Factores neurológicos asociados a la mortalidad en pacientes con accidente cerebrovascular y ventilación mecánica artificial / Neurological factors associated to mortality in patients with cerebrovascular accident and artificial mechanical ventilation

Introducción: El accidente cerebrovascular es una de las causas más comunes de mortalidad a nivel mundial. Objetivo: Determinar la asociación existente entre el desarrollo de afecciones neurológicas y la necesidad de ventilación mecánica con el aumento de la incidencia de mortalidad en la unidad de cuidados intensivos. Métodos: Estudio observacional, prospectivo de corte transversal, realizado en la unidad de cuidados intensivos de un hospital de atención secundaria. La población de estudio estuvo constituida por 52 pacientes con accidente cerebrovascular los cuales recibieron soporte respiratorio artificial entre los años 2018 y 2020. La variable de interés final fue la mortalidad. Los factores neurológicos estudiados fueron el tipo de accidente cerebrovascular, puntuación de la escala de coma de Glasgow, ausencia de reflejos de tallo encefálico, anisocoria y complicaciones neurológicas. El nivel de significación se halló según p valor ≤ 0,05 a través de Chi cuadrado de independencia. Resultados: La mortalidad proporcional predominó en el accidente cerebrovascular hemorrágico tipo hemorragia intracraneal no traumática (p= 0,118), ausencia de reflejos del tallo encefálico (p=0,000), anisocoria (p=0,000), escala de coma de Glasgow <8 puntos (p=0,000) y complicaciones neurológicas como la hipertensión endocraneana (p=0,010). Conclusiones: Los factores neurológicos asociados a la mortalidad fueron la ausencia de reflejos del tallo encefálico, anisocoria, escala de coma de Glasgow <8 puntos y complicaciones neurológicas como la hipertensión endocraneana(AU)

Introduction: Cerebrovascular accident is one of the commonest causes of mortality in the world. Objective: To determine the association between development of neurological disorders and the need for mechanical ventilation with an increased incidence of mortality in the intensive care unit. Methods: An observational, prospective and cross-sectional study was carried out in the intensive care unit of a secondary care hospital. The study population consisted of 52 patients with cerebrovascular accident who received artificial respiratory support between 2018 and 2020. The final variable of interest was mortality. The neurological factors studied were type of cerebrovascular accident, score according to the Glasgow coma scale, absence of brainstem reflexes, anisocoria, and neurological complications. The level of significance was determined according to P ≤ 0.05, through chi-square of independence. Results: Proportional mortality prevailed in hemorrhagic cerebrovascular accident of nontraumatic intracranial hemorrhage type (P=0.118), absence of brainstem reflexes (P=0.000), anisocoria (P=0.000), score of less than eight points according to the Glasgow coma scale (P=0.000), and neurological complications such as endocranial hypertension (P=0.010). Conclusions: The neurological factors associated with mortality were absence of brainstem reflexes, anisocoria, score of less than eight points according to the Glasgow coma scale, and neurological complications such as endocranial hypertension(AU)

Trastornos neurológicos asociados a la infección por virus dengue / Neurological disorders associated with dengue virus infection

Introducción: Los trastornos neurológicos asociados al dengue es un tema que cobra vital importancia en estos tiempos debido a que el espectro clínico del dengue ha cambiado y las manifestaciones atípicas están reportándose con mayor frecuencia. Los serotipos 2 y 3 son los más frecuentemente relacionados a manifestaciones neurológicas como la encefalitis, mielitis y síndrome de Guillain-Barré. Objetivo: Reportar una serie de casos con trastornos neurológicos asociados a la infección por DENV y sus variables clínicas, humorales e imagenológicas. Casos clínicos: Todos los pacientes cursaron en primera instancia con un cuadro clínico de dengue no complicado dado por fiebre de 38,5º C y escalofríos de aparición brusca, exantema maculo-papuloso céfalo-caudal que respeta palmas y plantas, artralgias, mialgias, cefalea frontal y retro-ocular, esta última a la contracción del músculo recto superior del globo ocular. Todos confirmados con anticuerpos específicos anti-virus del dengue. Un paciente desarrolló síndrome de Guillain-Barré, otro curso con encefalitis, bicitopenia y compromiso del nivel de conciencia. El resto cursó con mielitis transversa, meningoencefalitis y encefalitis autoinmune. Conclusiones: Los trastornos neurológicos asociados al virus del dengue deben ser sospechados en todo paciente que ingrese con esta infección y manifieste cualquiera de las alteraciones aquí descritas(AU)

Introduction: Neurological disorders associated with dengue is an issue of vital importance in these times because the clinical spectrum of dengue has changed and atypical manifestations are being reported much frequently. Serotypes 2 and 3 are the most frequently related to neurological manifestations such as encephalitis, myelitis and Guillain-Barré syndrome. Objective: To report a series of cases with neurological disorders associated with DENV infection and its clinical, humoral and imaging variables. Clinical case report: All patients had, in the first instance, uncomplicated dengue clinical status due to 38.5º C fever and chills of sudden appearance, Cephalo-caudal maculo-papular rash that spares palms and soles, arthralgias, myalgias, frontal and retro-ocular headache, the latter due to the contraction of the superior rectus muscle of the eyeball. All confirmed with specific anti-dengue virus antibodies. One patient developed Guillain-Barré syndrome, another had encephalitis, bicytopenia, and compromised level of consciousness. The rest had transverse myelitis, meningoencephalitis and autoimmune encephalitis. Conclusions: Neurological disorders associated with dengue virus should be suspected in every patient who is admitted with this infection and manifests any of the alterations described here(AU)

Manifestaciones neurológicas de COVID-19. Revisión de la literatura / Neurological manifestations of COVID-19. Literature review

El nuevo coronavirus se origino en la ciudad de Wuhan, China, esta enfermedad afecta principalmente el sistema respiratorio, los síntomas pueden ir desde leves a severos, así también, existe otra variedad de presentaciones clínicas en otros sistemas, como es el sistema nervioso central, actualmente existe evidencia de gran cantidad de publicaciones de presentación neurológica como manifestaciones de COVID-19. Actualmente se ha descrito el potencial neurotrópico del coronavirus para invadir el sistema nervioso central y también se ha descrito diversos mecanismos de daño secundario. Las diferentes presentaciones neurológicas en niños como en adultos pueden ser variables, y estas incluyen manifestaciones del sistema nervios central, periférico y enfermedades musculares...(AU)

Patogenia de las manifestaciones neurológicas asociadas al SARS-CoV-2 / Pathogenesis of neurological manifestations associated to SARS-CoV-2

Introducción: Los coronavirus infectan al ser humano y pueden causar manifestaciones neurológicas en individuos susceptibles. Objetivo: Describir la patogenia de las manifestaciones neurológicas en pacientes con la COVID-19. Estrategia de búsqueda y criterios de selección: Se realizó una revisión bibliográfica empleando la bibliografía nacional e internacional actualizada. Se realizó la búsqueda en Google Académico, se consultaron artículos de libre acceso en las bases de datos Pubmed y SciELO, desde enero de 2014 hasta el 6 de mayo de 2020. Fueron seleccionados 51 artículos (6 en idioma español, 45 en inglés) y un libro de neuroinmunología. Se utilizaron los términos de búsqueda COVID-19, coronavirus, SARS-CoV-2,manifestaciones neurológicas, sistema nervioso, patogénesis, según el descriptor de Ciencias de la Salud (DeCS). Análisis e integración de la información: El SARS-CoV-2 entra al sistema nervioso por la vía linfática, hematógena, transináptica retrógada, por diseminación local a través del etmoides o por disfunción de la barrera hematoencefálica. La patogenia puede ser por la acción directa del virus o inmunomediada. En la pandemia de COVID-19 se reportan pacientes con manifestaciones neurológicas centrales, periféricas y musculoesqueléticas. Los síntomas más frecuentes son los trastornos del gusto, el olfato, cefaleas, mialgias y mareos. En las formas graves se reportan meningitis, encefalitis, síndrome de Guillain-Barré, ictus y encefalopatías. Conclusiones: El SARS-CoV-2 puede afectar al sistema nervioso central y periférico. Causa principalmente manifestaciones leves y transitorias, aunque pueden ocurrir complicaciones neurológicas. Los mecanismos patogénicos principales son el daño citopático directo o mecanismos indirectos debido a una respuesta inflamatoria(AU)

Introduction: Coronaviruses infect humans and may cause neurological manifestations in susceptible individuals. Objective: Describe the pathogenesis of neurological manifestations in patients with COVID-19. Search strategy and selection criteria: A review was conducted of national and international updated bibliography. The search was carried out in Google Scholar and open access papers were consulted in the databases PubMed and SciELO from January 2014 to 6 May 2020. A total 51 papers (6 in Spanish and 45 in English) and a book on neuroimmunology were selected. The search terms used were COVID-19, coronavirus, SARS-CoV-2, neurological manifestations, nervous system and pathogenesis, in compliance with the Health Sciences Descriptors (DeCS). Data analysis and integration: SARS-CoV-2 enters the nervous system by lymphatic, hematogenous, transynaptic, retrograde routes, by local dissemination through the ethmoid, or by dysfunction of the hematoencephalic barrier. Pathogenesis may be due to direct action by the virus or immunomediated. During the COVID-19 pandemic patients have been reported with central, peripheral and musculoskeletal neurological manifestations. The most common symptoms are taste and smell disorders, headache, myalgia and dizziness. Meningitis, encephalitis, Guillain-Barré syndrome, stroke and encephalopathies have been reported in severe forms of the disease. Conclusions: SARS-CoV-2 may affect the central and the peripheral nervous system. It mainly causes mild, transient manifestations, but neurological complications may also occur. The main pathogenic mechanisms are direct cytophatic damage or indirect mechanisms resulting from an inflammatory response(AU)

Neurological complications in patients with SARS-CoV-2 infection: a systematic review / Complicações neurológicas em pacientes infectados pelo SARS-CoV-2: uma revisão sistemática

ABSTRACT Background: As the COVID-19 pandemic unfolds worldwide, different forms of reports have described its neurologic manifestations. Objective: To review the literature on neurological complications of SARS-CoV-2 infection. Methods: Literature search performed following systematic reviews guidelines, using specific keywords based on the COVID-19 neurological complications described up to May 10th, 2020. Results: A total of 43 articles were selected, including data ranging from common, non-specific symptoms, such as hyposmia and myalgia, to more complex and life-threatening conditions, such as cerebrovascular diseases, encephalopathies, and Guillain-Barré syndrome. Conclusion: Recognition of neurological manifestations of SARS-CoV-2 should be emphasized despite the obvious challenges faced by clinicians caring for critical patients who are often sedated and presenting other concurrent systemic complications.

RESUMO Introdução: À medida que a pandemia da COVID-19 se desenvolve em todo o mundo, diferentes tipos de publicações descreveram suas manifestações neurológicas. Objetivo: Revisar a literatura sobre complicações neurológicas da infecção por SARS-CoV-2. Métodos: A pesquisa bibliográfica foi realizada seguindo diretrizes de revisões sistemáticas, usando palavras-chave específicas baseadas nas complicações neurológicas da COVID-19 descritas até 10 de maio de 2020. Resultados: Foram selecionados 43 artigos, incluindo descrições que variam de sintomas comuns e inespecíficos, como hiposmia e mialgia, a condições mais complexas e com risco de vida, como doenças cerebrovasculares, encefalopatias e síndrome de Guillain-Barré. Conclusão: O reconhecimento das manifestações neurológicas da SARS-CoV-2 deve ser enfatizado apesar dos óbvios desafios enfrentados pelos clínicos que cuidam de pacientes críticos, muitas vezes sedados e apresentando outras complicações sistêmicas concomitantes.

Hipoparatiroidismo y calcificaciones cerebrales: reporte de caso / Hypoparathyroidism and brain calcifications: a case report

Introducción. El hipoparatiroidismo es una enfermedad caracterizada por la ausencia o concentraciones inadecuadamente bajas de hormona paratiroidea (PTH), que conduce a hipocalcemia, hiperfosfatemia y excreción fraccional elevada de calcio en la orina. Las calcificaciones del sistema nervioso central son un hallazgo frecuente en estos pacientes. Caso clínico. Mujer de 56 años con antecedente de hipotiroidismo, que ingresó por un cuadro de 6 días de evolución caracterizado por astenia, parestesias periorales y movimientos anormales de manos y pies. Las pruebas de laboratorio demostraron hipocalcemia, hiperfosfatemia y niveles bajos de hormona paratiroidea. Se realizó una tomografía computarizada de cráneo que mostró áreas bilaterales y simétricas de calcificaciones en hemisferios cerebelosos, ganglios basales y corona radiata. No se evidenciaron trastornos en el metabolismo del cobre y hierro. Se estableció el diagnóstico del síndrome de Fahr secundario a hipoparatiroidismo y se inició tratamiento con suplementos de calcio y vitamina D con evolución satisfactoria. Discusión. El síndrome de Fahr es un trastorno neurológico caracterizado por el depósito anormal de calcio en áreas del cerebro que controlan la actividad motora. Se asocia a varias enfermedades, especialmente, hipoparatiroidismo. La suplementación con calcio y vitamina D con el objetivo de normalizar los niveles plasmáticos de estos cationes es el tratamiento convencional. (AU)

Introduction. Hypoparathyroidism is a disease characterized by absence or inappropriately low concentrations of circulating parathyroid hormone, leading to hypocalcaemia, hyperphosphataemia and elevated fractional excretion of calcium in the urine. Central nervous system calcifications are a common finding in these patients. Case report. 56-year-old woman with a history of hypothyroidism who was admitted for a 6-day course of illness characterized by asthenia, perioral paresthesias, and abnormal movements of the hands and feet. Laboratory tests showed hypocalcemia, hyperphosphatemia, and low parathyroid hormone levels. A cranial computed tomography was performed. It showed bilateral and symmetrical areas of calcifications in the cerebellar hemispheres, basal ganglia, and radiata crown. No disorders of copper or iron metabolism were evident. The diagnosis of Fahr syndrome secondary to hypoparathyroidism was established and treatment with calcium and vitamin D supplements was started with satisfactory evolution. Discussion. Fahr's syndrome is a neurological disorder associated with abnormal calcium deposition in areas of the brain that control motor activity. It is associated with various diseases, especially hypoparathyroidism. The conventional treatment is supplementation with calcium and vitamin D, with the aim of normalizing their plasma levels. (AU)

Capacidad funcional, densidad mineral ósea y marcadores de neoformación-reabsorción ósea en pacientes menores de 18 años con movilidad reducida / Functional capacity, bone mineral density and neoformation-resorption bone markers in patients under 18 years of age with reduced mobility

Resumen: Introducción: La inmovilización prolongada asociada a diversas enfermedades neurológicas, causa osteoporosis secundaria con fracturas patológicas y dolor óseo persistente. Objetivos: Establecer la asociación entre densidad mineral ósea (DMO), marcadores de neoformación y reabsorción ósea y grado de capacidad funcional en pacientes menores de 18 años con movilidad reducida. Pacientes y Método: Estudio transversal, realizado entre 1/1/2016 y 31/12/2017 en pacientes de 6 a 18 años diagnosticados de distintas enfermedades neurológicas en Ciudad Real (España). Se analizaron las variables biodemográficas, capacidad funcional según la Functional Mobility Scale (FMS), que valora la movilidad en 5, 50 y 500 metros, DMO, 25-hidroxi-vitamina D, fosfatasa alcalina, osteocalcina en sangre y telopéptido amino terminal de cadena cruzada de colágeno tipo I en orina (NTX-I). Se expresan DMO, fosfatasa alcalina, osteocalcina y NTX-I en Z score según valores de referencia para edad y sexo. Se utilizaron estadísticas descriptivas y correlaciones de Pearson y Spearman. Resulta dos: 36 pacientes (52,7% niñas), edad media de 8,6 ± 4,7 años. Valor medio de FMS: 5,3 sobre 18. DMO media: -1,99 ± 1,7 desviaciones estándar (DE), fosfatasa alcalina media: -2,64 ± 1,08, osteocalcina media: -2,15 ± 1,39, y NTX-I medio: +3 ± 1,72. Hubo asociación significativa entre DMO y FMS para 5 metros (r = 0,395; p = 0,017) y para la puntuación total (r = 0,365; p = 0,029). No se encon traron diferencias significativas según estadios de desarrollo puberal. Conclusiones: En la población estudiada se observa disminución en la DMO y en marcadores de neoformación ósea y elevación de marcadores de reabsorción ósea sin asociación con el desarrollo puberal. Los pacientes con menor grado de movilidad presentan una DMO inferior.

Abstract: Introduction: Prolonged immobilization associated with several neurological disorders causes se condary osteoporosis with pathological fractures and persistent bone pain. Objectives: To establish the association between bone mineral density (BMD), neoformation and bone resorption markers and the degree of functional capacity in children under 18 years of age with reduced mobility. Pa tients and Method: Cross-sectional study conducted in Ciudad Real, Spain between January 1, 2016, and December 31, 2017 with patients aged between 6 and 18 years diagnosed with different neurological disorders. The following variables were analyzed: age, sex, pubertal stage, functional capacity according to the Functional Mobility Scale (FMS), which assesses the ability to walk from 5, 50 to 500 meters, BMD, 25-hydroxy-vitamin D, alkaline phosphatase and osteocalcin in blood, and N-terminal telopeptide crosslinks in collagen type I (NTX-I) in urine. BMD, alkaline phosphatase, osteocalcin, and NTX-I values are expressed in Z score according to reference values for age and sex. The Pear son and Spearman correlations were used for data analysis. Results: 36 patients (52.7% girls) with an average age of 8.6±4.7 years. Mean FMS value: 5.3 out of 18. Mean BMD: -1.99 ± 1.7 standard deviations (SD), mean alkaline phosphatase: -2.64 ± 1.08, mean osteocalcin: -2.15 ± 1.39, and mean NTX-I: +3 ± 1.72. There was a significant association between BMD and FMS for 5 meters (r = 0.395; p = 0.017) and for total score (r = 0.365; p = 0.029). There were no significant differences according to the stages of pubertal development. Conclusions: In this population, there was a decrease in BMD and bone neoformation markers, and an increase of bone resorption markers with no association with pubertal development. Patients with a lower degree of mobility present a lower BMD.

Mecanismos neuroinvasivos y del daño neurológico en las infecciones por coronavirus / Neuroinvasive and neurological damage mechanisms in the infections by coronavirus

Introducción: En diciembre de 2019, en China, se reportaba un nuevo coronavirus, que se clasificó y denominó como síndrome respiratorio agudo severo-coronavirus 2, causante de la enfermedad COVID-19; capaz de provocar síntomas y complicaciones neurológicas. Objetivo: Describir los mecanismos neuroinvasivos y del daño neurológico en las infecciones por coronavirus. Métodos: De las bases datos PubMed, SciELO y Latindex, se seleccionaron artículos publicados desde 2005 hasta mayo de 2020 que trataran: la lesión neurológica directa, la lesión neurológica indirecta y los mecanismos de la muerte neuronal en las infecciones por coronavirus. Se incluyeron estudios en humanos, animales de experimentación y estudios in vitro. Resultados: Las propiedades neuroinvasivas de los coronavirus está demostrada, pero la fisiopatología que media la neuroinvasión y el daño neurológico en la infección por SARS-CoV-2 no está totalmente aclarada. Las propiedades neurotrópicas del nuevo coronavirus, se demostraron por la presencia del virus en líquido cefalorraquídeo y cerebro. Los mecanismos que explican el daño neurológico están presentes en los pacientes con formas graves de la COVID-19 y justifican las manifestaciones clínicas y complicaciones neurológicas de la enfermedad. Consideraciones finales: Los coronavirus son neuroinvasivos y neurotrópicos, con potencialidad para provocar enfermedad neurológica. Las hipótesis fisiopatológicas indican que las manifestaciones en el sistema nervioso se deben a causas inflamatorias. Se necesitan estudios a nivel biomolecular para aceptar o refutar tales teorías. Esto ayudaría a clasificar mejor todo el espectro de la neuropatología y elaborar un consenso sobre las mejores estrategias de diagnóstico y modalidades de tratamiento(AU)

Introduction: On December 2019, it was reported in China a new coronavirus that was classified and named as severe acute respiratory syndrome by coronavirus 2 that caused COVID-19 disease being able to provoque neurologic symptoms and complications. Objective: To describe the neuroinvasive and neurological damage mechanisms in the infections by coronavirus. Methods: From PubMed, SciELO and Latindex databases it was a selection of articles published from 2005 to May 2020 on: direct neurologic lesion, indirect neurologic lesion, and the mechanisms of neuronal death in the infection by coronavirus. Studies in humans, animals for experiments and in vitro studies were included. Results: The neuroinvasive properties of coronavirus are demonstrated, but it is not fully clear the physiopathology that has to do with neuroinvasion and the neurological damage in the infection by SARS-CoV-2. The neurotropic properties of the new coronavirus were proved by the presence of the virus in the spinal fluid and the brain. The mechanisms which explain the neurological damage are present in patients with severe forms of COVID-19 and they justify the clinical manifestations and neurological complications of the disease. Final considerations: The coronaviruses are neuroinvasive and neurotropic, with potential for causing neurologic disease. The physiopathologic hypotheses show that the manifestations in the nervous system are due to inflammatory causes. There is a need of conducting studies in the biomolecular level to accept or refute those theories. That will help to better classify all the spectrum of neuropathologies and to achieve a consensus on the best diagnostic strategies and treatment modalities(AU)

Family Management and socioeconomic situation of children and adolescents with neurological disorders / Manejo Familiar y situación socioeconómica de niños y adolescentes con trastornos neurológicos / Manejo Familiar e situação socioeconômica de crianças e adolescentes com distúrbios neurológicos

ABSTRACT Objectives: to identify the relation between family management and socioeconomic situation of children and adolescents with neurological disorders. Methods: a descriptive and correlational study with 141 family members who answered a questionnaire on socioeconomic and family management information. Data collection took place between May and September 2016 in a pediatric neurology center. A descriptive and analytical analysis was carried out with the use of statistical tests in order to assess the relation of the variables researched. Results: a higher early childhood disease time was related with higher scores of management difficulty. There was relation between the highest family income and management skills and the lowest scores of family difficulty. The highest schooling level of mothers was associated with the lowest scores of disease impact view and family difficulty. Conclusions: family members with unfavorable socioeconomic situation had more difficulty with family management.

RESUMEN Objetivos: identificar la correlación entre el manejo familiar y el estado socioeconómico de niños y adolescentes con trastornos neurológicos. Métodos: estudio descriptivo y correlacional con 141 miembros de la familia, que respondieron un cuestionario sobre información socioeconómica y gestión familiar. La recopilación de datos tuvo lugar entre mayo y septiembre de 2016 en un Centro de Neurología Pediátrica. Se realizó un análisis descriptivo y analítico utilizando pruebas estadísticas para evaluar la correlación entre las variables investigadas. Resultados: la mayor duración de la enfermedad infantil y juvenil se correlacionó con una mayor puntuación de dificultad de manejo. El mayor ingreso familiar se relacionó con un puntaje de habilidad gerencial más alto y un puntaje de dificultad familiar más bajo. La educación materna superior se asoció con una puntuación de visión más baja del impacto de la enfermedad y la dificultad familiar. Conclusiones: las familias en la situación socioeconómica más desfavorable tuvieron mayores dificultades en el manejo familiar.

RESUMO Objetivos: identificar a correlação entre o manejo familiar e a situação socioeconômica de crianças e adolescentes com distúrbios neurológicos. Métodos: estudo descritivo, correlacional, com participação de 141 familiares, os quais responderam a um questionário sobre informações socioeconômicas e do manejo familiar. A coleta de dados ocorreu entre maio e setembro de 2016, em um Centro de Neurologia Pediátrica. Realizou-se análise descritiva e analítica, com uso de testes estatísticos para avaliar a correlação entre as variáveis investigadas. Resultados: o maior tempo de doença infanto-juvenil foi correlacionado a um maior escore de dificuldade de manejo. A maior renda familiar foi relacionada ao maior escore de habilidade de manejo e ao menor escore de dificuldade familiar. A maior escolaridade materna associou-se ao menor escore de visão do impacto da doença e dificuldade familiar. Conclusões: as famílias em situação socioeconômica mais desfavorável tiveram maiores dificuldades no manejo familiar.

Supervivencia de pacientes con linfoma no-Hodgkin y síntomas neurológicos según inmunofenotipo del líquido cefalorraquídeo / Survival in non-Hodgkin´s lymphoma patients with neurological symptoms according to the cerebrospinal fluid immunophenotype

Introducción: Los linfomas no-Hodgkin pueden infiltrar el sistema nervioso central y producir síntomas neurológicos, lo cual incrementa la mortalidad. El diagnóstico de esta infiltración se puede realizar mediante el estudio del líquido cefalorraquídeo por la técnica de citometría de flujo, con una mayor sensibilidad que la citología convencional. Objetivo: Estimar la supervivencia global de pacientes con Linfoma no-Hodgkin y síntomas neurológicos según el inmunofenotipo celular del líquido cefalorraquídeo. Métodos: Se realizó un estudio analítico y prospectivo en 15 pacientes con diagnóstico confirmado de linfoma no-Hodgkin y síntomas neurológicos, con citología negativa del líquido cefalorraquídeo, tratados en el servicio de oncología del Instituto Nacional de Oncología y Radiobiología, durante los años 2017 y 2018. El inmunofenotipo fue caracterizado mediante citometría de flujo multiparamétrica. Resultados: El 60,0 por ciento de los pacientes fue del sexo femenino y el 53,4 por ciento mayor de 60 años. Hubo una mortalidad del 26,7 por ciento. Se realizaron 17 inmunofenotipos, el 58,9 por ciento fue normal, el 23,4 por ciento reactivo y el 17,7 por ciento sospechoso de malignidad. La supervivencia global fue mayor en pacientes con líquido cefalorraquídeo con inmunofenotipo normal (HR. 0.04). Conclusiones: La citometría de flujo pudo discriminar células sospechosas de malignidad, en pacientes cuyas citologías fueron negativas. La presencia en el líquido cefalorraquídeo de células atípicas, de pleocitosis y de un índice de linfocito-monocito alto se asoció con una supervivencia global menor(AU)

Introduction: When non-Hodgkin lymphomas infiltrate the central nervous system increases mortality. The diagnosis of this infiltration can be made by the study of cerebrospinal fluid using flow cytometry, with a higher sensitivity than conventional cytology. Objective: To estimate the relationship between the cellular immunophenotype of the cerebrospinal fluid and the overall survival of patients with non-Hodgkin lymphoma and neurological symptoms. Methods: An analytical and prospective study was conducted in 15 patients with confirmed diagnosis of non-Hodgkin lymphoma and neurological symptoms, with negative cytology of the cerebrospinal fluid. Patients cared at Oncology Department of the National Institute of Oncology and Radiobiology, during the years 2017-2018. The immunophenotype was characterized by multiparametric flow cytometry. Results: 60.0 percent of the patients was female and 53.4 percent older than 60 years. There was an overall mortality of 26.7 percent 17 immunophenotypes were found, 58.9 percent of them was normal, 23.4 percent reactive and 17.7 percent suspected of malignancy. Overall survival advantage was obtained in patients with cerebrospinal fluid with normal immunophenotype (HR 0.04). Conclusions: Flow cytometry could discriminate cells suspected of malignancy, in patients whose cytologies were negative. The presence in the cerebrospinal fluid of atypical cells, pleocytosis and a high lymphocyte-monocyte index were associated with a lower overall survival(AU)

Trends in renal calculus composition and 24-hour urine analyses in patients with neurologically derived musculoskeletal deficiencies

ABSTRACT Purpose: To better characterize metabolic stone risk in patients with neurologically derived musculoskeletal deficiencies (NDMD) by determining how patient characteristics relate to renal calculus composition and 24-hour urine parameters. Materials and Methods: We performed a retrospective cohort study of adult patients with neurologically derived musculoskeletal deficiencies presenting to our multidisciplinary Kidney Stone Clinic. Patients with a diagnosis of NDMD, at least one 24-hour urine collection, and one chemical stone analysis were included in the analysis. Calculi were classified as primarily metabolic or elevated pH. We assessed in clinical factors, demographics, and urine metabolites for differences between patients who formed primarily metabolic or elevated pH stones. Results: Over a 16-year period, 100 patients with NDMD and nephrolithiasis were identified and 41 met inclusion criteria. Thirty percent (12 / 41) of patients had purely metabolic calculi. Patients with metabolic calculi were significantly more likely to be obese (median body mass index 30.3kg / m2 versus 25.9kg / m2), void spontaneously (75% vs. 6.9%), and have low urine volumes (100% vs. 69%). Patients who formed elevated pH stones were more likely to have positive preoperative urine cultures with urease splitting organisms (58.6% vs. 16.7%) and be hyperoxaluric and hypocitraturic on 24-hour urine analysis (37mg / day and 265mg / day versus 29mg / day and 523mg / day). Conclusions: Among patients with NDMD, metabolic factors may play a more significant role in renal calculus formation than previously believed. There is still a high incidence of carbonate apatite calculi, which could be attributed to bacteriuria. However, obesity, low urine volumes, hypocitraturia, and hyperoxaluria suggest an underrecognized metabolic contribution to stone formation in this population.

Identification of factors related to cases of benign paroxysmal positional vertigo refractory to canalicular repositioning maneuvers and evaluation of the need for magnetic resonance imaging in their management: retrospective analysis of a series of 176 cases

Introduction: Benign paroxysmal positional vertigo (BPPV) is the most common form of peripheral vertigo, and, in most cases, it presents a favorable prognosis. The treatment is based on a series of specific canalicular repositioningmaneuvers that offer an efficacy close to 100%. Despite this, there are cases that are refractory to treatment, with the persistence of the vertigo symptoms. Objectives: The objective of the present paper is to analyze the factors associated with an increased risk of refractory BPPV and the importance of nuclear magnetic resonance in the study of these patients. Methods: We retrospectively reviewed the cases of 176 patients diagnosed with BPPV in our center.We divided them into two groups: responders and non-responders to the treatment, and analyzed the possible risk factors associated with a higher risk of refractory vertigo. Fischer exact test was used. Results: We found 11 cases refractory to treatment; all of them underwent magnetic resonance imaging (MRI) with gadoliniumaccording to our protocol. Of these, four had an otoneurologic background or pathology, and two other patients presented a multicanal involvement. The difference between the two groups was statistically significant (p < 0.05). Conclusion: Otoneurologic background and multicanal involvement were associated with a higher risk of refractory BPPV. When dealing with a BPPV with persistent symptomatology/nystagmus or with early relapse after an initial improvement, other entities that enter into the differential diagnosis must always be considered. We consider it essential to perform an MRI with gadolinium to rule out cases of BPPV that have a central cause (AU)

Everything you always wanted to know about sex and Neurology: neurological disability and sexuality / Todo lo que siempre quiso saber sobre sexo y Neurologia pero nunca se atrevió a preguntar: estudio sobre discapacidad neurologica y sexualidad

ABSTRACT Chronic neurological disorders generate disabilities affecting multiple aspects of life, including sexuality. Objective To describe the presence of sexual dysfunction and comorbidities in a population with chronic neurological disorders. To analyze the relationship between disability and sexual dysfunction. Methods A cross-sectional case-control study was carried out. Patients with amyotrophic lateral sclerosis (ALS), multiple sclerosis (MS), Parkinson's disease (PD), and stroke of at least one year since the onset of symptoms were included, and compared with controls with no neurological disease, matched by age and sex. Results We included 71 participants: 29 controls, with a mean age of 49.4 years, and 42 patients with a mean age of 53.8 years. Sexual dysfunction was present in 22.5% of the controls and 77.5% of the patients. A statistically significant relationship between sexual dysfunction and disability was found in the logistic regression analysis (OR = 20.38, 95%CI: 2.5 -165.86). Conclusions Disability proved to be the main variable related to the presence of sexual dysfunction. Patients with ALS had the worst rates of sexual dysfunction. Patients with MS were similar to the control group. As for the PD group, no patient had normal sexuality. Finally, in stroke patients, the presence of comorbidities and their treatment may have negatively influenced sexuality. These findings showed that patients with chronic neurological diseases have sexual dysfunction and underscore the need for neurologists to know and address this problem.

RESUMO Las enfermedades neurológicas crónicas generan discapacidad afectando múltiples aspectos de la vida, incluida la sexual. Objetivo Describir en una población con enfermedades neurológicas crónicas la presencia de disfunción sexual y posibles comorbilidades acompañantes. Analizar la relación entre discapacidad y disfunción sexual. Métodos Se realizó un estudio transversal de tipo casos y controles. Se incluyeron pacientes con Esclerosis Lateral Amiotrófica (ELA), Esclerosis Múltiple (EM), enfermedad de Parkinson (EP) y secuelados por enfermedad cerebrovascular (sACV) de al menos un año de evolución, controlando con sujetos sin enfermedad neurológica pareados por edad y sexo. Resultados Se incluyeron 71 sujetos: 29 controles, con una edad media 49,4 años y 42 casos con una edad media de 53,8 años. Presentaron disfunción sexual el 22,5% de los controles y el 77,5% de los casos. En el análisis por regresión logística se encontró una relación estadísticamente significativamente entre disfunción sexual y discapacidad. (OR = 20.38, IC95%: 2.5-165.86). Conclusiones La discapacidad demostró ser la principal variable relacionada con la presencia de disfunción sexual. Los enfermos con ELA fueron los que peores índices de disfunción sexual presentaron. Los pacientes con EM se comportaron de forma similar al grupo control. En cuanto al grupo de EP todos los pacientes tuvieron algún trastorno en su sexualidad. Por último, en sACV la presencia de comorbilidades y su tratamiento podrían influir negativamente en la sexualidad. Estos hallazgos evidencian que la disfunción sexual está presente en los pacientes con enfermedades neurológicas crónicas y confirma la necesidad de conocer este problema por parte de los neurólogos.

Complicações observadas em cães e gatos com doenças neurológicas / Complications observed in dogs and cats with neurological diseases

Cães e gatos com problemas neurológicos estão sujeitos a várias sequelas e complicações, em decorrência das deficiências motoras, sensoriais e viscerais. Algumas complicações estão relacionadas a maior mortalidade, principalmente aquelas relacionadas ao trauma vertebromedular. O objetivo do presente estudo foi avaliar nessas espécies a frequência do aparecimento de complicações, a epidemiologia, localização da lesão e etiologia, avaliando ainda a evolução e o tempo de sobrevida. Foram acompanhados 100 cães e gatos com doenças neurológicas trazidos para atendimento no Serviço de Neurologia do Hospital Veterinário da Universidade Estadual de Londrina. Observou-se que 91% dos animais desenvolveram complicações, sendo que as mais frequentes foram atrofia muscular (n=32), retenção urinária (n=24), incontinência urinária (n=24), incontinência fecal (n=17) e feridas de decúbito (n=16). O principal local de lesão associado ao aparecimento das complicações foi medula espinhal (91,3%) e a principal etiologia foi o trauma vertebromedular (37,3%). O tempo mediano e médio de vida estimado foram, respectivamente, dois e sete meses sendo que as principais causas de morte ou eutanásia estavam relacionadas ao trauma vertebromedular, trauma cranioencefálico ou doença inflamatória/infecciosa. O tempo de vida estimado foi menor para animais mais velhos e mais pesados. Embora o índice de sobrevivência dos animais com complicações tenha sido alto, muitos permaneceram com sequelas, indicando que a sobrevivência não significou capacidade funcional normal. Cães e gatos com problemas neurológicos requerem alto grau de atenção do veterinário e de seus tutores, sendo fundamental a orientação a respeito da real expectativa de recuperação e das dificuldades de manejo que poderão ser encontradas no curso da doença.(AU)

Dogs and cats with neurological diseases can show several sequelae and complications as a result of motor, sensory and visceral deficiencies. Some complications are an important cause of mortality, mainly complications related to spinal cord injury. The aim of this study was to evaluate in 100 dogs and cats brought to the Neurology Service of Veterinary Hospital from Universidade Estadual de Londrina the frequency of complications, epidemiology, lesion localization and etiology, still assessing the evolution and survival time. It was observed that 91% of the animals developed complications. The most frequent were muscular atrophy (n=32), urinary retention (n=24), urinary incontinence (n=24), fecal incontinence (n=17) and pressure sores (n=16). The main site of injury associated with the onset of complications was spinal cord (91%) and the main cause was the spinal cord trauma (37.3%). The median survival time of animals with complications was two months and the mean was seven months. The main causes of death or euthanasia were related do spinal cord trauma, traumatic brain injury or inflammatory/infectious disease. The estimated lifetime was lower for older and heavier animals. Although the survival rate of animals with complications was high, most of them remained with sequelae, indicating that survival does not mean normal functional capacity. Dogs and cats with neurological problems require a high degree of attention of the veterinarian and their guardians, and guidelines regarding the real expectation of recovery and management difficulties that may be encountered in the course of the disease are essential.(AU)

Doenças do sistema nervoso central em cães / Central nervous system disorders diagnosed in dogs

São descritas doenças do sistema nervoso central (SNC) em cães diagnosticadas no sertão da Paraíba. Os registros de necropsia de 1.205 cães foram revisados. Em 354 casos (29,38%) foram registrados história clínica de alterações do sistema nervoso. Duzentos e noventa e seis casos tiveram diagnóstico definitivo e 58 foram inconclusivos. As doenças infecciosas foram observadas em 59,60% (211/354) de casos que representam a principal causa de distúrbios neurológicos; 53% dos casos (186/354) foram representadas por doenças virais; 3,11% (11/354) foram de etiologia parasitária, 2,54% (9/354) foram causadas por bactérias e 1,41% (5/354) por fungos. Os agentes físicos representaram a segunda causa mais importante de transtornos do SNC com 9,89% (35/354) e os tumores a terceira causa com 5,93% (21/354). Outras alterações pouco frequentes foram alterações metabólicas secundárias a insuficiência hepática ou renal, representando 2,54% (9/354). Casos raros de hidrocefalia congênita foram observados, 1,41% (5/354). Os casos de manifestações neurológicas associadas a alterações vasculares, degenerativas e inflamatórias não infecciosas, muitas das quais uma causa específica não foi estabelecida representaram 4,24% (15/354); Estavam dentro das seguintes categorias de doenças: Infartos isquêmicos e hemorrágicos (6/15), necrose vascular fibrinoide (5/15), doença do disco intervertebral (2/15), meningoencefalite granulomatosa (1/15) e granuloma de colesterol (1/15). Os distúrbios do sistema nervoso central representam uma importante causa de morte ou eutanásia em cães na região semiárida da Paraíba. Os sinais clínicos variaram de acordo com o agente envolvido, localização e distribuição das lesões. O conhecimento dos principais agentes que pode afetar o SNC canino é importante ao fazer uma lista de diagnóstico diferencial.(AU)

Central nervous system (CNS) diseases in dogs diagnosed in the backlands of Paraiba are described. The necropsy records of 1,205 of dogs were reviewed. In 354 cases (29.38%) a history of clinical alterations of the nervous system were recorded. Two hundred and ninety six cases had a definitive diagnosis and 58 were inconclusive. Infectious diseases were observed in 59.60% (211/354) of cases representing the main cause of neurological disorders; 53% of the cases (186/354) were represented by viral diseases; 3.11% (11/354) were of parasitic etiology, 2.54% (9/354) were caused by bacteria and 1.41% (5/354) by fungi. Physical agents represented the second most important cause of CNS disorders with 9.89% (35/354) and tumors third cause with 5.93% (21/354). Other uncommon observed disorders were metabolic changes secondary to liver or kidney failure, accounting for 2.54% (9/354). Rare cases of congenital hydrocephalus were observed, 1.41% (5/354). The cases of neurological manifestations associated with vascular, degenerative and inflammatory noninfectious lesions, for many of which were specific cause was not established accounted for 4.24% (15/354); they were within the following disease categories: ischemic and hemorrhagic infarcts (6/15), vasculitis fibrinoide necrosis (5/15), intervertebral disc disease (2/15), granulomatous meningoencephalitis (1/15) and cholesterol granuloma (1/15). The central nervous system disorders represent an important cause of death or reason for euthanasia in dogs in the semiarid region of Paraiba. Clinical signs vary according to the agent involved, and the location and distribution of the lesions. The knowledge of the main agents that can affect the canine CNS it is important when making a list of differential diagnosis.(AU)

Deterioro neurológico progresivo como forma de presentación inusual del neurobehçet / Progressive neurological deterioration as unusual form of neuro-behçet presentation

La enfermedad de Behçet puede afectar a varios órganos y tiene dentro de sus características la presencia de úlceras recurrentes genitales, orales y la uveítis. El neurobehçet es una de las manifestaciones clínicas más serias e infrecuente de la enfermedad de Behçet y debe ser considerado en el diagnóstico diferencial con enfermedades inflamatorias, infecciosas o desmielinizante del sistema nervioso central. Este reporte de caso describe a una paciente con manifestaciones neurológicas aguda severas, lo cual es inusual en esta enfermedad(AU)

Behçet's disease can affect several organs and the presence of recurrent genital, oral and uveitis ulcers characterize it. Neuro-Behçet's is one of the most serious and uncommon clinical manifestations of Behçet's disease, so it should be taken into account in the differential diagnosis with inflammatory, infectious or demyelinating diseases of the central nervous system. This case report describes a female patient with acute severe neurological manifestations, unusual in this disease(AU)

Trastornos neurológicos graves en lactante con anemia megaloblástica / Severe neurological disorders in an infant with megaloblastic anemia

La anemia megalobástica es un trastorno madurativo de los precursores eritroides y mieloides causado por déficit de vitamina B12, ácido fólico, o ambos. Es poco común en la infancia y su prevalencia se desconoce por ser una enfermedad poco frecuente. Se presenta el caso de una lactante, cuya madre tuvo una alimentación precaria durante el embarazo y la lactancia, que prolongó la lactancia materna exclusiva más de 6 meses. La paciente tuvo un desarrollo psicomotor normal hasta los 7 meses cuando comenzó a perder las habilidades ganadas y llegó a tener trastornos neurológicos graves, por lo que se consideró por neurología como una enfermedad progresiva del SNC. Al examen físico se encontró palidez en piel y mucosas, pérdida de los reflejos cocleopalpebrales bilaterales, ausencia de reflejo visual, hipertonía de los 4 miembros e hiperreflexia. Se realizaron exámenes de laboratorio por genética buscando errores congénitos del metabolismo, enfermedades mitocondriales u otras enfermedades como Tay Sachs, gangliosidosis tipo 1 y leucodistrofia metacromática, todas con resultado negativo. Se encontraron valores bajos de hemoglobina, con macrocitosis, por lo que se realizó dosificación de vitamina B12 que se encontró disminuida. Se concluyó como una anemia megaloblástica por déficit de vitamina B12.Se inició tratamiento sustitutivo con vitamina B12, con lo que se logróuna rápida recuperación clínica desde los primeros días de tratamiento y la remisión total de los trastornos neurológicos. El déficit de vitamina B12 debe ser sospechado en lactantes con anemia y trastornos neurológicos especialmente si la alimentación de la madre antes, durante el embarazo y la lactancia no es adecuada(AU)

Megaloblastic anemia is a madurative disorder of erythroid and myeloid precursors caused by deficiency of vitamin B12 and/or folic acid. It is rare in childhood and its prevalence is unknown because it is a rare disease.We report the case of a 14 month old infant with a normal psychomotor development (DPM) until 7 months after which began to lose the gained skills and had severe neurological disorders to such a degree of being studied by neurology by a progressive central nervous system disease. At physical examination pale skin and mucous membranes, loss of bilateral cocleopalpebral reflexes, no visual reflection of the 4 limbs hypertonia, hyperreflexia were found. Laboratory tests were performed reporting hemoglobin of 76 g/L, genetic tests to search for inborn errors of metabolism,mitochondrial diseases and other diseases as Tay Sachs, Gangliosidosis type 1, metachromatic leukodystrophy, all were negative. The dosage of vitamin B12 was 91 pg/mL, and megaloblastic anemia was diagnosed. At the beginning of the treatment with vitamin B12 there was a rapid clinical recovery, the severe neurological disorders were completely reversing. Vitamin B12 deficiency should be suspected in infants with anemia and neurological disorders especially if the mother´s feeding before and during pregnancy and lactation is not adequate(AU)

Erythromelalgia: a cutaneous manifestation of neuropathy?

Abstract: The low prevalence of erythromelalgia, classified as an orphan disease, poses diagnostic and therapeutic difficulties. The aim of this review is to be an update of the specialized bibliography. Erythromelalgia is an infrequent episodic acrosyndrome affecting mainly both lower limbs symmetrically with the classic triad of erythema, warmth and burning pain. Primary erythromelalgia is an autosomal dominant inherited disorder, while secondary is associated with myeloproliferative diseases, among others. In its etiopathogenesis, there are neural and vascular abnormalities that can be combined. The diagnosis is based on exhaustive clinical history and physical examination. Complications are due to changes in the skin barrier function, ischemia and compromise of cutaneous nerves. Because of the complexity of its pathogenesis, erythromelalgia should always be included in the differential diagnosis of conditions that cause chronic pain and/or peripheral edema. The prevention of crisis is based on a strict control of triggers and promotion of preventive measures. Since there is no specific and effective treatment, control should focus on the underlying disease. However, there are numerous topical and systemic therapies that patients can benefit from.

Síndrome de Guillain Barré secundario a infección por Chikungunya: reporte de un caso / Guillain Barré syndrome secondary to Chikungunya infection: report of a case

El síndrome de Guillain Barré (SGB) es la neuropatía paralítica aguda más común y más severa, con una incidencia anual entre 0.4 y 4 casos por 100000 habitantes. El riesgo de cursar con la enfermedad se incrementa con la edad y es más frecuente en hombres que en mujeres. Las formas desmielinizante y axonal ocurren en proporciones variadas a través de las diferentes regiones geográficas y usualmente es precedido por una infección que induce una respuesta autoinmune aberrante cuyo blanco son los nervios periféricos y sus raíces nerviosas. La progresión aguda de la debilidad de las extremidades, frecuentemente con afección del sensorio y los nervios craneales de evidencia a la primera o segunda semana luego de la estimulación inmune, siendo el pico del déficit a las 2 a 4 semanas. Cuando los pacientes presentan una parálisis rápidamente progresiva debe realizarse el diagnóstico de GB lo antes posible.(AU)