Uso de cannabidiol para el control de síntomas refractarios en síndromes convulsivos y enfermedades neurodegenerativas / Use of cannabidiol for the control of refractory symptoms in convulsive syndromes and neurodegenerative diseases

Como parte de las terapias alternativas para el control de síntomas refractarios en enfermedades avanzadas destaca el uso de cannabidiol. Este se ha estudiado en patologías como enfermedad de Alzheimer, Parkinson y trastornos convulsivos. Los síndromes convulsivos están presentes en todos los grupos etarios. Dentro de este, la epilepsia es refractaria hasta en un 40 % de los pacientes, quienes han demostrado disminución en la frecuencia de convulsiones con el uso concomitante de cannabidiol y antiepilépticos convencionales, con efectos secundarios leves, como diarrea y somnolencia. Con el objetivo de determinar el uso del cannabidiol para el control de síntomas neurológicos refractarios en pacientes con síndromes convulsivos y enfermedades neurodegenerativas, se realizó una búsqueda bibliográfica en Pubmed, Scopus y Embase. Se incluyeron metaanálisis, artículos originales, revisiones sistemáticas y bibliográficas, y documentos de la Organización Panamericana de la Salud, publicados entre 2017 y 2022. Los efectos del cannabidiol lo convierten en una alternativa, adicional a la terapéutica convencional, para el control de síntomas en trastornos neurológicos, disminuyendo de forma sostenida el número total de episodios con un perfil de seguridad aceptable. Existe limitada información respecto al uso de cannabidiol en enfermedades neurodegenerativas, por lo que no se ha evidenciado su efectividad

As part of the alternative therapies for the control of refractory symptoms in advanced diseases, the use of cannabidiol stands out. It has been studied in pathologies such as Alzheimer's disease, Parkinson's disease, and convulsive disorders. Convulsive syndromes are present in all age groups. Within this group, epilepsy is refractory in up to 40 % of patients, who have shown a decrease in the frequency of seizures with the concomitant use of cannabidiol and conventional antiepileptics, with mild side effects such as diarrhea and drowsiness. To determine the use of cannabidiol for the control of refractory neurological symptoms in patients with seizure syndromes and neurodegenerative diseases, a literature search was performed in PubMed, Scopus, and Embase. Meta-analyses, original articles, systematic and literature reviews, and documents from the Pan American Health Organization, published between 2017 and 2022, were included. The effects of cannabidiol make it an alternative, in addition to conventional therapeutics, for symptom control in neurological disorders, sustainably decreasing the total number of episodes with an acceptable safety profile. There is limited information regarding the use of cannabidiol in neurodegenerative diseases, the reason its effectiveness has not been demonstrated.

Cognitive Alterations in Elderly Patients with Heart Failure'

Abstract Background: Among the various pathologies that affect the elderly, Heart Failure (HF) stands out. Recently, an attempt has been made to verify the existence of cognitive impairment associated with HF. Objectives: To compare the cognitive performance of elderly people with heart failure with that of age-matched individuals without this pathology. Check the existence of marked impairment in some cognitive functions in the clinical group. Methods: The sample consisted of 78 elderly people, whose inclusion criterion was the presence of HF and no HF (control group); age over 60 years, both sexes, and any level of education. The control group consisted of 37 individuals (with a median age of 68 years - Interquartile range of 12) and the HF group, with 41 individuals (with a median age of 67 years - Interquartile range of 11). The subjects were matched in terms of education level, with a predominance of elderly people with 0 to 4 years of education (65.9% in the Clinical Group and 59.5% in the Control Group). Eleven neuropsychological tests covering cognitive functions were used: attention, language, memory, mood, and executive function. Statistical analysis was performed using SPSS software, version 23, with a significance level of 5%. The Chi-square test and the Mann-Whitney test were applied. Results: The results showed significant differences between the groups, mainly in executive functions, which include the ability to plan, switch, and recall previously stored information. Conclusion: Our study showed differences between the cognitive performance of elderly people with HF and elderly people without HF. The main alteration was found in the so-called executive functions, attention, and memory.

Toxoplasmosis congénita con enfermedad neurológica severa en un hospital de referencia del Perú / Congenital toxoplasmosis with severe neurological disease in a referral hospital in Peru

RESUMEN El propósito del presente estudio fue describir las características epidemiológicas, clínicas, y terapéuticas de pacientes con diagnóstico de toxoplasmosis congénita (TC) con enfermedad neurológica severa. Se revisaron las historias clínicas de los pacientes menores de 1 año con serología IgM positiva para Toxoplasma gondii y compromiso encefálico, ocular y/o auditivo. El estudio se realizó en el Instituto Nacional de Salud del Niño San Borja (INSN-SB) en Lima, Perú. Se evaluaron a 21 pacientes con diagnóstico de TC, el 57,1% fueron del sexo femenino y la mediana de edad al momento del diagnóstico fue 3,1 meses (RIC: 1,7-7,3). Las principales manifestaciones del sistema nervioso central fueron hidrocefalia (76,2%), calcificaciones intracraneales (52,4%), microcefalia (42,9%), y convulsiones (25,6%); la manifestación ocular más frecuente fue la coriorretinitis (38,1%). En conclusión, 64% de los casos de TC tuvieron una o más manifestaciones de enfermedad neurológica severa.

ABSTRACT The aim of this study was to describe the epidemiological, clinical, and therapeutic characteristics of patients diagnosed with congenital toxoplasmosis (CT) with severe neurological disease. We reviewed the medical records of patients under 1 year of age with positive IgM test for Toxoplasma gondii and brain, eye, and/or hearing involvement. This study was carried out at the Instituto Nacional de Salud del Niño San Borja (INSNSB), Lima, Peru. Twenty-one patients diagnosed with CT were evaluated; 57.1% were female, and the median age at diagnosis was 3.1 months (IQR: 1.7-7.3). The main central nervous system manifestations were hydrocephalus (76.2%), intracranial calcifications (52.4%), microcephaly (42.9%), and convulsions (25.6%); the most frequent ocular manifestation was chorioretinitis (38.1%). In conclusion, 64% of CT cases had one or more manifestations of severe neurological disease.

The p21-activated kinases in neural cytoskeletal remodeling and related neurological disorders

The serine/threonine p21-activated kinases (PAKs), as main effectors of the Rho GTPases Cdc42 and Rac, represent a group of important molecular switches linking the complex cytoskeletal networks to broad neural activity. PAKs show wide expression in the brain, but they differ in specific cell types, brain regions, and developmental stages. PAKs play an essential and differential role in controlling neural cytoskeletal remodeling and are related to the development and fate of neurons as well as the structural and functional plasticity of dendritic spines. PAK-mediated actin signaling and interacting functional networks represent a common pathway frequently affected in multiple neurodevelopmental and neurodegenerative disorders. Considering specific small-molecule agonists and inhibitors for PAKs have been developed in cancer treatment, comprehensive knowledge about the role of PAKs in neural cytoskeletal remodeling will promote our understanding of the complex mechanisms underlying neurological diseases, which may also represent potential therapeutic targets of these diseases.

Prioritising maternal mental health and infant neurodevelopment research in Africa ­ A call for action amidst the backdrop of the COVID­19 pandemic

No abstract

Pattern of neurological disorders managed in neurology clinics in Kano, Nigeria

To determine the pattern of neurological disorders managed in some neurology clinics in Kano, Nigeria. Materials and Methods: A five-year retrospective descriptive survey (2016-2020) of patients with neurological disorders attending neurology out-patient clinics at three selected hospitals in Kano metropolis. A research proforma was used to collect information on socio-demographic and health characteristics of the participants. Case folders and outpatient registers were used as a source of information about the patients. Data obtained was analyzed using IBM Statistical package for social science (SPSS) version 20 and results presented using descriptive statistics of frequency and percentage. Results: Out of the 433 cases with neurological disorders surveyed, 58% were adult and 42% were pediatric. Males (54.3%) were found to be more affected. Majority (83.4%) of the participants were from an urban area, with married (79.7%) individuals mostly affected. Stroke was the most prevalent (33.7%) neurological disorder. Hypokinesia (5.1%) and hyperkinesia(3%) as a primary and secondary movement disorder respectively were the most prevalent forms of movement disorder. Psychotic symptoms, weakness, paresthesia, fever, vomiting and convulsion were the most common manifestations that comes with the neurological disorders. Of the common neurological disorders, stroke was most associated with motor (56.2%), sensory (36.4%) and psychiatric (43.5%) manifestations. Conclusion: Paediatric age group contributed close to half of neurological disorders. Stroke was the most prevalent form of neurological disorder and most associated with motor, sensory and psychiatric manifestations. Hypokinesia and hyperkinesia had close prevalence and were the most common primary and secondary movement disorders respectively.

Factor VIII prophylactic therapy reduces neurological complications in patients with Hemophilia A / Terapia profilática com Fator VIII reduz complicações neurológicas em paciente com Hemofilia A

ABSTRACT Background: Bleeding in hemophiliacs can cause complications in the central and peripheral nervous system (CNS and PNS). The incidence of intracranial hemorrhage has reduced after the introduction of prophylactic treatment with factor VIII or IX, but the benefits of this therapy have not yet been evaluated on PNS complications. Objective: The aim of this study was to determine the prevalence of neurological complications in hemophiliacs and verify the effect of prophylactic therapy in these patients, including PNS disorders. Methods: We retrospectively evaluated the prevalence of CNS and PNS disorders caused by bleeding in hemophiliacs seen at the Hemocentro Regional Norte, Ceará, Brazil, from 1992 to 2018, and we compared the incidence in different periods (before and after the introduction of prophylactic treatment in 2011). Results: Of 75 hemophilia A patients evaluated (4.61/100.000 population), 13.3% (n=10) had either CNS (n=5) or PNS (n=5) disorders secondary to bleeding. Patients submitted to factor VIII replacement prophylactic therapy were less likely to have CNS events: from 1992 to 2011, 5 of 63 patients had CNS disease, while from 2011 to 2018, there were no new cases (p=0.0181). From 2011 to 2018, 5 PNS events occurred in patients without prophylactic therapy, whereas none occurred in those covered by prophylactic therapy (5/20 versus 0/29, p=0.0081). Conclusions: The prevalence of neurological complications in hemophiliacs in our cohort is similar to other studies. Similar to CNS, prophylactic therapy also reduces the risk of PNS complications. This is the first report in the literature showing this benefit.

RESUMO Antecedentes: O sangramento em hemofílicos causa complicações no sistema nervoso central e periférico (SNC e SNP). A incidência de hemorragia intracraniana diminuiu após a introdução da profilaxia com fator VIII ou IX, entretanto esse benefício ainda não foi avaliado no SNP. Objetivo: O objetivo deste estudo foi determinar a prevalência de complicações neurológicas em hemofílicos, verificando o efeito da terapia profilática também no SNP. Métodos: Avaliamos retrospectivamente a prevalência de complicações neurológicas causadas ​​por sangramentos em hemofílicos atendidos no Hemocentro Regional Norte, Ceará, Brasil, de 1992 a 2018, comparando a incidência em diferentes períodos (antes e depois da introdução do tratamento profilático em 2011). Resultados: Foram avaliados 75 pacientes com hemofilia A (4,61/100 mil habitantes). Deles, 13,3% (n=10) tinham distúrbios do SNC (n=5) ou do SNP (n=5) secundários a hemorragias. Os pacientes submetidos à terapia profilática com fator VIII apresentaram menor probabilidade de eventos do SNC: de 1992 a 2011, cinco de 63 pacientes apresentaram hemorragia no SNC, enquanto de 2011 a 2018 não ocorreram novos casos (p=0,0181). De 2011 a 2018, cinco eventos no SNP ocorreram entre pacientes sem terapia profilática, e nenhum ocorreu entre aqueles cobertos pela profilaxia (5/20 × 0/29, p=0,0081). Conclusões: A prevalência de complicações neurológicas em hemofílicos em nossa coorte é similar à de outros estudos. Assim como no SNC, a terapia profilática também reduz o risco de complicações no SNP. Este é o primeiro relato na literatura a mostrar esse benefício.

Caracterização das publicações fonoaudiológicas sobre síndromes neurológicas (2009-2019) / Characterization of speech therapy publications in the field of neurological syndromes (2009-2019) / Caracterización de publicaciones de logopedia em el campo de los síndromes neurológicos (2009-2019)

Introdução: Caracterização das publicações fonoaudiológicas sobre síndromes neurológicas. Objetivo: Identificar síndromes pesquisadas, aspectos clínicos abordados, metodologia utilizada e número de publicações em 4 periódicos brasileiros entre 2009-2019. Método: Revisão Integrativa da literatura. Resultados: A Fonoaudiologia participa ativamente deste campo, com publicações distribuídas de modo equilibrado ao longo do período estudado, com 36 síndromes pesquisadas, 8 delas com estudos mais sistemáticos; a maioria sob a forma de estudo de caso, abordando, sobretudo, questões ligadas aos procedimentos de avaliação. Conclusão: Na Fonoaudiologia, há, no período visitado, uma produção constante de artigos ligados ao campo das síndromes neurológicas. Portanto, este campo tem presença reconhecida nesta área. Estudos são necessários para uma caracterização mais abrangente do "estado da arte".

Introduction: Characterization of speech therapy publications on neurological syndromes. Objective: Identify syndromes surveyed, clinical aspects addressed, methodology used and number of publications in 4 Brazilian journals between 2009-2019. Methods: Integrative literature review. Results: Speech therapy actively participates in this field, with publications distributed in a balanced way over the period studied, with 36 syndromes surveyed, 8 of them with more systematic studies; the majority in the form of a case study, mainly addressing issues related to the evaluation procedures. Conclusion: In Speech Therapy, there is, during the period visited, a constant production of articles related to the field of neurological syndromes. Therefore, this field has a recognized presence in this area. Studies are needed for a more comprehensive characterization of the "state of the art".

Introduccion: Caracterización de publicaciones de logopedia sobre síndromes neurológicos. Objetivo: Identificar síndromes encuestados, aspectos clínicos abordados, metodología utilizada y número de publicaciones en 4 revistas brasileñas entre 2009-2019. Metodos: Revisión bibliográfica integradora. Resultados: La logopedia participa activamente en este campo, con publicaciones distribuidas de forma equilibrada durante el período estudiado, con 36 síndromes encuestados, 8 de ellos con estudios más sistemáticos; la mayoría en forma de estudio de caso, que abordan principalmente cuestiones relacionadas con los procedimientos de evaluación. Conclusión: En Logopedia, durante el período visitado, existe una producción constante de artículos relacionados con el campo de los síndromes neurológicos. Por tanto, este campo tiene una presencia reconocida en este ámbito. Se necesitan estudios para una caracterización más completa del "estado del arte".

Neurophysiological assessment of brachioradial pruritus patients / Avaliação neurofisiológica de pacientes com prurido braquiorradial

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

RESUMO Antecedentes: O prurido constitui queixa frequente e desafiadora na prática dermatológica. O primeiro estudo a relacionar prurido com neuropatia foi de Wartenberg, em 1943, que associou à "neuropatia do nervo cutâneo antebraquial posterior". Em 1968, Waisman descreveu pacientes com queixas recorrentes de prurido em membros superiores no verão, sendo denominado, então, "brachioradial summer pruritus". Atualmente, esse prurido é denominado como prurido braquiorradial (PBR). O PBR é caracterizado por prurido crônico, geralmente bem localizado, de longa duração e sem anormalidades cutâneas aparentes. Doenças neurológicas, tanto centrais, esclerose múltipla ou acidente vascular cerebral como do sistema nervoso periférico, estão associadas a prurido. Objetivo: Investigar os dermátomos sintomáticos pela eletroneuromiografia (ENMG). Métodos: Foram estudados 46 pacientes com diagnóstico dermatológico de PBR com a eletroneuromiografia dos membros superiores. Resultado: Foram avaliados 46 pacientes com queixa dermatológica de C5 a C8 somando 113 áreas dermatoméricas sintomáticas. Observou-se que 39 (85%) pacientes apresentavam comprometimento radicular, sendo que em 28 (60%) houve concordância plena entre as queixas e os achados da ENMG (p=0,015), e que 80% dos pacientes com queixa em território de C7 e 47% em C6 apresentavam comprometimento radicular no mesmo nível. Conclusões: As queixas mais frequentes foram as correspondentes aos territórios de C6 e C7, sendo que 47 e 80%, respectivamente, apresentaram alteração na ENMG nesses miótomos. Dessa forma, evidenciou-se correlação entre comprometimento do sistema nervoso periférico (i.e., radicular) com PBR.

A journey through the history of Neurogenetics / Uma jornada pela história da Neurogenética

ABSTRACT Since the late 19th century, when several inherited neurological disorders were described, the close relationship between Neurology and heredity were well documented by several authors in a pre-genetic era. The term Neurogenetics came to integrate two large sciences and clinical practices: Neurology and Genetics. Neurogenetics is the emerging field that studies the correlation between genetic code and the development and function of the nervous system, including behavioral traits, personality and neurological diseases. In this historical note, a timeline shows the main events and contributors since the first reports of neurogenetic diseases until the current days. In the recent years, neurologists are experiencing much broader use of new genetic diagnosis techniques in clinical practice. Thus, new challenges are arising in diagnostic approach, ethical considerations, and therapeutic options. This article aims to summarize the main historical hallmarks of Neurogenetics, from the pre-DNA era to the present, and the future directions of the field.

RESUMO Desde o final do século XIX, quando diversas doenças neurológicas hereditárias foram descritas, a associação entre neurologia e hereditariedade foi bem documentada por vários autores na era pré-genética. O termo Neurogenética integra dois campos da ciência e da prática clínica: Neurologia e Genética. A Neurogenética é o campo que estuda a correlação entre o código genético e o desenvolvimento e a função do sistema nervoso, incluindo comportamento, personalidade e doenças neurológicas. Nesta nota histórica, a linha do tempo mostra os principais eventos e pesquisadores desde os primeiros relatos de doenças neurogenéticas até os dias atuais. Recentemente, neurologistas estão se deparando com maior uso de técnicas diagnósticas genéticas na prática clínica; portanto, novos desafios surgem na abordagem diagnóstica, nas considerações éticas e na terapêutica. Este artigo almeja resumir os principais marcos históricos da Neurogenética, desde a era pré-DNA até o presente, e os caminhos futuros desse campo de conhecimento.

Anesthetic management in neurological disorders: is combined spinal-epidural a suitable option? Case report / Manejo anestésico en trastornos neurológicos: ¿es la anestesia espinal-epidural una alternativa apropiada? Reporte de caso

Abstract Tuberous sclerosis (TSC) is a rare disease with multi-systemic involvement, predominantly neurological. Little evidence exists about the anesthetic management of patients with this disorder, particularly in pregnant women. This article discusses a case of a patient with TSC admitted to our hospital for the delivery of a twin gestation. Twenty-four hours after surgery, the patient presented left-side facial-brachial hypoesthesia and headache. A brain CT revealed a right frontal cortical bleeding tumor, which was diagnosed as glioblastoma multiforme. The patient was discharged 15 days after admission and a neurosurgical approach was suggested.

Resumen La esclerosis tuberosa es una enfermedad poco frecuente asociada con compromiso multisistémico, principalmente neurológico. Es poca la evidencia sobre el manejo anestésico de los pacientes con este trastorno, en particular las mujeres embarazadas. En este artículo presentamos el caso de una paciente con esclerosis tuberosa ingresada en nuestro hospital para el parto de una gestación gemelar. Veinticuatro horas después de la cirugía, la paciente presentó hipoestesia facial y braquial izquierda y cefalea. La tomografía cerebral mostró un tumor cortical sangrante en el lóbulo frontal derecho, diagnosticado como glioblastoma multiforme. La paciente fue dada de alta 15 días después de su ingreso y, con recomendación de manejo por neurocirugía.

Tongue strength as a clinical feature of oral health in neurological patients: a systematic review / La fuerza lingual como una característica clínica de la salud oral en pacientes neurológicos: una revisión sistemática

Objective:Current oral health assessment has a comprehensive view of the relationship between hard and soft tissues of the mouth as seen by orthodontics and prosthodontics in a healthy population. Despite knowing the influence this relationship has on functional outcomes such as swallowing and mastication, motor evaluation of soft tissue such as the tongue is still scarce. This lack of knowledge is even greater in individuals with a neurological condition. In this sense, the measurement of lingual strength has been addressed by some research as a key element accompanying oral rehabilitation in healthy populations. Acknowledging the importance of tongue strength in oral biomechanics, the Iowa Oral Performance Instrument (IOPI) has become a gold standard instrument. The purpose of this article was to search for scientific studies on tongue strength using the IOPI as a research tool in populations with neurological conditions, to know about its inclusion in the clinical practice and comprehensive oral health rehabilitation in this population. Material and Methods: A systematic search in five major databases was carried out based on the PRISMA Protocol. Searches were conducted in the PubMed, Medline, Lilacs, Web of Science and MedCarib databases including articles from 2007 to 2020. To generate the search in each database, three main constructs were developed: (1) "tongue strength IOPI"; (2) "Swallowing Disorders"; (3) "Neurological Diseases". Results:152 studies were identified, 14 were included in the final review. The PEDro scale showed great heterogeneity in the level of evidence between the studies with only 5 RCTs and only two of them on lingual strength training. Conclusion: The IOPI was used mainly to measure tongue strength and only 36% as a clinical training device, which could contribute to improving oral health. The stroke was the most represented (79%).IIIISU.

Objetivo: La evaluación actual de la salud bucal tiene una visión integral de la relación entre los tejidos duros y blandos de la boca según se observa en práctica de la ortodoncia y la prostodoncia en la población sana. Apesar de conocer la influencia que tiene esta relación en resultados funcionales como la deglución y la masticación, la evaluación motora de los tejidos blandos como la lengua es aún escasa. Esta falta de conocimiento es aún mayor en personas con una condición neurológica. En este sentido, la medición de la fuerza lingual ha sido abordada por algunas investigaciones como un elemento clave que acompaña a la rehabilitación oral en población sana. Reconociendo la importancia de la fuerza lingual en la biomecánica bucal, el Iowa Oral Performance Instrument (IOPI) se ha convertido en un instrumento estándar de medición. El propósito de este artículo fue buscar estudios científicos sobre la fuerza lingual en pacientes neurológicos utilizando el IOPI como herramienta de investigación, para conocer su inclusión en la intervención clínica y rehabilitación integral de la salud bucal en esta población. Material y Métodos: Se realizó una búsqueda sistemática en cinco grandes bases de datos basada en el Protocolo PRISMA. Las búsquedas fueron realizadas en las bases PubMed, Medline and Lilacs, Web of Science y MedCarib incluyendo artículos desde 2007 al 2020. Para generar la búsqueda en cada base de datos, se desarrollaron tres constructos: (1) "tongue Strength IOPI"; (2) "Swallowing Disorders"; (3) "Neurological Diseases". Resultados: Se identificaron 152 estudios, 14 se incluyeron en la revisión final. La escala PEDro se evidenció gran heterogeneidad en el nivel de evidencia entre los estudios con sólo 5 RCT y únicamente dos de ellos sobre entrenamiento de fuerza lingual. El IOPI se utilizó principalmente para medir la fuerza lingual y sólo en un 36% como dispositivo de entrenamiento clínico, lo cual pudiese contribuir a mejorar la salud oral. Conclusion: El ACV fue el más representado (79%). Se necesita adicionar evidencia sobre el entrenamiento de la fuerza lingual en individuos con afecciones neurológicas como la enfermedad de Parkinson dada la creciente prevalencia reportada por la literatura científica.

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)

First occurrence of Arnold Chiari type II malformation and associated abnormalities in a Gir calf produced in vitro from Brazil - case report / [Primeira ocorrência de malformação de Arnold Chiari tipo II e anormalidades associadas em uma bezerra Gir de produção in vitro no Brasil - relato de caso]

This study characterized the clinical, radiological, ultrasound, and necroscopic findings of a case of Arnold-Chiari type II malformation in a Gir breed calf from Brazil. The animal was hospitalized at sixty days of age, in permanent sternal recumbency, cutaneous appendix at the 4th lumbar vertebra and kyphoscoliosis of the caudal and lumbosacral thoracic spine. Radiographic examination of the spine and skull revealed spina bifida and suspected occipital hypoplasia. Upon examination of myelography with an injection of lumbar and atlantooccipital contrast, it was possible to visualize the meningocele at the 4th lumbar vertebra region and findings at the rhombencephalon level of increased regional pressure with failure to fill the contrast in the posterior fossa, in the presence of clear demarcation of the circumvolutions of the cerebral cortex and the subarachnoid space of the cervical spinal cord. Ultrasonographic examination of the cerebellum showed an insinuation of the cerebellar worm through the foramen magnum. The animal did not show changes in complete blood count, biochemical series, and cerebrospinal fluid and was negative for Pestivirus. There was a worsening of the clinical conditions and the animal died. This malformation of unknown etiology must be studied as a differential diagnosis of the nervous system disorders.(AU)

Este estudo caracterizou os achados clínicos, radiológicos, ultrassonográficos e necroscópicos de um caso de malformação de Arnold-Chiari tipo II em uma bezerra Gir no Brasil. O animal foi hospilatizado aos 60 dias de idade, apresentando decúbito esternal permanente, apêndice cutâneo na altura da quarta vértebra lombar e cifoescoliose da coluna vertebral torácica caudal e lombossacra. Ao exame radiográfico da coluna e do crânio, foram observadas espinha bífida e suspeita de hipoplasia occipital. Ao exame de mielografia com injeção de contraste lombar e atlanto-occipital, foi possivel visualizar a meningocele na altura da quarta vértebra lombar e achados em nível rombencefálico de aumento da pressão regional com falha de preenchimento do contraste na fossa posterior, na presença de nítida demarcação das circunvoluções do córtex cerebral e do espaço subaracnoide da medula espinhal cervical. Ao exame ultrassonográfico do cerebelo, foi observada insinuação do verme cerebelar através do forame magno. O animal não apresentou alterações em hemograma completo, série bioquímica e fluido cérebro-espinhal e foi negativo para Pestivirus. Houve uma piora do quadro clínico e o animal morreu. Essa malformação de etiologia desconhecida deve ser estudada como um diagnóstico diferencial.(AU)

Caracterização dos pacientes com doenças neurogenéticas atendidos em um ambulatório universitário do Sul do Brasil / Characterization of patients with neurogenetic diseases treated at a university outpatient clinic in southern Brazil

RESUMO Introdução: As doenças genéticas, em crianças, abrangem uma ampla gama de condições e causas variadas. Objetivo: Verificar a frequência de doenças neurogenéticas em pacientes pediátricos de 0 a 5 anos atendidos em um ambulatório universitário, e os fatores associados. Métodos: estudo de coorte retrospectivo, realizado com pacientes pediátricos atendidos em 2017, em um ambulatório materno infantil da Universidade do Sul de Santa Catarina. Os dados foram obtidos dos prontuários físicos dos pacientes, exclusivos do serviço de neurogenética. Resultados: Foram analisados 82 prontuários de crianças acompanhadas pelo serviço de neurogenética em 2017. A idade das crianças variou entre um mês e um dia e 5,42 anos, sendo 28,0% com um ano completo. A consanguinidade dos pais foi reportada em cinco (6,1%) casos. As principais intercorrências maternas citadas durante a gestação resumiram-se em infecção por citomegalovírus, rubéola, toxoplasmose (2,4%) e infecção do trato urinário (19,5%). Os registros de complicações no ato do nascimento, foram PCR (2,4%), anóxia (1,2%) e Apgar baixo (3,7%). Quanto às intercorrências do neonato no pós-parto, as principais citações foram infecções (19,5%), traumas (20,7%), cirurgia (36,6%) e EIM (30,5%), sendo que um mesmo paciente pode ter apresentado mais de uma intercorrência. Como queixa principal, 23 (28,05%) dos pais referiam atraso no desenvolvimento neuropsicomotor das crianças. Como diagnóstico principal, 15,9% constaram Síndrome de Down e 12,3% Transtorno do Espectro Autista. Conclusão: O conhecimento do perfil de crianças acometidas por doenças neurogenéticas é imprescindível para a obtenção do diagnóstico precoce, do tratamento efetivo e melhor prognóstico da doença. PALAVRAS-CHAVE: Doenças do sistema nervoso, genética, pré-escolar, estudos epidemiológicos

ABSTRACT Introduction: Genetic diseases in children encompass a wide range of conditions and varied causes. Purpose: To verify the frequency of neurogenetic diseases in pediatric patients aged 0-5 years seen at a university outpatient clinic, and associated factors. Methods: A retrospective cohort study, carried out with pediatric patients seen at a maternal and child clinic at Universidade do Sul de Santa Catarina in 2017. Data were obtained from the patients' physical records, exclusive to the neurogenetics service. Results: 82 medical records of children followed by the neurogenetics service in 2017 were analyzed. The children's age ranged from 01 month and one day to 5.42 years, 28.0% over one year old. Parental consanguinity was reported in five (6.1%) cases. The main maternal complications mentioned during pregnancy were cytomegalovirus infection, rubella, toxoplasmosis (2.4%) and urinary tract infections (19,5%). The records of complications at birth were CRP (2.4%), anoxia (1.2%) and Low Apgar (3.7%). As for the complications of the newborn in the postpartum period, the main citations were infections (19.5%), trauma (20.7%), surgery (36.6%) and IEM (30.5%), but a patient may have had more than one complication. As the main complaint, 23 (28.05%) of the parents reported delay in their children's neuropsychomotor development. As the main diagnosis, 15.9% had Down Syndrome and 12.3% had Autistic Spectrum Disorder. Conclusion: Knowledge of the profile of children affected by neurogenetic diseases is essential to obtain an early diagnosis, effective treatment and a better prognosis of the disease. KEYWORDS: Nervous system diseases, genetics, preschool, epidemiologic studies

Reacciones adversas a medicamentos inhibidores del punto de control inmunitario / Adverse drug reactions associated with immune checkpoint inhibitors

Resumen El manejo de las reacciones adversas inducidas por los inhibidores del punto de control inmunitario (IPCI) en cáncer, demanda un trabajo multidisciplinario. Revisamos las causas y el curso clínico de las consultas e internaciones debidas a reacciones adversas de los IPCI entre septiembre de 2015 y julio de 2019 en el Instituto Alexander Fleming. Se registraron los datos demográficos, diagnóstico oncológico, reacción adversa y su grado, requerimiento de internación, tratamiento, mortalidad y evaluación de la reexposición. Se registraron 124 reacciones adversas por IPCI en 89 pacientes. Sesenta y ocho recibían monoterapia y 21 terapia combinada. Las manifestaciones cutáneas fueron las más frecuentes, seguidas de las generales, endocrinas (con mayor frecuencia hipotiroidismo), colitis, neumonitis, neurológicas y hepatitis. Fueron graves (grado ≥ 3), 26 toxicidades en 25 pacientes. Se internaron 15, y 6 de ellos requirieron terapia intensiva. Un caso fue fatal. Recibieron glucocorticoides 34 (12 de ellos por vía intravenosa). Un paciente recibió micofenolato y uno inmuno globulina endovenosa. En 20 se discontinuó el tratamiento. Ocho se reexpusieron y uno de ellos debió suspender definitivamente. Se presenta en esta serie de casos nuestra experiencia con el diagnóstico y tratamiento de las reacciones adversas de una familia de drogas cuya utilización ha crecido en los últimos años.

Abstract The management of patients with immune-related adverse events (irAEs) frequently demands a multidisciplinary approach. We reviewed the causes and clinical course of medical visits and admissions at the Instituto Alexander Fleming due to irAEs between September 2015 and July 2019. Demographic data, diagnosis, toxicity and its severity, requirement of admission, treatment, mortality, and evaluation of the re-administration of immunotherapy were collected. We found 124 irAEs in 89 patients. Sixty-eight of them received monotherapy (76.4%) and 21 (23.6%) combination of drugs. Cutaneous manifestations were the most frequent cause of irAEs, followed by general manifestations, endocrine dysfunctions (hypothyroidism the most frequent), colitis, pneumonitis, neurologic dis orders, and hepatitis. In 26 adverse events (in 25 patients), severity grade was ≥ 3. Fifteen were admitted and 6 required ICU admission. One patient died. Thirty-four received glucocorticoids, 12 of them by intravenous route. One patient received mycophenolate and one IVIG. In 20, the treatment was discontinued; 8 were re-exposed, with definitive discontinuation in one patient. In this case series we report our experience in the diagnosis and management of adverse reactions related to a family of drugs whose use has grown in recent years.

Disfunção neurológica associada à COVID-19 / Neurological dysfunction associated with covid-19

RESUMO A COVID-19 foi declarada pandemia pela Organização Mundial de Saúde no dia 11 de março de 2020. O quadro clínico apresenta predominantemente sintomatologia respiratória, no entanto, na literatura atual, têm sido descritas diversas manifestações neurológicas associadas à infeção por SARS-CoV-2. Os autores apresentam o caso clínico de um homem de 45 anos internado por pneumonia com resultado positivo para SARS-CoV-2, sem antecedentes neurológicos, que, ao décimo sexto dia de internamento, apresentou alteração súbita do estado de consciência acompanhada de desvio conjugado do olhar para a direita e mioclonias da face e da região torácica à esquerda, seguidas de crise convulsiva tônico-clônica generalizada, associadas à hemiparesia esquerda persistente. Do estudo realizado salienta-se a existência de RT-PCR para SARS-CoV-2 no líquido cefalorraquidiano positiva. O doente apresentou evolução clínica com melhoria gradual, tendo o desfecho sido favorável.

ABSTRACT COVID-19 was declared a pandemic by the World Health Organization on March 11, 2020. The clinical presentation is predominantly respiratory symptoms; however, in the current literature, several neurological manifestations associated with SARS-CoV-2 infection have been described. The authors present the clinical case of a 45-year-old man hospitalized for pneumonia with a positive test result for SARS-CoV-2, without a neurological history, who, on the sixteenth day of hospitalization, presented a sudden change in his state of consciousness accompanied by conjugated right gaze deviation and myoclonus of the face and thoracic region to the left, followed by generalized tonic-clonic seizures associated with persistent left hemiparesis. The present study highlights a positive RT-PCR test for SARS-CoV-2 in cerebrospinal fluid. The patient progressed with gradual improvement, and the outcome was favorable.

Craniofacial and neurological manifestations in patients with Guillain-Barré syndrome / Manifestaciones neurológicas craneofaciales en pacientes con síndrome de Guillain-Barré

Introducción: Among the broad range of symptoms of the Guillain-Barré Syndrome (GBS), patients can present craniofacial manifestations. Consequently, the participation of the dental surgeon in the evaluation of the stomatognathic system using a multidisciplinary approach model is very important. Objective: To identify the craniofacial and neurological manifestations of the Guillain-Barré Syndrome in patients from three hospitals in the district of Lambayeque, Peru, in 2019. Material and Methods: A descriptive, prospective, and cross-sectional study was carried out in 59 patients diagnosed with GBS by means of a clinical evaluation carried out by previously calibrated neurologists (k=0.911). Additionally, an oral evaluation of the oral hygiene index, the periodontal status, and the oral pH was carried out. Data were processed using frequency distribution tables. Results: 4 4.07% of the patients presented at least one functional alteration, such as unilateral limitation for facial expressions (25.42%), dysphagia (18.64%), alteration in phonation (11.86%), and loss of the sense of taste (6.78%), showing involvement of the corresponding cranial nerves. In addition, unfavorable conditions of oral hygiene and acid pH were observed in some cases, which proved adverse for the periodontal structures of the patients. Conclusion: The study found craniofacial manifestations of GBS, such as cases of unilateral facial palsy, dysphagia, difficulty in phonation, and loss of the sense of taste, due to the alteration of the cranial nerves that command these functions. In addition, poor oral hygiene, and the presence of acidic pH in some patients created an adverse environment for the integrity of the periodontal structures

Introducción: El Síndrome de Guillain-Barré (SGB) dentro de su amplia sintomatología puede presentar manifestaciones craneofaciales, por lo que resulta importante la participación del cirujano dentista en la evaluación del sistema estomatognático desde un modelo de abordaje multidisciplinario. Objetivo: Identificar las manifestaciones neurológicas craneofaciales del Síndrome de Guillain-Barré en pacientes de tres establecimientos de salud de Lambayeque, Perú en el año 2019. Materiales y Metodos: Se realizó un estudio descriptivo, prospectivo y transversal, con 59 pacientes diagnosticados con SGB, mediante una evaluación clínica realizada por médicos neurólogos previamente calibrados (k=0,911). Adicionalmente se realizó una evaluación bucodental del índice de higiene oral, del estado periodontal y del pH bucal, siendo procesados los datos mediante tablas de distribución de frecuencias. Resultados: El 44,07% de pacientes presentaron por lo menos una alteración funcional, identificando limitación unilateral para expresiones faciales (25.42%), disfagia (18.64%), alteración en la fonación (11.86%) y pérdida del sentido del gusto (6.78%), evidenciando compromiso de los pares craneales correspondientes. Además se observaron condiciones desfavorables de higiene oral y de pH ácido en algunos casos, que resultaron adversas para las estructuras periodontales de los pacientes. Conclusión: Fueron encontradas manifestaciones craneofaciales del SGB, identificando casos con parálisis facial unilateral, disfagia, dificultad para la fonación y pérdida del sentido del gusto, debido a la alteración de los pares craneales que comandan esas funciones. Además las condiciones desfavorables de higiene oral y la presencia de pH ácido en algunos casos, propiciaron un ambiente adverso para la integridad de las estructuras periodontales.