes con espasticidad de mano y muñeca

Introduction. The present study is a clinical case series describing the results in the treatment of hand and wrist spasticity by means of robotic orthoses carried out by the F-Ciber-Rehabilitation research group in Manizales (Colombia). Materials and methods. Five (5) patients with hand and wrist spasticity secondary to upper motor neuron lesion of different etiology were selected and underwent physical therapy and passive mobilization with the PRO-DWix® robotic orthosis. Results. The patients presented improvement of spasticity according to the Modified Ashworth scale, decrease of pain intensity according to the VAS scale and gradual recovery of joint mobility arc according to goniometry; furthermore, after three months of robotic rehabilitation they presented benefits in their quality of life. Discussion. Randomized clinical trials are required to determine the advantages of conventional physical therapy supplemented with robotic therapy, versus conventional therapy alone. Conclusions. Passive mobilization with robotic orthoses in patients with hand and wrist spasticity due to upper motor neuron lesions favors spasticity modulation, pain reduction, recovery of joint range of motion, and quality of life. Level of evidence. The purpose of the study is to generate level III clinical evidence regarding the advantages of passive mobilization with robotic orthoses in the rehabilitation of patients with hand and wrist spasticity.

Aproximación genética en la esclerosis lateral amiotrófica.

The superoxide dismutase type 1 (SOD1) gene is the first responsible gene mapped in amyotrophic lateral sclerosis type 1 (ALS1), and it codes for the enzyme SOD1, the function of which is to protect against damage mediated by free radicals deriving from oxygen. Its pathophysiological mechanism in ALS1 is related to ischemia. Several molecular studies of the SOD1 gene show that point mutations are the most frequent. The most common mutations in familial cases are p.A4V, p.I113Y, p.G37R, p.D90A and p.E100G, which account for more than 80% of cases, although intronic mutations have also been described as responsible for ALS1. Sporadic cases are explained by mutations in other genes such as SETX and C9orf72. ALS1 is a complex disease with genetic heterogeneity. On the other hand, familial and sporadic cases have a different etiology, which is explained by molecular heterogeneity and multiple pathogenic mechanisms that lead to ALS1; oxidative stress and ischemia are not the only cause. In Mexico, ALS molecular genetics studies are scarce. Clinical studies show an increase in cytokines such as adipsin in cerebrospinal fluid.

El gen SOD1 es el primer gen responsable mapeado en la esclerosis lateral amiotrófica tipo 1 (ELA1) y codifica para la enzima superóxido dismutasa tipo 1 (SOD1), cuya función es proteger del daño mediado de los radicales libres derivados del oxígeno; su mecanismo fisiopatológico en ELA1 se relaciona con isquemia. Diversos estudios moleculares del gen SOD1 muestran que las mutaciones puntuales son las más frecuentes. Las mutaciones más comunes en los casos familiares son p.A4V, p.I113Y, p.G37R, p.D90A y p.E100G, que explican más de 80 % de los casos, aunque también se han descrito mutaciones intrónicas como responsables de esclerosis lateral amiotrófica tipo 1. Los casos esporádicos se explican por mutaciones en otros genes como SETX y C9orf72. ELA1 es una enfermedad compleja con heterogeneidad genética. Por otra parte, los casos familiares y esporádicos tienen etiología distinta, lo cual se explica por la heterogeneidad molecular y múltiples mecanismos patogénicos que conducen a ELA1; el estrés oxidativo y la isquemia no son la única causa. En México son escasos los estudios de genética molecular de esclerosis lateral amiotrófica. Los estudios clínicos muestran incremento de citocinas como la adipsina en el líquido cefalorraquídeo.

Enfermedad de motoneurona inferior asociada al signo ojos de serpiente: una nueva entidad clínico-radiológica / Lower motor neuron disease with snake eyes sign: a new clinical-radiological entity

Las enfermedades de la moto neurona inferior constituyen un grupo heterogéneo de entidades con pronósticos diferentes. El signo radiológico "ojos de serpiente" hace referencia a la hiperintensidad bilateral en el asta anterior de la médula espinal en los cortes axiales de las imágenes por resonancia magnética, generalmente asociada a afecciones como infartos espinales, amiotrofia espondilótica, enfermedad de Hirayama y esclerosis lateral amiotrófica. Se ha descripto recientemente un reducido número de casos de enfermedad de moto neurona inferior asociada a "ojos de serpiente" con características clínicas y pronóstico distintivos. Presentamos dos casos de enfermedad de moto neurona inferior asociada al signo "ojos de serpiente" en pacientes jóvenes. El curso clínico fue progresivo con períodos prolongados de estabilidad clínica y ausencia de compromiso de moto neurona superior durante la evolución. Los pacientes presentaron debilidad segmentaria y asimétrica de miembros superiores con predominio distal en el primer caso y proximal en el segundo. Los casos presentados se corresponden en gran parte con lo comunicado en la literatura, dando apoyo a la existencia de una nueva entidad con pronóstico relativamente benigno denominada enfermedad de moto neurona inferior esporádica con signo de ojos de serpiente en las imágenes por resonancia magnética y cuya identificación debiera obviar tratamientos innecesarios (AU)

Lower motor neuron diseases are a heterogeneous group of entities with different prognosis. The "snake eyes" sign refers to bilateral hyper intensity of the anterior horns on axial magnetic resonance imaging of the spinal cord. It has been associated with ischemia, cervical spondylosis, Hirayama disease, and amyotrophic lateral sclerosis. Recently, a small number of cases of lower motor neuron disease associated with the "snake eyes" sign have been described as having distinctive clinical manifestations and prognosis. Two young patients with "snake eyes" sign associated with lower motor neuron disease had a progressive initial course followed by a stabilization of symptoms without involvement of upper motor neuron. They presented with asymmetric segmental arm weakness with distal predominance in the first a case and proximal predominance in the second. These cases match with those reported in the literature giving support to lower motor neuron disease with "snake eyes" as a pathological entity with a relatively good prognosis. This diagnosis should avoid unnecessary treatments (AU)

Mieloencefalite protozoária equina

A mieloencefalite protozoária eqüina é uma doença que acomete o sistema nervoso central (SNC) causando uma infecção. O principal agente etiológico é o Sarcocystis neurona e tem como hospedeiro definitivo o Didelphis albiventris (gambá). Há predominância em alguns sinais clínicos como ataxia e paresia. A rápida avaliação do animal e o rápido diagnóstico podem diminuir algumas complicações e diminuir os sinais clínicos presentes nele.(AU)

Equine protozoal myeloencephalitis is a disease that affects the central nervous system (CNS) causing an infection. The main etiological agent is Sarcocystis neuronal and has its definitive host Didelphis albiventris (opossum). There is a predominance of some clinical signs such as ataxia and paresis. Rapid assessment of the animal and rapid diagnosis may decrease some complications and decrease the clinical signs present in it.(AU)

Mieloencefalite equina por protozoario (MEP): revisão de literatura / Equine protozoal myeloencephalitis (EPM): literature review

A mieloencefalite protozoária equina (MEP) é uma doença infecciosa causada principalmente pelo protozoário Sarcocystis neurona que produz disfunção neurológica no cérebro e na medula, porém pode ser causada também pelo Neospora caninum e Neospora huguesi. O equino é o hospedeiro acidental e o vetor do protozoário é o gambá (hospedeiros definitivos). Um exame é o teste imunológico do líquor, que proporciona um diagnóstico preciso. No tratamento é utilizado coccidioestatico, antiinflamatório, vitaminas e analgésicos, e evitar que o hospedeiro definitivo tenha acesso aos alimentos dos equinos. O objetivo do presente trabalho foi desenvolver uma revisão de literatura sobre mieloencefalite protozoária equina.(AU)

Equine protozoal myeloencephalitis (EPM) is an infectious disease caused mainly by the protozoan Sarcocystis neuron that produces neurological dysfunction in the brain and marrow, but can also be caused by Neospora caninum and Neospora huguesi. The equine is the accidental host and the vector of the protozoan is the opossum (definitive hosts). An examination is the CSF test, which provides an accurate diagnosis. In the treatment coccidiostatic, anti-inflammatory, vitamins and analgesics are used, and to avoid that the definitive host has access to the food of the horses. The objective of the present work was to develop a literature review on equine protozoal myeloencephalitis.(AU)

Mieloencefalite equina por protozoario (MEP): revisão de literatura / Equine protozoal myeloencephalitis (EPM): literature review

A mieloencefalite protozoária equina (MEP) é uma doença infecciosa causada principalmente pelo protozoário Sarcocystis neurona que produz disfunção neurológica no cérebro e na medula, porém pode ser causada também pelo Neospora caninum e Neospora huguesi. O equino é o hospedeiro acidental e o vetor do protozoário é o gambá (hospedeiros definitivos). Um exame é o teste imunológico do líquor, que proporciona um diagnóstico preciso. No tratamento é utilizado coccidioestatico, antiinflamatório, vitaminas e analgésicos, e evitar que o hospedeiro definitivo tenha acesso aos alimentos dos equinos. O objetivo do presente trabalho foi desenvolver uma revisão de literatura sobre mieloencefalite protozoária equina.

Equine protozoal myeloencephalitis (EPM) is an infectious disease caused mainly by the protozoan Sarcocystis neuron that produces neurological dysfunction in the brain and marrow, but can also be caused by Neospora caninum and Neospora huguesi. The equine is the accidental host and the vector of the protozoan is the opossum (definitive hosts). An examination is the CSF test, which provides an accurate diagnosis. In the treatment coccidiostatic, anti-inflammatory, vitamins and analgesics are used, and to avoid that the definitive host has access to the food of the horses. The objective of the present work was to develop a literature review on equine protozoal myeloencephalitis.

Mieloencefalite protozoária equina

A mieloencefalite protozoária eqüina é uma doença que acomete o sistema nervoso central (SNC) causando uma infecção. O principal agente etiológico é o Sarcocystis neurona e tem como hospedeiro definitivo o Didelphis albiventris (gambá). Há predominância em alguns sinais clínicos como ataxia e paresia. A rápida avaliação do animal e o rápido diagnóstico podem diminuir algumas complicações e diminuir os sinais clínicos presentes nele.

Equine protozoal myeloencephalitis is a disease that affects the central nervous system (CNS) causing an infection. The main etiological agent is Sarcocystis neuronal and has its definitive host Didelphis albiventris (opossum). There is a predominance of some clinical signs such as ataxia and paresis. Rapid assessment of the animal and rapid diagnosis may decrease some complications and decrease the clinical signs present in it.

Signo de la copa de vino: A propósito de un caso con esclerosis lateral primaria y demencia frontotemporal / Wine glass sign in a patient with primary lateral sclerosis and frontotemporal dementia: a case report

La esclerosis lateral primaria es muy poco frecuente, representa 2-4 % del grupo de enfermedades de motoneurona. Se caracteriza por espasticidad corticoespinal y síndrome pseudobulbar. La resonancia magnética nuclear muestra lesiones hiperintensas de secuencias potenciadas en T2; cuando el compromiso es bilateral, la imagen da la apariencia de copa de vino . Caso clínico: Paciente varón de 56 años de edad, con un cuadro clínico de dos años de evolución, caracterizado por desinhibición, apatía, y conducta social inapropiada. La evolución clínica mostró criterios diagnósticos de esclerosis lateral primaria y de demencia frontotemporal. La resonancia magnética de encéfalo, protocolo T2, corte coronal reveló el compromiso bilateral de los haces cortico-espinales desde el centro semioval hasta las pirámides bulbares semejando la imagen en copa de vino . Aunque infrecuente, el caso muestra la posible asociación de Esclerosis Lateral Primaria (ELP) con demencia frontotemporal; la imagen en copa de vino puede estar presente en enfermedad de motoneurona y, cuando particularmente asociada a ELP, puede ser de gran ayuda en el diagnostico diferencial con otras entidades crónicas de curso clínico similar.

Primary lateral sclerosis is a very uncommon progressive disease and represents 2-4% of motor neuron diseases group. It is characterized by cortico-spinal spasticity and pseudo bulbar syndrome. Magnetic resonance shows white matter hyperintensities in T2 weight sequence. When the impairment is symmetrical, the image takes the appearance of a wine glass . Clinical case: A 56-year-old male patient with a clinical picture of 2-years duration characterized by disinhibition, apathy, and inappropriate social behavior. The clinical evolution met the diagnostic criteria of primary lateral sclerosis and frontotemporal dementia. Magnetic resonance imaging in coronal T2 weighted sequences, showed symmetrical impairment of corticospinal pathway from the semiovale centrum to the medullary pyramids showing the wine glass appearance. Although infrequent, the case shows the possible association of Primary Lateral Sclerosis (PLS) with frontotemporal dementia; the wine glass image in magnetic resonance may be present in motor neuron disease, when particularly associated with PLS may be of great help in the differential diagnosis with other chronic entities of similar clinical course.

Fatores de risco associados à prevalência de anticorpos anti-Sarcocystis neurona, Neospora spp. e Toxoplasma gondii em equinos de Roraima, Amazônia / Risk factors associated to the prevalence of anti-Sarcocystis neurona, Neospora spp. and Toxoplasma gondii antibodies in horses from Roraima, Brazilian Amazon

Amostras de sangue de 303 equinos provenientes de 56 propriedades do município de Rorainópolis, Roraima, foram avaliadas por Reação de Imunofluorescência Indireta (RIF) para pesquisa de anticorpos contra Sarcocystis neurona, Toxoplasma gondii e Neospora spp. Algumas amostras de soros positivos para Sarcocystis spp. foram avaliadas pelo Western Blotting (WB) utilizando antígenos crus de S. neurona. A partir dos resultados sorológicos, possíveis fatores de risco foram avaliados frente a variáveis individuais e de propriedade. A prevalência de anticorpos anti-Sarcocystis spp. foi estimada em 43,2% (37,6-49,0%), anti-Neospora sp. em 26,7% (21,9-32,2%) e anti-T. gondii de 18,5% (14,3-23,4%). Quatorze amostras (14/15) testadas por WB resultaram positivas para antígenos de S. neurona. Das propriedades, 76,8% (43/56) apresentaram pelo menos um equino positivo para Sarcocystis spp.; 69,6% (39/56) para Neospora spp. e 55,4% (31/56) para T. gondii. Dos equinos, 13 (4,3%) apresentarem anticorpos para os três agentes, 50 (16,5%) para Sarcocystis spp. e Neospora spp., 10 (3,3%) para Neospora spp. e T. gondii, e oito (2,6%) para Sarcocystis spp. e T. gondii. As variáveis associadas (P≤0,05) à presença de anticorpos foram: para Neospora spp. não pastejar em áreas alugadas, ausência de assistência veterinária na propriedade, sexo masculino, não estabular animais e plantel equino acima de 5 animais; enquanto para T. gondii foram o contato com felinos, animais da raça lavradeiro, animal estabulado, criação de bovinos na propriedade e plantel equino acima de 5 animais. Não houveram variáveis associadas a presença de anticorpos contra S. neurona. Relata-se no presente estudo a primeira detecção de anticorpos anti-S. neurona, Neospora spp. e T. gondii em equinos do estado de Roraima, localizado na Amazônia Setentrional Brasileira, ressaltando para a elevada frequência de fazendas com equinos soropositivos.

Samples of 303 horses from 56 ranches of Rorainópolis municipality, state of Roraima, were evaluated by means of the Indirect Immunofluorescence Test (IFAT) to detect antibodies against Sarcocystis spp., Toxoplasma gondii and Neospora spp. A subset of positive sample (n=15) against Sarcocystis spp. was evaluated by Western Blotting (WB) with crude antigen of S. neurona. From the serological result, possible risk factors were evaluated against individual or farming variables. The prevalence of anti-Sarcocystis spp. antibodies was estimated to be 43.2% (37.6-49.0%), anti-Neospora sp. was 26.7% (21.9-32.2%), and anti-T. gondii was 18.5% (14.3-23.4%). Fourteen samples (14/15) evaluated by WB were positive for S. neurona antigens. From the ranches, 76.8% (43/56) presented at least one positive horse for Sarcocystis spp., 69.6% (39/56) for Neospora spp., and 55.4% (31/56) for T. gondii. Thirteen (14.3%) horses had antibodies against all agents, 50 (16.5%) had antibodies against Sarcocystis spp. and Neospora spp., 10 (3.3%) for Neospora spp. and T. gondii, and eight (2.6%) for Sarcocystis spp. and T. gondii. Associated variables (P≤0.05) for antibodies against Neospora spp. were not found in horses fed on rented pastures, not in horses without veterinary assistance and stables, and not in herds up to 5 horses; while they were associated for T. gondii by contact with cats, in the Lavradeiro breed with use of stables, in horses raise with cattle, and in herds up to 5 horses. There were no variables associated with the presence of antibodies against S. neurona. Antibodies against S. neurona, Neospora spp. and T. gondii were reported in horses from the state of Roraima, Northern Brazilian Amazon, highlighting to the elevate prevalence on ranches.(AU)

Fatores de risco associados à prevalência de anticorpos anti-Sarcocystis neurona, Neospora spp. e Toxoplasma gondii em equinos de Roraima, Amazônia / Risk factors associated to the prevalence of anti-Sarcocystis neurona, Neospora spp. and Toxoplasma gondii antibodies in horses from Roraima, Brazilian Amazon

Amostras de sangue de 303 equinos provenientes de 56 propriedades do município de Rorainópolis, Roraima, foram avaliadas por Reação de Imunofluorescência Indireta (RIF) para pesquisa de anticorpos contra Sarcocystis neurona, Toxoplasma gondii e Neospora spp. Algumas amostras de soros positivos para Sarcocystis spp. foram avaliadas pelo Western Blotting (WB) utilizando antígenos crus de S. neurona. A partir dos resultados sorológicos, possíveis fatores de risco foram avaliados frente a variáveis individuais e de propriedade. A prevalência de anticorpos anti-Sarcocystis spp. foi estimada em 43,2% (37,6-49,0%), anti-Neospora sp. em 26,7% (21,9-32,2%) e anti-T. gondii de 18,5% (14,3-23,4%). Quatorze amostras (14/15) testadas por WB resultaram positivas para antígenos de S. neurona. Das propriedades, 76,8% (43/56) apresentaram pelo menos um equino positivo para Sarcocystis spp.; 69,6% (39/56) para Neospora spp. e 55,4% (31/56) para T. gondii. Dos equinos, 13 (4,3%) apresentarem anticorpos para os três agentes, 50 (16,5%) para Sarcocystis spp. e Neospora spp., 10 (3,3%) para Neospora spp. e T. gondii, e oito (2,6%) para Sarcocystis spp. e T. gondii. As variáveis associadas (P≤0,05) à presença de anticorpos foram: para Neospora spp. não pastejar em áreas alugadas, ausência de assistência veterinária na propriedade, sexo masculino, não estabular animais e plantel equino acima de 5 animais; enquanto para T. gondii foram o contato com felinos, animais da raça lavradeiro, animal estabulado, criação de bovinos na propriedade e plantel equino acima de 5 animais. Não houveram variáveis associadas a presença de anticorpos contra S. neurona. Relata-se no presente estudo a primeira detecção de anticorpos anti-S. neurona, Neospora spp. e T. gondii em equinos do estado de Roraima, localizado na Amazônia Setentrional Brasileira, ressaltando para a elevada frequência de fazendas com equinos soropositivos.

Samples of 303 horses from 56 ranches of Rorainópolis municipality, state of Roraima, were evaluated by means of the Indirect Immunofluorescence Test (IFAT) to detect antibodies against Sarcocystis spp., Toxoplasma gondii and Neospora spp. A subset of positive sample (n=15) against Sarcocystis spp. was evaluated by Western Blotting (WB) with crude antigen of S. neurona. From the serological result, possible risk factors were evaluated against individual or farming variables. The prevalence of anti-Sarcocystis spp. antibodies was estimated to be 43.2% (37.6-49.0%), anti-Neospora sp. was 26.7% (21.9-32.2%), and anti-T. gondii was 18.5% (14.3-23.4%). Fourteen samples (14/15) evaluated by WB were positive for S. neurona antigens. From the ranches, 76.8% (43/56) presented at least one positive horse for Sarcocystis spp., 69.6% (39/56) for Neospora spp., and 55.4% (31/56) for T. gondii. Thirteen (14.3%) horses had antibodies against all agents, 50 (16.5%) had antibodies against Sarcocystis spp. and Neospora spp., 10 (3.3%) for Neospora spp. and T. gondii, and eight (2.6%) for Sarcocystis spp. and T. gondii. Associated variables (P≤0.05) for antibodies against Neospora spp. were not found in horses fed on rented pastures, not in horses without veterinary assistance and stables, and not in herds up to 5 horses; while they were associated for T. gondii by contact with cats, in the Lavradeiro breed with use of stables, in horses raise with cattle, and in herds up to 5 horses. There were no variables associated with the presence of antibodies against S. neurona. Antibodies against S. neurona, Neospora spp. and T. gondii were reported in horses from the state of Roraima, Northern Brazilian Amazon, highlighting to the elevate prevalence on ranches.(AU)