Lingual Frenulum Phenotypes in Brazilian Infants With Congenital Zika Syndrome.

OBJECTIVE: The present study aimed to evaluate lingual frenulum in children affected by congenital Zika syndrome (CZS) and to analyze the association of lingual frenulum phenotypes with other variables. DESIGN: This present work had a cross-sectional, descriptive study design. SETTING: This study was carried out in Fortaleza (Brazil). The health professionals provided tertiary level of care. Data collection occurred during a multidisciplinary task force for evaluating infants affected by CZS in December 2 to 3, 2016. PATIENTS: Fifty-four patients with CZS (1-12 months old; 32 girls and 22 boys) were recruited from a population of 70 infants. INTERVENTIONS: A multidisciplinary group comprised of speech-language pathologist/audiologists and pediatric dentists evaluated all patients through an intraoral examination and a specific tongue maneuver protocol for infants. MAIN OUTCOME MEASURES: Lingual frenulum visibility was the primary outcome measure. Before initiating the study, we hypothesized that children with CZS had an absent lingual frenulum. RESULTS: Lingual frenula were visible in 34 (63%) infants, whereas in 20 (37%) infants lingual frenula visibility required a specific maneuver to retract the tongue. Six of 20 infants presented posteriorly positioned lingual frenula that were visible after maneuver. Lingual frenula were covered by mucous tissue in 14 infants. Presence of posterior frenulum was associated with dysphagia ( P = 0.038). However, the presence of dysphagia in a multivariate model did not associate with the presence of a posterior lingual frenulum ( P = .069) or neurologic symptoms ( P = .056). CONCLUSION: Children with CZS showed predominance of a posterior lingual frenula covered by an overlapping curtain-like mucous membrane.

Preschoolers' Performance on the Brazilian Adaptation of the Preschool Language Assessment Instrument - Second Edition.

PURPOSE: The objective was to test whether the Brazilian version of the Preschool Language Assessment Instrument - Second Edition (PLAI-2) has the potential to assess and identify differences in typical language development of Portuguese-speaking preschoolers. METHODS: The study included 354 children of both genders with typical language development who were between the ages of 3 years and 5 years 11 months. The version of the PLAI-2 previously translated into Brazilian Portuguese was used to assess the communication skills of these preschool-age children. RESULTS: Statistically significant differences were found between the age groups, and the raw score tended to increase as a function of age. With nonstandardized assessments, the performances of the younger groups revealed behavioral profiles (e.g., nonresponsive, impulsive behavior) that directly influenced the evaluation. CONCLUSIONS: The findings of this study show that the PLAI-2 is effective in identifying differences in language development among Brazilian children of preschool age. Future research should include studies validating and standardizing these findings.

Sleep disturbance in children with attention-deficit hyperactivity disorder: relationship with melatonin and behavior.

AIM: To evaluate the prevalence and types of sleep problems and their correlations with melatonin content and behavior in Attention-Deficit Hyperactivity Disorder (ADHD) children. METHOD: Sleep in ADHD children and typically developing children (TD) aged 6-14 was assessed by the Sleep Disorders Scale for Children (SDSC) and actigraphy, salivary melatonin quantified by ELISA, and behavior was analyzed using the Strengths and Difficulties Questionnaire. RESULTS: ADHD children showed a higher frequency of sleep disturbances, higher sleep latency, and lower sleep efficiency than in the TD group. The ADHD group presented lower melatonin nocturnal content compared to the TD group. Disorders of Initiating and Maintaining Sleep (DIMS) was moderately associated with nocturnal melatonin. The total behavior difficulties were correlated with Disorders of Initiating and Maintaining Sleep (DIMS), Sleep/Wake Transition Disorders (SWTD), Disorders of Excessive Somnolence (DES), Sleep Hyperhidrosis (SHY) and Total SDSC Score. The behavior was the only determinant of the total SDSC score (R2 = 0.499; p < 0.002). CONCLUSION: This study provides, for the first time, evidence that among the frequent sleep disturbances in ADHD, the disorders in initiating and maintaining sleep are associated with the low levels of melatonin found in this population. Additionally, these, along with other sleep disturbances, are linked to behavioral problems in ADHD.

Language skills development in children with congenital Zika virus syndrome.

BACKGROUND: The characterization of the phenotype of children with congenital Zika virus syndrome (CZS) is an ongoing process, since many characteristics can only be described with the advancing age of children providing some insights into the long-term sequelae. AIMS: To describe emerging findings on the impact of CZS on language development in children with CZS in a 4-year longitudinal study. METHODS AND PROCEDURES: 39 boys and 44 girls with CZS were allocated into four groups according to age ranging from 12 to 36 months. Language development was assessed using the Early Language Milestone Scale. OUTCOMES AND RESULTS: Visual, expressive, and receptive auditory skills of patients were lower than expected for their age. Despite producing vowel sounds, they did not babble; despite present hearing, the majority of the children did not understand simple commands. In over 4 years of follow-up, there was no evolution in language parameters, with the children stagnating at the language skills corresponding to 3 months of age. CONCLUSIONS AND IMPLICATIONS: Most children with CZS are not able to produce vocalic sounds, but some may be able to communicate basic needs through alternative communication. WHAT THIS PAPER ADDS?: Some babies with CZS died prenatally, at birth, or in the first year of life due to associated complications such as respiratory infection, dysphagia, and epilepsy. However, the functionality of the future remains uncertain for surviving babies. This study adds information about the impact of Zika Virus on the central nervous system and, consequently, the severity and complexity of the CZS language. Over 4 years of follow-up, no evolution in language parameters was observed in children with CSZ. Children with CZS demonstrate severe neurodevelopmental impairment, stagnating in language skills at the age of 3 months. In the future, some of them may be able to communicate their basic needs through alternative communication.

Performance of preschool and schoolchildren on the ProNOH protocol: macrostructure aspects. / Desempenho de pré-escolares e escolares no protocolo ProNOH: aspectos macroestruturais.

PURPOSE: To investigate if the narrative score of the ProNOH protocol allows for discriminating age groups, as well as its relation with the global coherence level of the story. The performance of preschool and schoolchildren on the macrostructure aspects. METHODS: Participants were 97 preschoolers and schoolchildren with typical language development, aged between five and 12 years old, and both sexes who attended public schools. The "Protocolo de Avaliação da Narrativa Oral de História (ProNOH)" (Protocol for the Evaluation of Oral Storytelling) was applied and the narrative score in the macrostructure dimension was calculated with story grammar elements. These same story elements were used to obtain the global coherence level of the story, as proposed by Spinillo and Martins (1997). RESULTS: A statistically significant difference was found between age groups, mainly between the borderline ages of 5-6 years, 7-8 years, 9-10 years, and 11-12 years. Positive and statistically significant correlations were found between the narrative score and global coherence and age, as well as between the narrative score and global coherence. CONCLUSION: The protocol proved to be useful for identifying the repertoire of typical story grammar elements as an objective measure that differs in oral narrative across age groups. The results also indicate that the narrative score can provide an idea about the global coherence of the story, although this value does not replace a specific analysis.

OBJETIVO: Investigar se o escore narrativo obtido por meio da aplicação do "Protocolo de Avaliação da Narrativa Oral de História (ProNOH)" permite discriminar os grupos etários estudados, bem como sua relação com o nível de coerência global da história. MÉTODO: Participaram 97 pré-escolares e escolares com desenvolvimento típico de linguagem, entre 5 e 12 anos, ambos os sexos que frequentavam escola pública. Foi aplicado o "Protocolo de Avaliação da Narrativa Oral de História (ProNOH)" e calculado o escore narrativo na dimensão macroestrutural a partir dos elementos estruturais previstos no modelo da gramática de história. Esses mesmos elementos foram usados para obter o nível de coerência global da história, segundo proposta de análise de Spinillo e Martins (1997). RESULTADOS: Diferença estatisticamente significante foi encontrada entre os grupos etários, principalmente entre as idades fronteiriças de 5-6 anos, 7-8 anos, 9-10 anos e 11-12 anos. Correlação positiva e estatisticamente significante foi encontrada com a idade e o escore narrativo e a coerência global, bem como entre o escore narrativo e o nível de coerência global. CONCLUSÃO: O protocolo mostrou ser útil para identificar o repertório de elementos típicos da gramática de história na narrativa oral como uma medida objetiva que diferenciou a narrativa oral de grupos etários. Os resultados indicaram ainda que o escore narrativo pode ser uma medida norteadora do nível de coerência da história, apesar deste valor não substituir uma análise específica.

Voice and Behavior of Children and Adolescents With Obesity: Integrative Literature Review.

PURPOSE: To verify the relationship between obesity, voice and behavior in childhood and adolescence, through an integrative review. METHODS: The researchers surveyed the articles indexed in the following databases: Embase, Web of Science, Scopus and Pubmed, using uniterms found in the Medical Subject Headings, related to the subject of the study, combined by the Boolean operators "and" and "or", published until January 2022. In the end, three reviewers selected the articles. RESULTS: When searching for studies related to voice, obesity and behavior, 62 articles were found that did not meet the selection criteria and were excluded. Given this result, new searches were carried out with the following crossing strategies: voice and obesity and; Voice, Behavior and Child Behavior Checklist (CBCL). After the evaluation of the reviewers, two articles were selected referring to the intersection "voice and obesity in children and adolescents" and, two studies, in the intersection between "voice, behavior and CBCL". The first study on voice and obesity showed that the greater the abdominal circumference, the greater the maximum expiratory force and sound pressure found. The second study showed high Jitter and NHR values in obese children. Furthermore, studies on voice, behavior and CBCL showed that children with vocal nodules were more sociable than children without vocal nodules, and had behaviors described as "screams a lot" and "teases a lot". On the other hand, another study found potential risks of behavioral changes in the face of vocal complaints. CONCLUSIONS: Although this literature review did not find studies associating obesity with vocal and behavioral disorders, the literature found showed the presence of vocal alteration in some acoustic parameters in obese children. Regarding voice, behavior and CBCL, two studies were found reporting the presence of behavioral changes in children with voice-related complaints, according to the opinion of their parents.

Sleep disturbances and behavior in Smith-Magenis syndrome.

BACKGROUND: The Smith-Magenis syndrome (SMS) shows a collection of neurodevelopmental problems including mild to moderate intellectual disability, change-related anxiety, impulsivity, speech delay, Attention-Deficit/Hyperactivity Disorder (ADH) and sleep disturbances. Sleep disorders, when present, have been treated in several populations with consecutive improvements in cognitive and behavioral aspects. AIMS: To better understand the existing relationships between sleep disturbances and behavioral problems in SMS syndrome this study describes the sleep and behavior problems in the SMS and explores the possible relation between both. METHODS AND PROCEDURES: 17 individuals with SMS (50% males; 11.2 ± 4.9 years old) and 12 individuals with typical development (50% male; 11.1 ± 4.4 years old) were investigated using the Sleep Disturbance Scale for Children and the Child Behavior Checklist. RESULTS: A high percentage (60%) of individuals with SMS have an indication of sleep disorders, being the most frequent disorders the sleep-wake transition disorders, and disorders of initiating and maintaining sleep with sleep latency higher than acceptable and total sleep time below acceptable. More than 94% of the SMS group presented clinical or borderline scores on the total behavioral problems scale. The most common behavioral problems were Externalizing Problems, Thought and Attention, ADH and Aggressive problems. There was a positive correlation between disorders of initiating and maintaining sleep, sleep-wake transition disorders, disorders of arousal, disorders of excessive somnolence and behavioral problems. CONCLUSIONS AND IMPLICATIONS: The worse the sleep disturbances investigated, the more severe the behavioral problems characteristics reinforcing the importance to address the sleep problems in the treatment of SMS individuals.

Cultural adaptation of the Expressive One-Word Picture Vocabulary Test, 4th edition (EOWPVT-4), for Brazilian Portuguese speakers. / Adaptação cultural do Expressive One-Word Picture Vocabulary Test, 4th edition (EOWPVT-4), para falantes do Português Brasileiro.

PURPOSE: To present a brief report of the first steps that involved the process of the cultural translation and adaptation of the Expressive One-Word Picture Vocabulary Test, fourth edition to Brazilian Portuguese (BP). METHODS: The process of translation and adaptation of this instrument was performed in the following steps: (1) translation of the original text (English) to Brazilian Portuguese (target culture) by two different sworn translators oriented towards our research goal; (2) parity analysis between both translations and design, by a group of experts, of a synthesis version; (3) back translation of the synthesis version by two other sworn translators who did not participate in step 1; and (4) Comparison between back-translation and the original version made by a group of specialists, thus shaping the pre-final adapted version of the EOWPVT-4. RESULTS: In the Brazilian version, the number of items from the original version was maintained and the cultural adaptation of the EOWPVT-4 to BP followed the steps recommended in the literature besides considering the differences in the socio-cultural context, showing no significant discrepancies regarding semantic equivalence. Relevant adaptations (e.g., items not representative within the Brazilian culture) were required during this process so that the instrument could be used with the same methodological rigor as the original instrument. CONCLUSION: The process of cultural adaptation of this instrument indicated that there was theoretical, semantic, idiomatic and cultural equivalence with the original version in English.

OBJETIVO: Apresentar um breve relato sobre as primeiras etapas que envolveram o processo de tradução e adaptação cultural do teste Expressive One-Word Picture Vocabulary Test, fourth edition para o Português Brasileiro (PB). MÉTODO: O processo de tradução e adaptação desse instrumento foi realizado nas seguintes etapas: (1) tradução do texto original (inglês) para o PB (cultura alvo) por dois tradutores juramentados distintos e orientados quanto ao objetivo da pesquisa; (2) análise de paridade entre as traduções realizadas e concepção, por um grupo de especialistas, de uma versão síntese; (3) retrotradução da versão síntese por outros dois tradutores juramentados que não participaram da etapa 1; e (4) comparação entre a retrotradução e a versão original feita por um grupo de especialistas, moldando, assim, a versão adaptada pré-final do EOWPVT-4. RESULTADOS: Na versão brasileira, foi mantida a quantidade de itens da versão original e a adaptação cultural do EOWPVT-4 para o PB seguiu as etapas recomendadas pela literatura, além de considerar as diferenças do contexto sociocultural, não apresentando discrepâncias significativas no que se refere à equivalência semântica. Foram necessárias adaptações consideradas relevantes (e.g., itens não representativos da cultura brasileira) durante esse processo para que o instrumento pudesse ser utilizado com o mesmo rigor metodológico do instrumento original. CONCLUSÃO: O processo de adaptação cultural desse instrumento indicou que houve equivalência teórica, semântica, idiomática e cultural com a versão original em inglês.

Sleep quality, functional skills, and communication in preschool-aged children with autism spectrum disorder.

AIM: This study aimed to correlate sleep quality, the performance of functional skills (mobility, self-care, and social function), communication, independence, and severity of ASD in children with ASD. METHOD: 58 children between 3 and 5 years and 11 months old were investigated. The Childhood Autism Rating Scale was applied to determine the severity of autism; the Sleep Disturbance Scale for Children was used to investigate sleep quality, and the Pediatric Evaluation of Disability Inventory to investigate functional abilities and independence of the children. RESULTS: 68.9 % of the children showed indicative of sleep disorders. There was no correlation between the different sleep disorders and communication. Sleep disorders showed a negative correlation with functional performance and a positive correlation with ASD severity. INTERPRETATION: The current study offers an exploration between sleep and functional skills in children with ASD. These findings provide important clinical implications in the diagnosis and intervention process of children with ASD and also stimulate reflections on the importance in minimize the impact of sleep disorders and functional abilities on the quality of life of these individuals and their families.

Sentence contexts and cloze probabilities for Brazilian Portuguese children and adolescents.

PURPOSE: In this study we investigated a set of 100 sentence contexts and their cloze probabilities to develop a database of linguistic stimuli for Brazilian Portuguese children and adolescents. The study also examined age-related changes on cloze probabilities, and specified the predictor effects of age and cloze probabilities on idiosyncratic responses and errors (semantic, syntactic, and other errors). Finally, the study also aimed to shed light on cultural effects on word generation by comparing Brazilian and Portuguese sentence databases. METHOD: 361 typically developing monolingual Brazilian speakers, with ages ranging from 7 to 18 years, participated in the study. The cloze task was composed by 100 sentence contexts, grounded on the European Portuguese database. Responses were classified as valid (correct) or invalid (semantic, syntactic, and other-type errors). Statistical analyses were based on mixed-effects logistic models. RESULTS: Sixty-three sentences met criteria for high cloze probabilities, 30 for medium cloze, and 7 for low cloze. Age was a significant predictor of idiosyncratic responses, semantic and syntactic errors: older participants were less likely to produce idiosyncratic responses, as well as semantic and syntactic errors. Cloze probability values were concordant in the Brazilian and Portuguese databases for 31 out of 49 (83.7%) high-cloze sentences and for 7 low-cloze sentences. CONCLUSION: In this study we have provided a database with cloze probability values for a set of 100 sentence-final word contexts for Brazilian Portuguese children and adolescents. Results showed that both age and sentence contextual level predicted sentence final word completion. Older participants were more likely to choose more consistently the same final word, with the contextual level of a given sentence also contributing to the final word selection. Age should be controlled for in future studies probing semantic processing with this set of sentences.

Desempenho de pré-escolares e escolares no protocolo ProNOH: aspectos macroestruturais / Performance of preschool and schoolchildren on the ProNOH protocol: macrostructure aspects

RESUMO Objetivo Investigar se o escore narrativo obtido por meio da aplicação do "Protocolo de Avaliação da Narrativa Oral de História (ProNOH)" permite discriminar os grupos etários estudados, bem como sua relação com o nível de coerência global da história. Método Participaram 97 pré-escolares e escolares com desenvolvimento típico de linguagem, entre 5 e 12 anos, ambos os sexos que frequentavam escola pública. Foi aplicado o "Protocolo de Avaliação da Narrativa Oral de História (ProNOH)" e calculado o escore narrativo na dimensão macroestrutural a partir dos elementos estruturais previstos no modelo da gramática de história. Esses mesmos elementos foram usados para obter o nível de coerência global da história, segundo proposta de análise de Spinillo e Martins (1997). Resultados Diferença estatisticamente significante foi encontrada entre os grupos etários, principalmente entre as idades fronteiriças de 5-6 anos, 7-8 anos, 9-10 anos e 11-12 anos. Correlação positiva e estatisticamente significante foi encontrada com a idade e o escore narrativo e a coerência global, bem como entre o escore narrativo e o nível de coerência global. Conclusão O protocolo mostrou ser útil para identificar o repertório de elementos típicos da gramática de história na narrativa oral como uma medida objetiva que diferenciou a narrativa oral de grupos etários. Os resultados indicaram ainda que o escore narrativo pode ser uma medida norteadora do nível de coerência da história, apesar deste valor não substituir uma análise específica.

ABSTRACT Purpose To investigate if the narrative score of the ProNOH protocol allows for discriminating age groups, as well as its relation with the global coherence level of the story. The performance of preschool and schoolchildren on the macrostructure aspects. Methods Participants were 97 preschoolers and schoolchildren with typical language development, aged between five and 12 years old, and both sexes who attended public schools. The "Protocolo de Avaliação da Narrativa Oral de História (ProNOH)" (Protocol for the Evaluation of Oral Storytelling) was applied and the narrative score in the macrostructure dimension was calculated with story grammar elements. These same story elements were used to obtain the global coherence level of the story, as proposed by Spinillo and Martins (1997). Results A statistically significant difference was found between age groups, mainly between the borderline ages of 5-6 years, 7-8 years, 9-10 years, and 11-12 years. Positive and statistically significant correlations were found between the narrative score and global coherence and age, as well as between the narrative score and global coherence. Conclusion The protocol proved to be useful for identifying the repertoire of typical story grammar elements as an objective measure that differs in oral narrative across age groups. The results also indicate that the narrative score can provide an idea about the global coherence of the story, although this value does not replace a specific analysis.

Oral narrative structure and coherence of children with attention deficit hyperactivity disorder. / Estrutura e coerência da narrativa oral de crianças com transtorno de déficit de atenção e hiperatividade.

PURPOSE: This study aimed to characterize and compare the use of typical story grammar elements and global coherence level in the oral narrative of children with Attention Deficit Hyperactivity Disorder with the narrative of children without the disorder and with typical development. METHODS: A total of 40 children of both sexes aged 5 to 10 years who attended elementary school participated in the study, 20 of whom were diagnosed with Attention Deficit Hyperactivity Disorder (ADHD Group), and 20 with typical development (TD Group). Participants from each group were similar in sex, chronological age, schooling and socioeconomic status. The wordless picture book Frog, Where Are You? was used to elicit the oral narrative analyzed for the presence of the main typical elements of the story schema (character, theme/topic, event/plot and outcome), and afterwards their narration was classified according to four different levels of organization corresponding to the global story coherence level. RESULTS: The ADHD Group presented lower scores on the structural elements "theme/ topic" and "outcome" and a narrative with lower degree of coherence compared to the TD Group. CONCLUSION: The children with ADHD included in this study presented difficulties to use typical story grammar elements, mainly related to the maintenance of the central theme and outcome of the story. These elements are considered fundamental for construction of narrative coherence, which justifies the lower levels of global coherence found in the oral narrative of the ADHD Group.

OBJETIVO: O objetivo do estudo foi caracterizar e comparar o uso de elementos típicos da gramática de história e o nível de coerência global na narrativa oral de crianças com Transtorno de Déficit de Atenção e Hiperatividade à narrativa de crianças sem o transtorno e com desenvolvimento típico. MÉTODO: Participaram 40 crianças com idade entre 5 e 10 anos, de ambos os sexos, que frequentavam o ensino fundamental, sendo 20 com diagnóstico de Transtorno de Déficit de Atenção e Hiperatividade (Grupo TDAH) e 20 com desenvolvimento típico (Grupo TD). Os participantes de cada grupo eram semelhantes quanto ao sexo, idade cronológica, escolaridade e nível socioeconômico. O livro "Frog Where Are You?" foi utilizado para eliciar a narrativa oral de história, que foi analisada quanto à presença dos principais elementos típicos do esquema de história (personagem, tema/tópico, evento/trama e desfecho) e posteriormente classificada dentre quatro diferentes níveis crescentes de organização, correspondendo ao nível de coerência global da história. RESULTADOS: O grupo TDAH apresentou menor pontuação nos elementos estruturais "tema/tópico" e "desfecho" e narrativa com grau de coerência inferior quando comparado ao grupo TD. CONCLUSÃO: As crianças com TDAH deste estudo apresentaram dificuldades no uso de elementos típicos da gramática de história, principalmente relacionados com a manutenção do tema central e desfecho da história. Tais elementos são considerados fundamentais para a construção do sentido da narrativa, o que justifica os níveis inferiores de coerência encontrados na narrativa oral do grupo TDAH.

Performance of Brazilian Portuguese speakers in the Test of Narrative Language (TNL). / Desempenho de falantes do português brasileiro no "Test of Narrative Language (TNL)".

PURPOSE: Investigate the performance of native speakers of Brazilian Portuguese in the Test of Narrative Language (TNL) and correlate their performance in its oral narration and narrative comprehension subtests. METHODS: Participants were 140 individuals with typical language development aged 5-0 to 11-11 (years-months) divided according to age group. RESULTS: Statistically significant difference was observed between the 5-year-old group and all the other age groups and between the 6-year-old group and the 8-, 9-, 10- and 11-year-old groups. The 7-year-old group did not differ from the 6- and 8-year-old groups. Half of the 7-year-old group was in 1st grade of Elementary School and the other half was in 2nd grade, which may have contributed to the non-differentiation between this group and its border-age groups. No statistically significant difference was found between groups as of the age of eight years. Positive correlation was observed between performance in the narrative comprehension and oral narration subtests of the TNL in the 5-, 6- and 7-year-old groups, which did not occur for the other age groups. CONCLUSION: The Test of Narrative Language differentiated the 5-, 6- and 7-year-old groups, but did not differentiate groups as of the age of eight years. No correlation was found between performance in the oral narration and narrative comprehension subtests of the TNL as of the age of eight years, which suggests mastery of both skills from this age.

OBJETIVO: Investigar o desempenho de falantes do português brasileiro no Test of Narrative Language e correlacionar o desempenho nas tarefas de produção e compreensão da narrativa. MÉTODO: Participaram 140 indivíduos, entre 5 e 11 anos e 11 meses de idade com desenvolvimento típico de linguagem, divididos de acordo com a faixa etária. RESULTADOS: Diferença significativa foi encontrada entre o grupo de 5 anos e todos os demais grupos etários, bem como para o grupo de 6 anos quando comparado ao grupo de 8, 9, 10 e 11 anos. O grupo de 7 anos não diferiu estatisticamente dos grupos de 6 e 8 anos. Metade do grupo de 7 anos cursava o primeiro ano e a outra metade, o segundo ano, o que pode ter contribuído para a não diferenciação do grupo com as faixas etárias fronteiriças. Não foi encontrada diferença estatisticamente significante entre os grupos a partir dos 8 anos. Observou-se correlação positiva entre o desempenho de compreensão e produção nos grupos etários de 5, 6 e 7 anos, o que não ocorreu para os demais grupos. CONCLUSÃO: O teste diferenciou as faixas etárias de 5, 6 e 7 anos, o que não ocorreu nas faixas etárias a partir dos 8 anos. Também, a partir dos 8 anos, não foi encontrada correlação entre a produção e a compreensão narrativa, sinalizando o domínio de ambas as habilidades a partir desta faixa etária.

Correlation between sleep profile and behavior in individuals with specific learning disorder. / Correlação entre o perfil do sono e o comportamento em indivíduos com transtorno específico da aprendizagem.

PURPOSE: This study aimed to correlate sleep profile and behavior in individuals with Specific Learning Disorder (SLD). METHODS: The Sleep General Habits Questionnaire, Sleep Diary, and Sleep Disturbance Scale for Children (SDSC) were used in analysis of sleep, whereas the Child Behavior Checklist (CBCL) inventory was used in analysis of behavior. RESULTS: 65.5% of the individuals with SLD presented symptoms of sleep disorders, most frequently wakefulness-sleep transition and sleep disturbance total score, which showed values higher than acceptable. In addition, individuals with SLD presented higher sleep latency than those with typical development. Concerning behavior, 72.4% of the individuals with SLD presented clinical condition of behavior problems. In the control group, none of the participants showed symptoms of sleep or behavior problems. In the SLD group, correlation was observed between behavioral problems and sleep disturbance. CONCLUSION: Individuals with SLD showed high rates of sleep disturbance and behavioral problems. The worse the sleep disturbance, the worse the behavioral aspects in these individuals.

OBJETIVO: Correlacionar o sono e o comportamento em indivíduos com transtorno específico da aprendizagem. MÉTODO: Na análise do sono, foram utilizados o Questionário de Hábitos Gerais de Sono, o Diário de Sono e a Escala de Distúrbios do Sono em Crianças (EDSC) e, para análise do comportamento, foi utilizado o Child Behavior Checklist (CBCL). RESULTADOS: 65,5% dos indivíduos com transtorno específico de aprendizagem apresentaram indicativo de distúrbios de sono, sendo os mais frequentes os distúrbios de transição sono-vigília e escores totais para distúrbios de sono acima do aceitável. Além disso, os indivíduos com transtorno específico de aprendizagem apresentaram maior latência de sono que o respectivo grupo controle. Quanto ao comportamento, 72,4% dos indivíduos com transtorno específico de aprendizagem apresentaram quadro clínico de problemas comportamentais. No grupo controle, nenhum dos participantes apresentou indicativo de problemas de sono ou comportamento. No grupo transtorno específico de aprendizagem, os distúrbios de sono encontrados apresentaram correlação com os problemas comportamentais. CONCLUSÃO: Indivíduos com transtorno específico da aprendizagem apresentaram altos índices de distúrbios de sono e alterações comportamentais. Quanto piores os distúrbios de sono, piores foram os aspectos comportamentais dos indivíduos com transtorno específico da aprendizagem.

Hearing Screening in children with Congenital Zika Virus Syndrome in Fortaleza, Ceará, Brazil, 2016. / Triagem auditiva de crianças com síndrome congênita pelo vírus Zika atendidas em Fortaleza, Ceará, 2016.

OBJECTIVE: to describe the results of hearing screening performed in children with Congenital Zika Virus Syndrome (CZS) in Fortaleza, Ceará, Brazil. METHODS: this was a descriptive cross-sectional study involving children with CZS receiving health care in Fortaleza, 2016; the hearing screening tests performed were immittance audiometry, transient otoacoustic emissions (TOAE), acoustic reflexes, and cochleopalpebral reflex (CPR). RESULTS: The study included 45 children with an average age of 10 months. 44 of them underwent tympanometric screening, with 16 of these having the right ear within the normal range and 22 having the left ear within the normal range. Among the 43 children evaluated by TOAE, 30 "passed" in both ears, nine "refered" in both ears and four "refered" just in ear; 13/43 "refered" and needed to repeat screening. 43 children evaluated by CPR, 37 showed responses. CONCLUSION: most of the children evaluated had completed cochlear function and middle ear results refer in compatible with their age range.

Oral narrative of individuals with Fetal Alcohol Spectrum Disorder. / Narrativa oral de indivíduos com Transtorno do Espectro Alcoólico Fetal.

PURPOSE: To investigate and compare the oral narrative of individuals with FASD and individuals with typical language development (TLD), as well as to correlate the narrative performance with the score from 4-Digit Diagnostic Code. METHODS: Participants were 20 individuals with FASD, of both genders, with chronological age between 6 and 16 years, and 20 individuals with TLD, same gender and similar to the FASD group in age and socioeconomic status. The oral narrative was elicited using the book Frog, where are you? and the data were analyzed in terms of macrostructure, microstructure and global coherence level. Measures regarding the macrostructure included the presence of typical structural elements of storytelling, while the microstructural aspects included words (total and different words), communication units (C-Units), lexical diversity, and mean length of C-Units. RESULTS: Low performance was found in the FASD group for all macrostructural aspects, with the exception of linguistic markers. Among the microstructural aspects, lexical diversity and incomplete C-Units were different between the FASD and TLD groups. The FASD group presented lower global coherence level compared to the TLD group. Negative correlations were found between macrostructural aspects, facial characteristics, and Central Nervous System impairment. CONCLUSION: Restricted use of typical structural elements of storytelling with lower levels of coherence and reduced vocabulary distinguished the FASD from the TDL group. Future studies may explore whether the association between narrative performance and the 4-Digit Diagnostic Code items present predictive values in the narrative performance of individuals with FASD.

Qualitative and quantitative analysis of oropharyngeal swallowing in Down syndrome. / Análise qualitativa e quantitativa da deglutição orofaríngea na Síndrome de Down.

PURPOSE: To describe the qualitative and quantitative temporal analysis of oropharyngeal swallowing in children diagnosed with Down syndrome (DS) through a case series study of six individuals aged 4 to 17 months (mean age = 11.16 months; median = 12 months). METHODS: Qualitative and quantitative temporal analysis of swallowing using videofluoroscopy and specific software. The following parameters were assessed: presence or absence of oral incoordination, labial sphincter sealing incompetence, oral residue, posterior oral spillage, laryngotracheal penetration and aspiration, pharyngeal and total oral transit time (TOTT). RESULTS: Qualitative analysis identified individuals with disorders in at least four of the swallowing parameters investigated. Only one individual presented total oral transit time (TOTT) different from the others. No difference was observed between the cases regarding pharyngeal transit time. CONCLUSION: Qualitative swallowing disorders are observed in children with DS, with difference in TOTT only in the case report of the youngest infant.

OBJETIVO: Este estudo tem por objetivo descrever a análise qualitativa e quantitativa temporal da deglutição orofaríngea em crianças com diagnóstico de Síndrome de Down (SD). MÉTODO: Estudo de série de seis casos, com idade variando de quatro a 17 meses (Média de 11,16 meses e mediana de 12 meses). Realizada análise qualitativa e quantitativa temporal da deglutição orofaríngea por meio de videofluoroscopia de deglutição e software específico. Foram analisados os parâmetros qualitativos de coordenação oral, resíduos orais, escape oral posterior, penetração, aspiração laringotraqueal e realizada análise do tempo de trânsito oral total (TTOT) e faríngeo. RESULTADOS: Verificou-se alteração em pelo menos quatro dos parâmetros qualitativos investigados. Somente um dos indivíduos apresentou diferença no TTOT quando comparado com os demais e não houve diferença no tempo de trânsito faríngeo entre os casos. CONCLUSÃO: Houve alterações qualitativas na deglutição em crianças com SD e diferença no TTOT somente no caso de menor faixa etária.

[Genetics and language in Williams-Beuren Syndrome: a distinct neurobehavioral disorder]. / Genética e linguagem na síndrome de Williams-Beuren: uma condição neuro-cognitiva peculiar.

BACKGROUND: genetic, cognitive and language aspects of the Williams-Beuren Syndorme (WBS). AIM: to present a review of the literature about WBS, highlighting its genetic, cognitive and language characteristics. CONCLUSION: the literature indicates that although the etiology of WBS is known, early diagnosis is difficult due to the great variability of its clinical characteristics. This great phenotypic variability has been associated to a deletion of several genes in region 7q 11.23 which includes the elastin gene. The deletion of this gene is identified by the Fluorecent in situ Hibridization test (FISH). The incidence of this syndrome is 1 in every 20,000 birth and is the result of a "de novo" genetic alteration. The syndrome is characterized by an elfin type face, cardiac alterations, cognitive deficits and behavioral aspects that include language. A peculiar cognitive profile has often been described as consisting of outstanding social and verbal skills associated to visuo-spatial impairments. Cognitive deficits are variable and may not be present. Studies that describe language abilities indicate that syntax might be intact or partially intact; speech can be precise and intelligible indicating that the phonological system is preserved. The receptive vocabulary is mentioned in a few studies as being adequate and in others as being impaired according to mental age. Researches have produced incongruent findings regarding the cognitive and linguistic abilities. The correlation between the language and cognitive abilities and the divergent findings presented in the literature will be discussed in this article.

Correlations between behavior, memory, sleep-wake and melatonin in Williams-Beuren syndrome.

Williams-Beuren syndrome (WBS), a neurodevelopmental disorder caused by a microdeletion on chromosomic region 7q11.23, presents with peculiar behavioral and neurocognitive phenotypes that are marked by apparently preserved social and communicative abilities, which contrasts with low overall cognitive and particularly visuospatial performance. In addition, parents often report complaints of sleep disorders and behavioral problems of unknown cause. Sleep is a biological phenomenon that is modulated by the plasma concentration of melatonin and with influence on behavioral aspects and memory. Thus, this study sought to investigate the behavior, memory and the presence of sleep disorders in WBS and to correlate these factors with each other and with the plasma melatonin content. We used the Child Behavior Checklist for ages 6-18 (CBCL), the digit subtest of the Wechsler scale for auditory memory, the visual sequential memory subtest of the Illinois Test of Psycholinguistic Abilities (ITPA) and the Sleep Disturbance Scale for Children (SDSC). Determination of urinary aMT6s, an indirect measure of plasma melatonin content, was held for 72h by ELISA, and the analysis of the circadian rhythm of this content was performed by the Cosinor method. The results of the CBCL showed that 87% of the WBS group presented with a clinical score on the overall competence and total behavioral problems. Furthermore, the behavioral problems that were most frequently reported by parents were anxiety and problems of thought. All individuals with WBS presented with impairments in auditory memory and 47% with impairments in visual sequential memory; 65% of the WBS group presented with an indicative of at least one sleep disorder, where respiratory, initiation and maintenance of sleep (DIMS) and hyperhidrosis were the most frequent disorders. The night time aMT6s levels were lower in individuals with WBS when compared with controls; 53% of the WBS group did not present with circadian rhythm variations in aMT6s levels. In addition, there was a negative correlation between the scores of auditory memory and the total score of sleep disorders and between the DIMS and nocturnal aMT6s content. In conclusion, in the present study, individuals with WBS showed a high frequency of behavioral and memory problems, sleep disturbances and no rhythm variation in aMT6s levels. The low melatonin content may be related with sleep disorders in this population, which, in turn, can have an adverse effect on specific cognitive skills such as memory.

Language, behavior and neurodevelopment in Joubert syndrome: a case report. / Linguagem, comportamento e neurodesenvolvimento na Síndrome de Joubert: relato de caso.

The Joubert syndrome (JS) is a rare, heterogeneous genetic condition among the ciliopathies. More than 20 genes have been identified associated with this phenotype. The main manifestations include hypotonia, ataxia, psychomotor retardation, ocular-motor apraxia and neonatal respiratory abnormalities. The objective of this paper was to present language and neurodevelopmental findings of an individual diagnosed with JS. The following procedures were performed: anamnesis, clinical genetic evaluation observation of communicative behavior, evaluation of language, the Denver Developmental Screening Test II (DDST-II) and the Early Language Milestone Scale (ELMS). The main findings of the MRI brain showed severe hypoplasia of the cerebellar vermis, "molar tooth sign", hypoplastic brain stem and atrophy of the cerebellar hemispheres. The observation and evaluation of the language showed no oral, impaired reception of language, confirming the diagnosis of language disorder with severe degree of impairment. The DDST-II and the ELMS confirmed the observation and clinical assessment and indicated serious delay in motor domains, self-care and receptive and expressive language. Given the presence of hypotonia, ataxia, delayed psychomotor and neonatal respiratory abnormalities it is essential to carry out examination imaging and genetic evaluation for the diagnosis of this condition, so complex, with unique therapeutic needs. This set of findings, along with the familial history and unique phenotypic characteristics reinforce the clinical genetic diagnosis JS. This genetic syndrome is rarely recognized and deserves to be presented to the recognition of the scientific community, targeting the correct diagnosis and treatment planning that minimizes the deleterious effects of this condition.