Definitions, phenomenology, diagnosis, and management of the disorders of laughter and crying in amyotrophic lateral sclerosis (ALS): Consensus from ALS and Motor Neuron Disease Scientific Department of the Brazilian Academy of Neurology / Definições, fenomenologia, diagnóstico e manejo das desordens do riso e do choro em esclerose lateral amiotrófica (ELA): Consenso do Departamento Científico de ELA e Doença do Neurônio Motor da Academia Brasileira de Neurologia

Abstract The spectrum of neuropsychiatric phenomena observed in amyotrophic lateral sclerosis (ALS) is wide and not fully understood. Disorders of laughter and crying stand among the most common manifestations. The aim of this study is to report the results of an educational consensus organized by the Brazilian Academy of Neurology to evaluate the definitions, phenomenology, diagnosis, and management of the disorders of laughter and crying in ALS patients. Twelve members of the Brazilian Academy of Neurology - considered to be experts in the field - were recruited to answer 12 questions about the subject. After exchanging revisions, a first draft was prepared. A face-to-face meeting was held in Fortaleza, Brazil on 9.23.22 to discuss it. The revised version was subsequently emailed to all members of the ALS Scientific Department from the Brazilian Academy of Neurology and the final revised version submitted for publication. The prevalence of pseudobulbar affect/pathological laughter and crying (PBA/PLC) in ALS patients from 15 combined studies and 3906 patients was 27.4% (N = 1070), ranging from 11.4% to 71%. Bulbar onset is a risk factor but there are limited studies evaluating the differences in prevalence among the different motor neuron diseases subtypes, including patients with and without frontotemporal dementia. Antidepressants and a combination of dextromethorphan and quinidine (not available in Brazil) are possible therapeutic options. This group of panelists acknowledge the multiple gaps in the current literature and reinforces the need for further studies.

Resumo O espectro de fenômenos neuropsiquiátricos observados na ELA é amplo e não completamente entendido. Desordens do riso e do choro estão entre as manifestações mais comuns. O objetivo deste estudo é relatar os resultados de um Consenso organizado pela Academia Brasileira de Neurologia para avaliar definições, fenomenologia, diagnóstico, e manejo dos distúrbios do riso e do choro em pacientes com ELA. Doze membros da Academia Brasileira de Neurologia - considerados experts na área - foram recrutados para responder 12 questões na temática. Depois da verificação das revisões, um primeiro manuscrito foi preparado. Após, foi realizado um encontro presencial em Fortaleza, Brasil, em 23/09/2022, para discussão do conteúdo. A versão revisada foi posteriormente enviada por e-mail para todos os membros do Departamento Científico de DNM/ELA da Academia Brasileira de Neurologia e a versão final revisada foi submetida para publicação. A prevalência da síndrome pseudobulbar em pacientes com ELA em 15 estudos combinados com 3906 pacientes foi de 27,4% (n = 1070), variando entre 11,4% e 71%. Início bulbar é um fator de risco, mas há limitados estudos avaliando as diferenças em prevalência entre os diferentes subtipos de Doença do Neurônio Motor, incluindo pacientes com e sem Demência Frontotemporal. Antidepressivos e uma combinação de dextrometorfana e quinidina (indisponíveis no Brasil) são opções terapêuticas possíveis. Esse grupo de panelistas reconhece as múltiplas demandas não atendidas na literatura atual e reforça a necessidade de futuros estudos.

Colic and sleep outcomes of nonpharmacological intervention in infants with infantile colic: systematic review and metaanalysis

SUMMARY OBJECTIVE: The aim of this study was to systematically review the colic and sleep outcomes of nonpharmacological intervention in infants with infantile colic and perform a meta-analysis of the available evidence. METHODS: The literature review for this systematic review was conducted between December 2022 and January 2023 using five electronic databases, namely PubMed, CINAHL, Scopus, Web of Science, and ULAKBİM. Published articles were scanned using MeSH-based keywords. Only randomized controlled trials conducted in the past 5 years were included. The data were analyzed using the Review Manager computer program. RESULTS: This meta-analysis included three studies involving a total of 386 infantile colic infants. After nonpharmacological treatment, it was found that infants with infantile colic reduced crying time (standardized mean difference: 0.61; 95%CI 0.29-0.92; Z=3.79; p=0.00002), improved sleep duration (standardized mean difference: 0.22; 95%CI −0.04 to 0.48; Z=1.64; p=0.10), and decreased crying intensity (mean difference: −17.24; 95%CI −20.11 to 14.37; Z=11.77; p<0.000001). CONCLUSION: According to the meta-analysis findings, it was determined that the risk of bias was low in the studies included and that nonpharmacological chiropractic, craniosacral, and acupuncture treatments applied to infantile colic infants in the three included studies reduced crying time and intensity and increased sleep duration.

A clinical evaluation of gelastic and dacrystic seizures: a multicenter study / Uma avaliação clínica das crises gelásticas e dacrísticas: um estudo multicêntrico

Abstract Background Gelastic seizures are extremely rare, short-lasting, unprovoked, and uncontrollable laughing attacks. We conducted this retrospective evaluation to determine whether these symptoms, manifesting in different forms, such as cheerful laughter, laughing, smiling, and sobbing had any value in terms of etiology or localization. Methods A total of 31 patients who exhibited bouts of laughing or crying and who were under follow-up between 2000 and 2019 at tertiary epilepsy centers were included in the study. Laughing seizures were divided into three groups in terms of semiology (i.e., laughter with mirth, laughter without mirth, and smile). Dacrystic seizures were accompanied by some gelastic seizures and were divided into two groups in terms of semiology (i.e., weeping loudly [motor and voice-sobbing] and crying). Results Of the 27 patients with laughing seizures, 12 had seizures that manifested with smiling, 7 had seizures that manifested with laughing and mirth, and 8 had seizures that manifested with laughter without mirth. Dacrystic-gelastic seizures were observed in four patients, among whom 2 patients had crying and laughter without mirth and 2 patients had weeping loudly and laughter without mirth episodes. Conclusion Gelastic and dacrystic seizures often suggest hypothalamic hamartomas, in the literature. This rare ictal behavior can originate from different cortical locations and lesions of a different nature. However, we found that gelastic seizures with smiling were a more homogenous group with regard to location in the temporal lobe, which we aimed to show by evaluating the patients included in this study.

Resumo Antecedentes Crises gelásticas são ataques de riso extremamente raros, de curta duração, não provocados e incontroláveis. Realizamos esta avaliação retrospectiva para determinar se esses sintomas, manifestando-se de diferentes formas, como riso alegre, riso, sorriso e soluço, tinham algum valor em termos de etiologia ou localização. Métodos Foram incluídos no estudo 31 pacientes que apresentavam crises de riso ou choro e que estavam em acompanhamento entre 2000 e 2019 em centros terciários de epilepsia. As crises de riso foram divididas em três grupos em termos de semiologia (ou seja, riso com alegria, riso sem alegria e sorriso). As crises dacrísticas foram acompanhadas por algumas crises gelásticas e foram divididas em dois grupos em termos de semiologia (ou seja, choro alto [motor e soluçar a voz] e choro). Resultados Dos 27 pacientes com crises de riso, 12 tiveram crises que se manifestaram com sorriso, 7 tiveram crises que se manifestaram com riso e alegria e 8 tiveram crises que se manifestaram com riso sem alegria. Crises dácristico-gelásticas foram observadas em quatro pacientes, sendo 2 pacientes com choro e riso sem alegria e 2 pacientes com choro alto e riso sem alegria. Conclusão Crises gelásticas e dacrísticas frequentemente sugerem hamartomas hipotalâmicos, na literatura. Este comportamento ictal raro pode ter origem em diferentes localizações corticais e lesões de natureza diversa. No entanto, verificamos que as crises gelásticas com sorriso foram um grupo mais homogêneo quanto à localização no lobo temporal, o que buscamos evidenciar avaliando os pacientes incluídos neste estudo.

Repercussões do choro dos bebês durante o processo de adaptação à creche / Repercussions of infants´s crying during the process of adaptation to day care / Repercusiones del llanto de bebés durante el proceso de adaptación a guardería

O presente estudo buscou compreender a percepção das mães e dos pais sobre o choro dos bebês no contexto de adaptação à creche. Participaram do estudo dez mães e dois pais e a idade dos bebês variou de dois meses e meio a quinze meses. A partir de análise de conteúdo qualitativa, os resultados apontaram uma preocupação quanto ao choro, considerando-o, às vezes, como uma dificuldade no processo de adaptação. A discussão salientou a naturalidade dessa reação manifestada pela criança durante esse momento, ressaltando-se a importância de se compreender o choro como uma forma de comunicação. É preciso observar outros aspectos ao refletir sobre como esse momento está sendo sentido pelo bebê, visto que a ausência do choro não garante o bem-estar. Dessa forma, compreende-se que o processo de adaptação contempla a tríade: criança, família e escola. Além disso, o choro pode estar relacionado aos diversos sentimentos vivenciados pelos envolvidos e a partir das relações estabelecidas entre eles. (AU)

The present study aimed to understand the perception of mothers and fathers about the crying of infants in the context of adaptation to early childhood education. Ten mothers and two fathers participated in the study, and the age of the infants ranged from two months and a half to fifteen months. Based on qualitative content analysis, the results indicated a concern about crying, sometimes considering it a difficulty in the adaptation process. The discussion emphasized the naturalness of this reaction manifested by the child during this moment, emphasizing the importance of understanding crying as a form of communication. In addition, it is necessary to observe other aspects when reflecting on how this moment is being felt by the infant, since the absence of crying does not guarantee well-being. In this way, it is understood that the process of adaptation contemplates the triad: child, family and school. In addition, crying may be related to the various feelings experienced by those involved and from the relationships established between them. (AU)

El presente estudio buscó comprender la percepción de las madres y de los padres sobre el llanto de los bebés en el contexto de adaptación a la guardería. Participaron del estudio diez madres y dos padres y la edad de los bebés varía de dos meses y medio a quince meses. A partir de análisis de contenido cualitativo, los resultados apuntaron una preocupación en cuanto al llanto, considerándolo, a veces, como una dificultad en el proceso de adaptación. La discusión subrayó la naturalidad de esa reacción manifestada por el niño durante ese momento, resaltando la importancia de comprender el llanto como una forma de comunicación. Además, hay que observar otros aspectos al reflexionar sobre cómo ese momento está siendo sentido por el bebé, ya que la ausencia del llanto no garantiza el bienestar. De esta forma, se comprende que el proceso de adaptación contempla la tríada: niño, familia y escuela. Además, el llanto puede estar relacionado a los diversos sentimientos vivenciados por los involucrados ya partir de las relaciones establecidas entre ellos. (AU)

Analgesic effect of kangaroo position method versus sucrose during heel punctures in newborn: randomized clinical trial / Efeitos analgésicos da posição canguru versus sacarose durante punções de calcâneo em recém-nascidos: ensaio clínico randomizado

ABSTRACT BACKGROUND AND OBJECTIVES: 25% sucrose and kangaroo position are effective for neonatal analgesia in single procedures and sucrose is also effective in repeated procedures. Behavioral and physiological responses and adverse effect on newborns submitted to kangaroo position or 25% sucrose for analgesia were compared in two heel punctures applied during the first hours of life. METHODS: Randomized clinical trial with 80 newborns and 40 mothers. Data were collected during two heel punctures. The kangaroo position was performed for three minutes before, during and three minutes after the punctures. Oral 25% sucrose (0.5 mL/kg) was administered two minutes before the punctures and the newborns were kept in a crib. Each data collection was divided into nine phases. The facial action, crying and heart rate data were analyzed using descriptive and comparative analyses. Facial actions, assessed by the Newborn Facial Coding System scale, was used to test the hypothesis. The frequency of gastric side effects was calculated. Ethical aspects were preserved. RESULTS: Facial actions did not differ (p>0.05) between groups in the two data collections. There were no relevant changes in heart rate between and within groups. The newborns in the kangaroo group cried more than in the sucrose group (p<0.05) in the second collection. In the administration of sucrose, more side effects were observed in the first (p=0.02) and second collection (p=0.007). CONCLUSION: The kangaroo position is as effective as the administration of 25% sucrose to relieve pain resulting from two repeated heel punctures.

RESUMO JUSTIFICATIVA E OBJETIVOS: Sacarose a 25% e posição canguru são efetivos para analgesia neonatal em procedimentos únicos e a sacarose também é efetiva em procedimentos repetidos. Comparou-se as respostas comportamentais e fisiológicas e o efeito adverso entre recém-nascidos submetidos a posição canguru ou sacarose a 25% na analgesia em duas punções de calcâneo, aplicadas durante as primeiras horas de vida. MÉTODOS: Ensaio clínico randomizado com 80 recém-nascidos e 40 mães. Os dados foram coletados durante duas punções de calcâneo. A posição canguru foi realizada por três minutos antes, durante e três minutos após as punções. A sacarose a 25% oral (0,5 mL/kg) foi administrada dois minutos antes das punções e os recém-nascidos permaneceram em berço. Cada coleta de dados foi dividida em nove fases. Os dados da mímica facial, choro e frequência cardíaca foram analisados por meio de análises descritivas e comparativas. A mímica facial, avaliada pela escala Newborn Facial Coding System, foi utilizada para testar a hipótese. A frequência dos efeitos adversos gástricos foi calculada. Os aspectos éticos foram preservados. RESULTADOS: A mímica facial não diferiu (p>0,05) entre os grupos nas duas coletas de dados. Não houve alterações relevantes na frequência cardíaca entre e intragrupos. Os recém-nascidos do grupo canguru choraram mais que no grupo sacarose (p<0,05) na segunda coleta. Na administração de sacarose, foram observados mais efeitos adversos, na primeira (p=0,02) e segunda coleta (p=0,007). CONCLUSÃO: A posição canguru é tão eficaz quanto a administração de sacarose a 25% para aliviar a dor decorrente de duas punções de calcâneo repetidas.

Involuntary Emotional Expression Disorder in a Patient With Toluene Leukoencephalopathy / Trastorno de expresión emocional involuntaria en un paciente con leucoencefalopatía por tolueno

ABSTRACT Objective: Inhalant users may develop toluene leukoencephalopathy, a devastating neuropsychiatric disorder. We present a case of toluene-induced damage to the corticospinal and the corticonuclear tracts, which presented with involuntary emotional expression dis-order. Methods: Case study of a 20-year-old man with a 3-year history of frequent solvent abuse was admitted to the Neuropsychiatry Unit of the National Institute of Neurology and Neurosurgery because "he could not speak or walk" but would keep "laughing and crying without reason". Results: Neuropsychiatric examination revealed pathological laughter and crying, facial and speech apraxia, a bilateral pyramidal syndrome, and lack of control of urinary sphincter. Magnetic resonance imaging revealed a highly selective bilateral damage to the pyramidal system and the somatosensory pathway. SPECT imaging showed left fronto-parietal hypoperfusion. Conclusions: This document provides support for the understanding of involuntary emotional expression disorders as a differential diagnosis in the clinical practice of psychiatrists, as well as the functional anatomy of these conditions.

RESUMEN Objetivo: Los usuarios de inhalantes pueden contraer leucoencefalopatía por tolueno, un trastorno neuropsiquiátrico devastador. Se presenta un caso de daño inducido por tolueno en el tracto corticoespinal y corticonuclear, que se manifestó con un trastorno involuntario de la expresión emocional. Métodos: Un varón de 20 años con antecedente de 3 años de abuso de solventes ingresó en la Unidad de Neuropsiquiatría del Instituto Nacional de Neurología y Neurocirugía porque «no podía hablar ni caminar¼ y presentaba episodios súbitos de risa y llanto sin razón aparente. Resultados: La valoración neuropsiquiátrica reveló risa y llanto patológicos, apraxia facial y fonatoria, síndrome piramidal bilateral y ausencia de control del esfínter urinario. La resonancia magnética cerebral mostró un daño bilateral muy selectivo del sistema piramidal y la vía somatosensorial. La imagen de tomografía computarizada por emisión monofotónica mostró hipoperfusión frontoparietal izquierda. Conclusiones: Este documento proporciona apoyo para la comprensión de los trastornos de la expresión emocional involuntaria como diagnóstico diferencial en la práctica clínica de los psiquiatras, así como de la anatomía funcional de estas condiciones.

"Catatrenia": un trastorno del sueño poco conocido: descripción de tres casos clínicos pediátricos / Catathrenia: unknown sleep disorder: report 3 cases in pediatric patients

Catatrenia (gemido nocturno) es una condición rara caracterizada por sonidos irregulares que ocurren durante el sueño. Los comportamientos ocurren intermitentemente durante cualquiera de las dos etapas de sueño, REM o NREM y se caracterizan por gemidos prolongados, a menudo muy fuertes, socialmente perturbadores, durante la expiración. Es poco conocido y espera más definición y estudios terapéuticos. Hay pocos reportes y en su mayoría de pacientes adultos. Se presentan 3 casos en pacientes pediátricos.

Catathrenia (nocturnal groaning) is a rare condition characterized by irregular sounds that occur during sleep. The behaviors occur intermittently during either REM or NREM sleep and are characterized by prolonged, often very loud, socially disruptive groaning sounds during expiration. It is poorly understood and awaits further definition and therapeutic studies. There are few reports mostly adult patients are presented below 3 cases in pediatric patients.

Breve historia de las lágrimas y el llanto

Las lágrimas y el llanto son fenómenos biológicos enmarcados en diferentes contextos sociales, culturales y de conocimiento a lo largo de los años. Este artículo es una revisión sobre la construcción de las concepciones alrededor de las lágrimas y el llanto. Se exploran las formulaciones en el tema desde las civilizaciones más antiguas, pasando por la discusión alrededor del origen de las lágrimas (corazón vs. cerebro), así como los hallazgos con la introducción de la disección anatómica y la experimentación fisiológica. Finalmente, se exponen las posturas evolucionistas y psicológicas del llanto para estudiar su funcionalidad en la supervivencia y su papel en las relaciones sociales. El objetivo de este es presentar, en forma sucinta, los aspectos históricos de las lágrimas y el llanto.

SUMMARY Tears and crying are biological phenomena framed in different social and cultural contexts throughout the years. This article is a review of the construction of different conceptions around tears and crying. We explore proposals on the subject from the oldest civilizations, going through the discussion around the origin of tears (heart vs. brain), and the findings with the introduction of anatomical dissection and physiological experimentation. Finally, the evolutionary and psychological positions of crying are exposed, to study its functionality in survival and its role in social relations. The aim of this article is to present, succinctly, what have been the tears and crying through the history.

Neuropsiquiatría del síndrome de Susac: a propósito de un caso / Neuropsychiatry of Susac Syndrome: a Case Report

RESUMEN El síndrome de Susac es una entidad clínica poco frecuente, posiblemente mediada por un proceso autoinmune; la tríada clásica se compone de retinopatía, disminución en la agudeza auditiva y síntomas neuropsiquiátricos (encefalopatía). Hay pocos casos descritos con sintomatología neuropsiquiátrica como la sintomatología principal. Presentamos un caso de síndrome de Susac, que corresponde a una mujer de 34 arios, con predominio de sintomatologia neuropsiquiátrica, caracterizada por un síndrome de Klüver-Bucy parcial, un síndrome apático, risa y llanto patológico y alteraciones cognitivas de predominio atencional; dichos síntomas mejoraron cualitativamente con el uso de terapia inmunológica. Este caso revela la importancia de las manifestaciones neuropsiquiátricas como presentación clínica en pacientes con entidades neurológicas.

ABSTRACT Susac syndrome is a rare clinical condition, possibly mediated by an autoimmune process; the classic triad is composed of retinopathy, decreased hearing acuity and neuropsychiatric symptoms (encephalopathy). There are few cases reported with neuropsychiatric symptoms as the main manifestation. We present a case of Susac syndrome in a 34-year-old female with a predominance of neuropsychiatric symptoms, characterised by partial Klüver-Bucy syndrome, apathy syndrome, pathological laughter and crying, and cognitive dysfunction predominantly affecting attention, which showed a qualitative improvement with the use of immunological therapy. This case report highlights the importance of neuropsychiatric manifestations as clinical presentation in patients with neurological conditions.

Intervenção de enfermagem-primeiro banho do recém-nascido: estudo randomizado sobre o comportamento neonatal / Intervención de enfermería-primer baño del recién nacido: estudio aleatorizado sobre el comportamiento neonatal / Nursing intervention-first bath of the NB: a randomized study on neonatal behavior

Resumo Objetivo Avaliar os efeitos da intervenção Enfermagem-primeiro banho sobre o choro e o sono do recém-nascido. Métodos Ensaio clínico randomizado controlado realizado no alojamento conjunto do Hospital Universitário do Espírito Santo (Brasil). A amostra constituiu-se de 33 neonatos a termo. O grupo experimental composto por 18 recém-nascidos recebeu a técnica intervenção de enfermagem-primeiro banho. As variáveis dependentes foram a presença de choro e o tempo de sono após o banho do RN a Intervenção-Primeiro Banho foi definida como variável independente. As variáveis de controle relacionadas ao recém-nascido foram: idade gestacional; peso ao nascimento; peso antes do banho; perda ponderal; dor neonatal; saturação; sinais vitais; temperatura do ambiente; temperatura da água; tempo do banho; e tempo do cuidado corporal por formulário específico após 24 horas de nascimento. Utilizou-se a Escala de Avaliação do Estado de Sono e Vigília, adaptada de Brazelton, a escala de NIPS para avaliação da dor neonatal. Resultados Os recém-nascidos do grupo intervenção do estudo dormiram cerca de 180 minutos, não apresentaram choro durante o experimento, e a avaliação da escala de dor neonatal foi menor. Conclusão A intervenção de enfermagem-primeiro banho pode apresentar melhora no estado comportamental dos recém-nascidos.

Resumen Objetivo Evaluar los efectos de la intervención enfermería-primer baño sobre el llanto y el sueño del recién nacido. Métodos Ensayo clínico aleatorizado controlado, realizado en la internación conjunta del Hospital Universitario de Espírito Santo (Brasil). La muestra consistió en 33 neonatos a término. El grupo experimental compuesto por 18 recién nacidos recibió la técnica intervención de enfermería-primer baño. Las variables dependientes fueron la presencia de llanto y tiempo de sueño después del baño del RN, la intervención-primer baño fue definida como variable independiente. Las variables de control relacionadas con el recién nacido fueron: edad gestacional, peso al nacer, peso antes del baño, pérdida ponderal, dolor neonatal, saturación, signos vitales, temperatura del ambiente, temperatura del agua, tiempo del baño y tiempo del cuidado corporal por formulario específico luego de 24 horas del nacimiento. Se utilizó la Escala de evaluación del estado del sueño y vigilia, adaptada de Brazelton, la escala de NIPS para evaluar el dolor neonatal. Resultados Los recién nacidos del grupo experimental del estudio durmieron cerca de 180 minutos, no presentaron llanto durante el experimento y la evaluación de la escala de dolor neonatal fue menor. Conclusión La intervención de enfermería-primer baño puede presentar mejoras en el estado de comportamiento de los recién nacidos. Número REBEC: U1111-1239-4388

Abstract Objective To assess the effects of nursing intervention-first bath on NB crying and sleep. Methods Randomized controlled clinical trial conducted at the joint accommodation of the University Hospital of the state of Espírito Santo (Brazil). Sample consisted of 33 full-term infants. The experimental group of 18 NBs received the nursing intervention-first bath technique. The dependent variables were the presence of crying and sleep time after the NB's bath. First bath intervention was defined as an independent variable. The control variables related to the NB were: gestational age; birth weight; weight before bath; weight loss; neonatal pain; saturation; vital signs; room temperature; water temperature; bath time; and time of body care by form after 24 hours of birth. We used the Brazelton Sleep and Wake Status Assessment Scale, the NIPS scale for assessing neonatal pain. Results The NBs in the study intervention group slept for about 180 minutes, did not cry during the experiment, and the neonatal pain scale assessment was lower. Conclusion Nursing intervention-first bath may improve NBs' behavioral state. REBEC number: U1111-1239-4388

Approach to infantile colic in primary care

Infantile colic is a common self-limiting condition that causes significant distress to parents and caregivers. There is no clear cause, gold standard remedy or preventative action. The role of the family physician is to rule out sinister causes while providing counselling and reassurance for parents. The mainstay of management is parental support and reassurance while looking out for red flags in the baby such as fever, lethargy, distended abdomen and failure to thrive. This article provides a framework to approaching infantile colic and practical pointers to share with parents.

Infantile Colic: An Update

Context: Infantile colic is self-limiting condition but it can be a cause of anxiety for parents and challenge for doctors. The challenge for thedoctors lies in correct identification of the condition and appropriate management. The objective of this review article is to summarize thepathophysiology, treatment options and outcome in infantile colic so that clinicians can have a fair idea about the condition, recentupdates and future prospects.Evidence: A search of the Cochrane Library, PubMed, and Google Scholar was made using the key words “Infant colic”, Infantile colic”,“excessive crying in infants”. All the materials were analyzed and summarized.Results: At present, infantile colic is an area of clinical research both in terms of etiology and treatment. Various etiological theories havebeen proposed but none of them are strong enough to completely describe the condition. Various treatment agents are being tried forcolic like counseling and behavioral modification, dietary modification, lactase and probiotic supplementation, pain relieving agents, andchiropathy. Proper counseling of the parents is the first line of management at present. Simethicone has no role in decreasing thesymptoms of colic and Dicyclomine is not recommended in children younger than six months. No specific recommendations have beenmade on the use of pain relieving agents and manipulative therapies in colic. At present strong evidence is lacking regarding the use ofprobiotics, lactase supplementation and dietary modification.Conclusion: Counseling of parents about the benign nature of the condition is considered first line for now until an effective treatment isestablished. Other treatment options are prescribed on a case-based manner, and based on the parental perception of the condition.

Principales aspectos neurofisiológicos, clínicos, y genéticos, en una recién nacida con Síndrome de Cayler / Clinical, genetic, and neurophysiological main features in a newborn girl with Cayler Syndrome

En los neonatos, la hipoplasia o agenesia congénita del músculo depresor del ángulo de la boca (depresor anguli oris), es la causa de la asimetría facial durante el llanto. Los pacientes con esta anomalía, presentan caída de la comisura labial del lado intacto a diferencia de la parálisis facial de origen traumático o congénito, y su diagnóstico se obtiene fácilmente, mediante el examen físico. Esta anomalía está asociada con otras de origen congénito, sobre todo los defectos cardíacos, por lo que se nombra síndrome cardiofacial de Cayler. Se presenta una niña, recién nacida de 56 horas, atendida en el servicio de Neonatología del Hospital Pediátrico "Octavio de la Concepción de la Pedraja" de Holguín, Cuba. La paciente egresa con diagnóstico de síndrome de Cayler, al presentar asimetría facial durante el llanto, y tetralogía de Fallot. Se realizan estudios neurofisiológicos como: electroneurografía del nervio facial y electromiograma de los músculos depresores del labio.

Asymmetrical Crying Facies Syndrome, is caused by congenital hypolapsia or anguli oris agenesis. Patients usually present drop of the intact corner of their mouths while crying. Though it is easily diagnosed, it must be differentiated from traumatic or congenital facial palsies. This condition has been associated with several other anomalies, especially with heart issues. That´s why it is called Cayler Cardiofacial Syndrome. We have reported a case of a 56 hours newborn girl. She shows facial asymmetry as a valuable sign for suspecting other congenital anomalies. Clinical evaluation, including facial nerve electroneurography and electromyography, were performed.

Assessment of a Brief Intervention with Parents to Prevent Shaken Baby Syndrome / Avaliação de Intervenção Breve com Pais para Prevenção da Síndrome do Bebê Sacudido / Evaluación de Intervención Breve con Padres para Prevención del Síndrome del Bebé Sacudido

Abstract Shaken Baby Syndrome (SBS) is a form of physical abuse, which may result in serious consequences for child development. Despite its severity, SBS can be prevented through parent training. This study aimed to evaluate the effectiveness of a brief intervention to increase knowledge on SBS with 252 parents. Participants were randomly assigned into two conditions: Experimental Group (EG) and a waiting Control Group (CG). The intervention consisted of exhibition of an educational video and reading a pamphlet on SBS. To measure parental knowledge, the Attitudes towards the Infant Crying Scale was used. An increase in participants' knowledge about consequences of shaking, caregiver's wellbeing, caregiver strategies to deal with crying, beliefs about infant care and about characteristics of infant crying were observed in the EG at posttest, indicating the effectiveness of the intervention.

Resumo A Síndrome do Bebê Sacudido (SBS) é uma forma de maus-tratos físicos que pode acarretar consequências gravíssimas para o desenvolvimento infantil, podendo ser prevenida pela educação parental. Este estudo teve por objetivo avaliar a eficácia de uma intervenção breve para o aumento de conhecimento sobre a SBS em 252 pais. Os participantes foram divididos aleatoriamente em dois grupos: Experimental e Controle de Espera. A intervenção consistiu na exibição de vídeo educativo e leitura de panfleto sobre a SBS. Para avaliação do conhecimento foi utilizada a Escala de Atitudes Frente ao Choro do Bebê. Após a intervenção foi observado no grupo experimental o aumento do conhecimento sobre as consequências de se sacudir o bebê, estratégias para o bem-estar do cuidador, estratégias para lidar com o choro relacionadas ao cuidador, crenças sobre o cuidado do bebê e características do choro do bebê nos primeiros meses de vida, indicando a efetividade da intervenção.

Resumen El Síndrome del Bebé Sacudido (SBS) es una forma de abuso físico que puede resultar en consecuencias muy graves para el desarrollo infantil y puede ser prevenido mediante la educación de los padres. Este estudio evaluó la eficacia de una intervención breve para aumentar el conocimiento sobre el SBS con 252 padres. Los participantes fueron divididos aleatoriamente en dos grupos: Grupo experimental y Grupo de control de espera. La intervención consistió en la proyección de un vídeo educativo y de la lectura de un folleto sobre el SBS. Para evaluar el conocimiento se utilizó la Escala de Actitudes Frente al Llanto del Bebé. Después de la intervención, se observó que el grupo experimental obtuvo mayor conocimiento sobre las consecuencias de sacudir a los bebes, estrategias para el bienestar del cuidador, estrategias del cuidador para lidiar con el llanto del bebé, las creencias sobre el cuidado del bebé y las características del llanto del bebé en los primeros meses de vida, indicando la efectividad de la intervención.

Clinical characteristics and genetic analysis of neonatal asymmetric crying facies / 中国新生儿科杂志

Objective To study the clinical and genetic characteristics of neonatal asymmetric crying facies (ACF).Method From January 2007 to December 2016,clinical data were retrospectivelyreviewed in patients with ACF admitted to Neonatal Unit of Xinhua Hospital.The data included maternalpregnancy and delivery history,clinical manifestation,results of chromosome florescent in situ hybridization(FISH) and gene chip test.Result Among 32 patients with classic ACF,8 were female and 24 weremale.5 cases were one of the twins or muhiplets.16 patients were born from mothers with history ofspontaneous abortion or induced abortion.16 patients presented with ACF on the left side and 16 patients onthe right side.7 patients had single ACF malformation,7 with malformation of another organ,and18 patients with malformation of more than two other organs.Combined malformation included earmalformation in 11 cases,facial malformation in 6 cases,cardiovascular malformation in 19 cases,urinarytract malformation in 3 cases,digestive system malformation in 3 cases,abnormal nervous system image in7 cases,and immune/endocrine system abnormality in 3 cases.10 patients received genetic analysis withFISH and/or gene chip tests and 4 patients had positive results.Among the patients who completed geneticanalysis,8 patients received FISH test and 2 patients had 22q11.2 distal deletion.1 patient hadheterozygous deletion in 22q21 region using gene chip test after he got a negative result with FISH.Amongthe 32 cases,6 patients died until now,among them,5 patients had multi-organ malformation.ConclusionACF is a tiny facial deformity,however it is often associated with other congenital malformations.Earlygenetic detection and systematic multiple malformations screening are particularly important for diagnosis andprediction of prognosis.

The lesion distribution pattern of poststroke pathological laughing and crying:an MRI study / 中华行为医学与脑科学杂志

Objective To explore the lesion distribution pattern of poststroke pathological laughing or crying (PSPLC) by using MRI,and to discuss its pathogenesis. Methods All 24 cases selected from stroke patients who were treated in the department of neurology from May 2012 to December 2015 and had complete follow?up information after their discharge through 6 months were screened for pathological laughing and crying( PLC) . MRI characteristics of cases were analyzed retrospectively to identify lesion distribution. 28 cases with acute isolated pontine infarction who did not demonstrate PLC were enrolled as a comparison group. Lesion pattern difference between 14 cases of the pontine infarction with PLC and 28 cases of acute i?solated pontine infarction without PLC was compared. Results In these 24 cases,the highest occurrence rate of PLC was 41.67% in the second month. 15 cases had forced crying (62.5%),3 cases had forced laughing (12.5%),6 cases had pathological laughing or crying (25%). MRI studies showed lesion location in the left side were 3 cases,in the right side were 1 case,in the bilateral brain were 20 cases,in the basal ganglia were 9 cases,in the thalamus were 8 cases,in the corona radiata were 6 cases,in the semioval center were 5 cases, in the cerebral cortex were 4 cases,in the cerebellum were 4 cases and in the brain stem were 14 cases. In the cases of PLC with brain stem lesion,only 1 case was isolated pontine infarction,the other patients all combined with lesions of other parts. Compared with the pontine infarction with PLC,the isolated pontine in?farction without PLC was more likely to suffer with unilateral brain lesions(71%vs 50%),but there was no significant difference (χ2=0.844, P=0.358) . Conclusion PSFLC is not a rare complication after stroke, which is associated with multiple site of brain involvement especially pontine infarction.

Algorithms for managing common gastrointestinal symptoms in infants / 中华实用儿科临床杂志

Gastrointestinal symptoms such as regurgitation,infantile colic,constipation,dyschezia,and diarrhoea are frequent in infants.These symptoms always were functional gastrointestinal symptoms.It is easily confused with other diseases.The knowledge and management of these symptoms are no uniform or clear.Since double-blind placebo controlled prospective intervention trials are very limited,the algorithms are still based on consensus using the evidence.This article respectively introduces each gastrointestinal symptoms,include clinical manifestations,diagnosis and management referring to Rome Ⅳ and domestic and overseas paediatricians consensus,to improve clinical pediatricians the knowledge of gastrointestinal symptoms and specify the management.

Llanto excesivo en los primeros meses de vida: Revisión / Excessive crying in infants in the first months of life: Review

El comportamiento caracterizado por llanto e irritabilidad, una de las presentaciones clínicas más frecuentes en los primeros meses de vida, surge de una compleja interacción de factores culturales, psicosociales, ambientales y biológicos. El 5% de los casos presenta alguna afección orgánica. Un enfoque holístico de tipo biopsicosocial del problema parece ser el más conveniente. El presente manuscrito examina la evidencia reciente con el propósito de actualizar el manejo clínico de esta afección e intentar redefinirla.

Cry-fuss problems are among the most common clinical presentations in the first few months of life. Cry-fuss behaviour emerges out of a complex interplay of cultural, psychosocial, environmental and biologic factors, with organic disturbance implicated in only 5% of cases. A holistic biopsychosocial approach can be most effective. This article reviews recent evidence to update clinical management, and will attempt to reconceptualize infant colic definition.

Signs and symptoms associated with digestive tract development / Sinais e sintomas associados com o desenvolvimento do trato digestivo

ABSTRACT Objective: To analyze the development and prevalence of gastrointestinal signs and symptoms associated with the development of the digestive tract, and to assess the measures aimed to reduce their negative impacts. Source of data: Considering the scope and comprehensiveness of the subject, a systematic review of the literature was not carried out. The Medline database was used to identify references that would allow the analysis of the study topics. Synthesis of results: Infants frequently show several gastrointestinal signs and symptoms. These clinical manifestations can be part of gastrointestinal functional disorders such as infantile colic, infant regurgitation, and functional constipation. Allergy to cow's milk protein and gastroesophageal reflux disease are also causes of these clinical manifestations and represent an important and difficult differential diagnosis. The diseases that course with gastrointestinal signs and symptoms can have an impact on family dynamics and maternal emotional status, and may be associated with future problems in the child's life. Comprehensive pediatric care is essential for diagnosis and treatment. Maternal breastfeeding should always be maintained. Some special formulas can contribute to the control of clinical manifestations depending on the established diagnosis. Conclusion: During the normal development of the digestive tract, several gastrointestinal signs and symptoms may occur, usually resulting from functional gastrointestinal disorders, gastroesophageal reflux disease, and allergy to cow's milk protein. Breastfeeding should always be maintained.

RESUMO Objetivo: Analisar o desenvolvimento e a prevalência de sinais e sintomas gastrintestinais associados com o desenvolvimento do tubo digestivo e as medidas que visam a diminuir suas repercussões negativas. Fontes dos dados: Considerando a abrangência e amplitude do tema, não foi feita revisão sistemática da literatura. Usou-se a base de dados do Medline para a identificação de referências bibliográficas que permitissem contemplar os temas de estudo. Síntese dos resultados: O lactente apresenta com elevada frequência sinais e sintomas gastrintestinais. Essas manifestações clínicas podem fazer parte de distúrbios funcionais gastrintestinais, como cólica, regurgitação e constipação intestinal funcional. A alergia à proteína do leite de vaca e a doença do refluxo gastroesofágico também são causas dessas manifestações clínicas e representam um importante e difícil diagnóstico diferencial. As doenças que cursam com sintomas e sinais gastrintestinais podem ter consequências na dinâmica familiar e no estado emocional das mães. Podem se associar com problemas na vida futura da criança. A atenção pediátrica completa é fundamental para o diagnóstico e o tratamento. O aleitamento natural deve sempre ser mantido. Algumas fórmulas especiais podem contribuir para o controle das manifestações clínicas na dependência do diagnóstico estabelecido. Conclusão: Durante o desenvolvimento normal do tubo digestivo podem ocorrer sinais e sintomas gastrintestinais em geral decorrentes dos distúrbios gastrintestinais funcionais, da doença do refluxo gastroesofágico e da alergia à proteína do leite de vaca. Aleitamento natural deve sempre ser mantido.

Excessive crying in infants / Excessive crying in infants

ABSTRACT Objective: Review the literature on excessive crying in young infants, also known as infantile colic, and its effects on family dynamics, its pathophysiology, and new treatment interventions. Data source: The literature review was carried out in the Medline, PsycINFO, LILACS, SciELO, and Cochrane Library databases, using the terms “excessive crying,” and “infantile colic,” as well technical books and technical reports on child development, selecting the most relevant articles on the subject, with emphasis on recent literature published in the last five years. Summary of the findings: Excessive crying is a common symptom in the first 3 months of life and leads to approximately 20% of pediatric consultations. Different prevalence rates of excessive crying have been reported, ranging from 14% to approximately 30% in infants up to 3 months of age. There is evidence linking excessive crying early in life with adaptive problems in the preschool period, as well as with early weaning, maternal anxiety and depression, attention deficit hyperactivity disorder, and other behavioral problems. Several pathophysiological mechanisms can explain these symptoms, such as circadian rhythm alterations, central nervous system immaturity, and alterations in the intestinal microbiota. Several treatment alternatives have been described, including behavioral measures, manipulation techniques, use of medication, and acupuncture, with controversial results and effectiveness. Conclusion: Excessive crying in the early months is a prevalent symptom; the pediatrician's attention is necessary to understand and adequately manage the problem and offer support to exhausted parents. The prescription of drugs of questionable action and with potential side effects is not a recommended treatment, except in extreme situations. The effectiveness of dietary treatments and use of probiotics still require confirmation. There is incomplete evidence regarding alternative treatments such as manipulation techniques, acupuncture, and use of the herbal supplements and behavioral interventions.

RESUMO Objetivo: Revisar a literatura sobre choro excessivo em bebês pequenos e cólicas infantis e suas repercussões na família e a fisiopatologia e as estratégias de tratamento. Fonte dos dados: Revisadas as principais bases de dados, Medline, PsycINFO, Lilacs e SciELO e Cochrane Library com o uso das expressões “choro excessivo do lactente” e “cólicas do lactente”. Foram selecionadas as publicações mais relevantes com ênfase nos últimos cinco anos. Síntese dos dados: É um sintoma comum nos primeiros meses de vida e é motivo de cerca de 20% das consultas pediátricas. As prevalências de choro excessivo variam de 14 a 30% nesses lactentes. Existem evidências que ligam o choro excessivo nos primeiros meses de vida com problemas futuros, bem como ao desmame precoce, à ansiedade, à depressão materna, ao transtorno do déficit de atenção/hiperatividade (TDAH) e a outros problemas comportamentais. Distintos mecanismos fisiopatológicos podem explicar esse quadro clínico, como alterações no ritmo circadiano, imaturidade do SNC e alterações na microbiota intestinal. São descritas opções de tratamento, desde medidas comportamentais, técnicas manipulativas e uso de medicação até acupuntura, com resultados e eficácia controversos. Conclusão: Para o choro excessivo nos primeiros meses é necessária a atenção do pediatra para o entendimento e manejo do problema e oferecer apoio para pais em exaustão. A prescrição de drogas de efeitos duvidosos e potenciais efeitos colaterais não é terapêutica preconizada, a não ser em situações extremas. A eficácia dos tratamentos dietéticos e o uso de probióticos ainda necessitam de confirmação. Existem evidências incompletas a respeito de tratamentos opcionais, como técnicas manipulativas, acupuntura, uso de suplemento à base de ervas e intervenções comportamentais.