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Rev. Cient. CRO-RJ (Online) ; 7(1): 16-23, Jan-Apr 2022.
Article in English | LILACS, BBO | ID: biblio-1382133


Introduction: Children with cerebral palsy (CP) are at high risk of developing oral diseases. Objective: To propose an early dental care protocol for infants with CP. Materials and Methods: A computerized systematic search was performed in the PubMed, Scopus and Embase electronic databases for relevant articles. An early dental care protocol was then proposed for infants with cerebral palsy focused on comprehensive care with a multidisciplinary approach and effective health promotion by caregivers. Results: Fifteen published papers were included in the present literature review and protocol proposal. The protocol comprised the following topics: First dental visit, aspects related to the appointment, oral hygiene recommendations, dietary recommendations and recommendations for the prevention and control of harmful oral habits. The first dental visit should occur prior to the eruption of the teeth. As a special group, it is important to determine the affective bond between the patient and caregiver who will receive the oral health care recommendations. During the clinical examination, the correct positioning and stabilization of the infant is important for the control of involuntary movements and the minimization of swallowing difficulties. Counseling with regards to adequate oral hygiene, a healthy diet and the prevention of harmful oral habits is important to the prevention of dental diseases. Children with oral-motor motility problems and feeding difficulties should be referred to therapeutic follow-up. Due to the neuropsychomotor disorders often found in cerebral palsy, affected children are more vulnerable to oral diseases. Thus, oral health care must be performed as early as possible by the parents/caregivers of these children. Conclusion: Individuals with cerebral palsy are at greater risk of developing oral problems. Thus, oral health programs starting in early childhood and targeting the specificities of these individuals is a strategy for minimizing the occurrence of such problems and the associated burden.

Introdução: Crianças com Paralisia Cerebral (PC) apresentam um alto risco para o desenvolvimento de doenças bucais. Objetivo: Propor um protocolo de atendimento odontológico precoce para lactentes com PC. Materiais e Métodos: Foi realizada uma busca computadorizada sistemática nas bases de dados eletrônicas PubMed, Scopus e Embase. Também foi desenvolvida uma proposta de protocolo de atendimento a bebês com Paralisia Cerebral com foco no cuidado integral, abordagem transdisciplinar e promoção efetiva da saúde pelos cuidadores. Resultados: Foram incluídos 15 artigos publicados na literatura científica. O protocolo desenvolvido é composto pelos seguintes tópicos: Primeira consulta odontológica, tempo ideal da consulta, recomendações de higiene bucal, recomendações sobre dieta e recomendações sobre prevenção e controle dos hábitos bucais. A primeira visita ao dentista deve ser feita antes da erupção do primeiro dente. Como um grupo especial, é importante determinar o vínculo afetivo entre o paciente e o cuidador que receberá as recomendações de cuidados com a saúde bucal. Durante o exame clínico, a correta posição e estabilização do bebê na cadeira odontológica é importante para controlar os movimentos involuntários e reduzir a dificuldade de deglutição. Recomendações quanto à higiene bucal adequada, alimentação saudável e prevenção de hábitos bucais deletérios são importantes para prevenir o desenvolvimento de doenças bucais. Crianças com problemas de motilidade oral-motora e dificuldades de alimentação devem ser encaminhadas para acompanhamento terapêutico. Devido à presença de distúrbios neuropsicomotores normalmente presentes na Paralisia Cerebral, as crianças afetadas são mais vulneráveis às doenças bucais. Assim, os cuidados com a saúde bucal devem ser realizados o quanto antes pelos pais/responsáveis. Conclusão: Indivíduos com PC apresentam maior risco de desenvolver doenças bucais e programas de saúde bucal iniciados na primeira infância e direcionados às suas especificidades podem ser uma estratégia para minimizar as consequências que possam vir a acontecer.

Nervous System Diseases , Cerebral Palsy , Dental Care for Disabled , Disabled Children
Int. j. cardiovasc. sci. (Impr.) ; 35(3): 364-372, May-June 2022. tab
Article in English | LILACS | ID: biblio-1375640


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.

Humans , Male , Female , Middle Aged , Aged , Aged, 80 and over , Attention , Aging , Executive Function , Cognitive Dysfunction , Heart Failure , Memory , Anxiety , Cross-Sectional Studies , Depression , Nervous System Diseases , Neuropsychology
Protein & Cell ; (12): 6-25, 2022.
Article in English | WPRIM | ID: wpr-929158


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.

Animals , Cytoskeleton/genetics , Humans , Nervous System Diseases/genetics , Neurons/enzymology , Signal Transduction , p21-Activated Kinases/metabolism
Arq. neuropsiquiatr ; 79(12): 1116-1122, Dec. 2021. tab, graf
Article in English | LILACS | ID: biblio-1355708


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.

Humans , Hemophilia A/complications , Nervous System Diseases/prevention & control , Brazil , Factor VIII , Central Nervous System , Retrospective Studies , Peripheral Nervous System/physiopathology , Hemorrhage , Nervous System Diseases/etiology
Arq. neuropsiquiatr ; 79(10): 900-903, Oct. 2021. graf


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.

Humans , Pruritus , Peripheral Nervous System , Arm , Radiculopathy , Electromyography , Muscles , Nervous System Diseases
Arq. neuropsiquiatr ; 79(10): 929-932, Oct. 2021. graf
Article in English | LILACS | ID: biblio-1345313


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.

Humans , History, 19th Century , History, 20th Century , Neurosciences , Nervous System Diseases/genetics , Neurology , Neurologists
Rev. colomb. anestesiol ; 49(3): e600, July-Sept. 2021. tab, graf
Article in English | LILACS, COLNAL | ID: biblio-1280183


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.

Humans , Female , Pregnancy , Cesarean Section , Glioblastoma , Headache , Anesthesia, Epidural , Anesthetics , Neurosurgery , Tuberous Sclerosis , Brain , Rare Diseases , Parturition , Hemorrhage , Hospitals , Hypesthesia , Neoplasms , Nervous System Diseases
Rev. cuba. anestesiol. reanim ; 20(2): e688, 2021. tab, graf
Article in Spanish | LILACS, CUMED | ID: biblio-1289349


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)

Humans , Stroke/mortality , Respiration, Artificial/adverse effects , Secondary Care , Cross-Sectional Studies , Prospective Studies , Intensive Care Units , Nervous System Diseases/complications
Arq. bras. med. vet. zootec. (Online) ; 73(4): 916-922, Jul.-Aug. 2021. ilus
Article in English | LILACS, VETINDEX | ID: biblio-1285267


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)

Animals , Female , Cattle , Arnold-Chiari Malformation/veterinary , Arnold-Chiari Malformation/diagnostic imaging , Cerebellar Vermis/diagnostic imaging , Congenital Abnormalities/veterinary , Nervous System Diseases/diagnostic imaging
Medicina (B.Aires) ; 81(2): 208-213, June 2021. graf
Article in Spanish | LILACS | ID: biblio-1287272


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.

Humans , Drug-Related Side Effects and Adverse Reactions , Neoplasms/drug therapy , Nervous System Diseases , Immune Checkpoint Inhibitors , Immunologic Factors/therapeutic use , Immunotherapy
Rev. Assoc. Méd. Rio Gd. do Sul ; 65(2): 01022105, Abr. - Jun. 2021.
Article in Portuguese | LILACS | ID: biblio-1367450


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

Humans , Child, Preschool , Epidemiologic Studies , Genetics , Nervous System Diseases
Rev. bras. ter. intensiva ; 33(2): 325-325, abr.-jun. 2021. tab
Article in English, Portuguese | LILACS | ID: biblio-1289082


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.

Humans , Male , Middle Aged , SARS-CoV-2/isolation & purification , COVID-19/complications , Nervous System Diseases/virology , Pneumonia, Viral/diagnosis , Seizures/virology , Hospitalization , Nervous System Diseases/physiopathology
J. oral res. (Impresa) ; 10(2): 1-9, abr. 30, 2021. tab
Article in English | LILACS | ID: biblio-1381739


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.

Humans , Male , Female , Child , Adolescent , Adult , Middle Aged , Aged , Aged, 80 and over , Young Adult , Guillain-Barre Syndrome/complications , Nervous System Diseases/etiology , Peru/epidemiology , Phonation , Acids , Oral Health , Epidemiology, Descriptive , Cross-Sectional Studies , Cranial Nerves , Facial Expression , Facial Paralysis
Arq. neuropsiquiatr ; 79(1): 30-37, Jan. 2021. tab, graf
Article in English | LILACS | ID: biblio-1153133


ABSTRACT Background: Multimorbidity is common among adults and associated with socioeconomic deprivation, polypharmacy, poor quality of life, functional impairment, and mortality. Objectives: To identify the frequency of multimorbidity among older adults inpatients with neurological disorders (NDs), stratify clusters of chronic comorbidities associated with NDs in degrees, and verify whether multimorbidity was associated with demographic data, readmission, long length of hospital stay (LOS), and hospital mortality in this population. Methods: We enrolled patients aged ≥60 years successively admitted to a tertiary medical center with NDs between January 1, 2009, and December 31, 2010. Results: Overall, 1,154 NDs and 2,679 comorbidities were identified among 798 inpatients aged ≥60 years (mean: 75.76±9.12). Women comprised 435 (54.51%) of patients. Multimorbidity was detected in 92.61% (739) of patients, with a mean of 3.88±1.67 (median: 4.0), ranging from 2 to 10 chronic diseases. Patients with epilepsy, dementia, and movement disorders had the highest degrees of clusters of chronic morbidities (>50% of them with ≥5 chronic disorders), followed by those with cerebrovascular and neuromuscular disorders. Multimorbidity was associated with long LOS (p<0.001) and readmission (p=0.039), but not with hospital mortality (p=0.999). Conclusions: Multimorbidity was preponderant among older adults inpatients with NDs, and NDs had a high degree of associated chronic comorbidities. Multimorbidity, but not isolated NDs, was associated with readmission and long LOS. These results support ward-based, neurohospitalist-directed, interdisciplinary care for older adults inpatients with NDs to face multimorbidity.

RESUMO Introdução: A multimorbidade é comum entre idosos e está associada a privação socioeconômica, polifarmácia, má qualidade de vida, déficit funcional e mortalidade. Objetivos: Identificar a frequência da multimorbidade entre pacientes idosos hospitalizados com doenças neurológicas (DN), estratificar combinações de comorbidades crônicas associadas às DN em graus e verificar se a multimorbidade foi associada a dados demográficos, readmissão, longo tempo de internação (TDI) e mortalidade hospitalar nessa população. Métodos: Foram incluídos pacientes com ≥60 anos sucessivamente admitidos com DN em um centro médico terciário entre 1º de janeiro de 2009 e 31 de dezembro de 2010. Resultados: Um total de 1.154 DN e 2.679 comorbidades foram identificados entre 798 pacientes com idade ≥60 anos (média: 75,76±9,12). Mulheres representaram 435 (54,51%) dos pacientes. A multimorbidade foi detectada em 92,61% (739) dos pacientes (média de 3,88±1,67; mediana de 4), variando de 2 a 10 doenças crônicas. Pacientes com epilepsia, demência e distúrbios do movimento apresentaram os maiores graus de morbidades crônicas (>50% deles com ≥5 doenças crônicas), seguidos por doenças cerebrovasculares e neuromusculares. A multimorbidade foi associada com longo TDI (p<0,001) e readmissão (p=0,039), mas não com mortalidade hospitalar (p=0,999). Conclusões: A multimorbidade foi preponderante entre os pacientes idosos internados com DN, as quais tiveram altos graus de comorbidades crônicas. A multimorbidade, mas não as DN isoladas, foi associada a readmissões e longo TDI. Esses resultados respaldam uma assistência interdisciplinar para idosos hospitalizados com DN em enfermarias lideradas por neurologistas hospitalistas para enfrentar a multimorbidade.

Humans , Female , Aged , Multimorbidity , Nervous System Diseases/epidemiology , Quality of Life , Chronic Disease , Inpatients , Length of Stay
Chinese Journal of Biotechnology ; (12): 3757-3780, 2021.
Article in Chinese | WPRIM | ID: wpr-921463


The human intestinal flora is a highly diverse ecosystem composed of trillions of bacteria. The imbalance of the flora is related to a variety of diseases. The intestinal flora interacts with the nervous system bidirectionally in many ways through the gut-brain axis. It causes neuroimmune inflammatory response, dysfunction of gut mucosa and blood-brain barrier, direct stimulation of the vagus nerve, spinal nerve of the enteric nervous system, and the neuroendocrine hypothalamus-pituitary-adrenal axis, causing neurological disorders. The metabolites of the intestinal microbial community also play a role. This article summarizes the characteristics of the altered intestinal flora and intervention measures in autism spectrum disorder, multiple sclerosis, Parkinson's disease, epilepsy, Guillain-Barré syndrome, Alzheimer's disease, neuromyelitis optica, hepatic encephalopathy, amyotrophic lateral sclerosis, schizophrenia, depression, chronic fatigue syndrome, Huntington's disease and stroke. The current research on intestinal flora is still in its infancy, and very few studies were carried out on causality and the underlying mechanisms, which prevents the development of precise flora-based clinical intervention measures. It is expected the research on intestinal flora would lead to novel approaches for treatment of some neurological disorders.

Autism Spectrum Disorder , Brain , Gastrointestinal Microbiome , Humans , Microbiota , Nervous System Diseases
Article in English | WPRIM | ID: wpr-888606


BACKGROUND@#Particulate matter (PM), a major component of ambient air pollution, accounts for a substantial burden of diseases and fatality worldwide. Maternal exposure to PM during pregnancy is particularly harmful to children's health since this is a phase of rapid human growth and development.@*METHOD@#In this review, we synthesize the scientific evidence on adverse health outcomes in children following prenatal exposure to the smallest toxic components, fine (PM@*RESULTS@#Maternal exposure to fine and ultrafine PM directly and indirectly yields numerous adverse birth outcomes and impacts on children's respiratory systems, immune status, brain development, and cardiometabolic health. The biological mechanisms underlying adverse effects include direct placental translocation of ultrafine particles, placental and systemic maternal oxidative stress and inflammation elicited by both fine and ultrafine PM, epigenetic changes, and potential endocrine effects that influence long-term health.@*CONCLUSION@#Policies to reduce maternal exposure and health consequences in children should be a high priority. PM

Adult , Air Pollutants/adverse effects , Air Pollution/prevention & control , Animals , Cardiovascular Diseases/chemically induced , Child Health , Child, Preschool , Disease Models, Animal , Endocrine System Diseases/chemically induced , Epigenomics , Female , Humans , Immune System Diseases/chemically induced , Infant , Infant, Newborn , Male , Maternal Exposure/adverse effects , Nervous System Diseases/chemically induced , Oxidative Stress , Particle Size , Particulate Matter/adverse effects , Placenta , Pregnancy , Pregnancy Outcome/epidemiology , Prenatal Exposure Delayed Effects/epidemiology , Respiratory Tract Diseases/chemically induced , Young Adult
Article in Chinese | WPRIM | ID: wpr-888171


Astragali Radix is a traditional Chinese herbal medicine with a long history, which has the functions of tonifying Qi and promoting urination and granulation. Astragalosides are the main effective components of Astragali Radix, and more than 40 triterpenoid saponins have been obtained from Astragalus membranaceus and its related plants, mainly including astragalosides Ⅰ-Ⅷ, isoastragalosides Ⅰ, Ⅱ, and Ⅳ, acetylastragalosides, and soyasaponins. Astragalosides have a wide range of biological activities, such as immunomodulation, antioxidation, and neuroprotection. Nervous system diseases seriously affect people's quality of life, threaten human physical and mental health, and impose a burden on families and society. As natural drugs, astragalosides have good preventive and therapeutic effects on central nervous system diseases. This paper reviews the main pharmacological effects and mechanisms of astragalosides in the treatment of multiple sclerosis, Parkinson's disease, Alzheimer's disease, and cerebral ischemic stroke and proposes the research prospects and potential problems, aiming to provide reference for the clinical application and basic research of astragalosides.

Astragalus Plant , Astragalus propinquus , Drugs, Chinese Herbal , Humans , Nervous System Diseases , Quality of Life , Saponins/pharmacology
Article in Chinese | WPRIM | ID: wpr-879889


Coronavirus disease 2019 (COVID-19) has become a worldwide pandemic and can occur at any age, including children. Children with COVID-19 can develop the clinical symptoms of multiple systems, among which symptoms of the nervous system have been reported increasingly, and thus it is particularly important to understand COVID-19-associated neurological damage in children. This article reviews the mechanisms and types of COVID-19-associated neurological damage in children.

COVID-19 , Child , Humans , Nervous System Diseases , Pandemics , SARS-CoV-2