This review article aimed to present a clinical approach, emphasizing the diagnostic investigation, to children and adolescents who present in the emergency room with acute-onset muscle weakness.
A ...systematic search was performed in PubMed database during April and May 2017, using the following search terms in various combinations: “acute,” “weakness,” “motor deficit,” “flaccid paralysis,” “child,” “pediatric,” and “emergency”. The articles chosen for this review were published over the past ten years, from 1997 through 2017. This study assessed the pediatric age range, from 0 to 18 years.
Acute motor deficit is a fairly common presentation in the pediatric emergency room. Patients may be categorized as having localized or diffuse motor impairment, and a precise description of clinical features is essential in order to allow a complete differential diagnosis. The two most common causes of acute flaccid paralysis in the pediatric emergency room are Guillain–Barré syndrome and transverse myelitis; notwithstanding, other etiologies should be considered, such as acute disseminated encephalomyelitis, infectious myelitis, myasthenia gravis, stroke, alternating hemiplegia of childhood, periodic paralyses, brainstem encephalitis, and functional muscle weakness. Algorithms for acute localized or diffuse weakness investigation in the emergency setting are also presented.
The clinical skills to obtain a complete history and to perform a detailed physical examination are emphasized. An organized, logical, and stepwise diagnostic and therapeutic management is essential to eventually restore patient's well-being and full health.
O objetivo deste artigo de revisão é apresentar uma abordagem clínica, enfatizando a investigação diagnóstica, voltada para crianças e adolescentes no pronto socorro com fraqueza muscular de surgimento agudo.
Foi realizada uma pesquisa sistemática na base de dados PubMed entre abril e maio de 2017, utilizando os seguintes termos de pesquisa em várias combinações: “agudo”, “fraqueza”, “déficit motor”, “paralisia flácida”, “criança”, “pediátrico” e “emergência”. Os trabalhos escolhidos para esta revisão foram publicados nos últimos dez anos, de 1997 a 2017. Este trabalho aborda a faixa etária pediátrica, de 0 a 18 anos.
O déficit motor agudo é uma causa razoavelmente comum para crianças e adolescentes procurarem o pronto socorro. Os pacientes podem ser classificados como apresentando deficiência motora localizada ou difusa, e uma descrição precisa das características clínicas é essencial para possibilitar um diagnóstico diferenciado completo. As duas causas mais comuns de paralisia flácida aguda no pronto socorro pediátrico são síndrome de Guillain-Barré e mielite transversa, independentemente de outras etiologias serem consideradas, como encefalomielite disseminada aguda, mielite infecciosa, miastenia grave, derrame, hemiplegia alternante da infância, paralisia periódica, encefalite do tronco encefálico e fraqueza muscular funcional. Os algoritmos da investigação de fraqueza aguda localizada ou difusa na configuração de emergência também são apresentados.
São enfatizadas as habilidades clínicas para obter um histórico completo e realizar um exame físico detalhado. Um manejo diagnóstico e terapêutico organizado, lógico e por etapas é essencial para eventualmente restaurar o bem-estar e a saúde total do paciente.
To describe the effect of prednisolone on language in children with autism spectrum disorder. This study is based upon two hypotheses: autism etiology may be closely related to neuroinflammation; ...and, an effective treatment should restore the individual's language skills.
This is a prospective, double-blinded, randomized, placebo-controlled clinical trial, carried out in a federal university hospital. The initial patient sample consisted of 40 subjects, which were randomized into two parallel groups. Inclusion criteria were: male gender, 3–7 years of age, and meeting the Diagnostic and Statistical Manual of Mental Disorders – 4th edition (DSM-IV) diagnostic criteria. The final sample consisted of 38 patients, of whom 20 were randomized to the placebo group and 18 to the active group. The latter received prednisolone for 24 weeks, at an initial dose of 1mg/kg/day and a tapering dose from the ninth week onward. Language was measured on four occasions over a 12-month period by applying two Brazilian tools: the Language Development Assessment (ADL) and the Child Language Test in Phonology, Vocabulary, Fluency, and Pragmatics (ABFW).
The side effects were mild: two patients had hypertension, five had hyperglycemia, and two had varicella. Prednisolone increased the global ADL score in children younger than 5 years of age who had developmental regression (p=0.0057). The ABFW's total of communicative acts also responded favorably in those participants with regression (p=0.054). The ABFW's total of vocal acts showed the most significant results, especially in children younger than 5 years (p=0.004, power=0.913).
The benefit of prednisolone for language scores was more evident in participants who were younger than five years, with a history of developmental regression, but the trial's low dose may have limited this benefit. The observed side effects do not contraindicate corticosteroid use in autism.
ABSTRACT
OBJECTIVE
To analyze and compare cerebral white matter tracts through diffusion tensor imaging in autistic and normal children.
METHODS
This is a case‐control study on a sample of eight ...male, right‐handed children diagnosed with autism according to Diagnostic and Statistical Manual of Mental Disorders‐4th Edition criteria, and eight healthy age‐ and sex‐matched controls. Imaging studies were performed on a 1.5‐T scanner (Symphony Maestro Class, Siemens, Erlangen, Germany). Fractional anisotropy was calculated for the frontopontine and corticospinal tracts, frontal subcortical white matter, anterior cingulate, corpus callosum, striatum, internal capsule, optic radiation, superior and inferior longitudinal fascicles, and cerebellum. Analysis of significance was based on analysis of variance test for the mean fractional anisotropy values.
RESULTS
Median age of cases was 9.53 ± 1.83 years, and of controls, 9.57 ± 1.36 years. Diffusion tensor imaging findings included significant reduction of fractional anisotropy in the anterior corpus callosum (P= .008), right corticospinal tract (P= .044), posterior limb of right and left internal capsules (P= .003 and .049, respectively), left superior cerebellar peduncle (P= .031), and right and left middle cerebellar peduncles (P= .043 and .039, respectively) in autistic children.
CONCLUSIONS
The diffusion tensor imaging findings in children with autistic disorder suggest impairment of white matter microstructure, possibly associated with reduced connectivity in corpus callosum, internal capsule, and superior and middle cerebellar peduncles.
Objectives: This review article aimed to present a clinical approach, emphasizing the diagnostic investigation, to children and adolescents who present in the emergency room with acute-onset muscle ...weakness. Sources: A systematic search was performed in PubMed database during April and May 2017, using the following search terms in various combinations: “acute,” “weakness,” “motor deficit,” “flaccid paralysis,” “child,” “pediatric,” and “emergency”. The articles chosen for this review were published over the past ten years, from 1997 through 2017. This study assessed the pediatric age range, from 0 to 18 years. Summary of the data: Acute motor deficit is a fairly common presentation in the pediatric emergency room. Patients may be categorized as having localized or diffuse motor impairment, and a precise description of clinical features is essential in order to allow a complete differential diagnosis. The two most common causes of acute flaccid paralysis in the pediatric emergency room are Guillain–Barré syndrome and transverse myelitis; notwithstanding, other etiologies should be considered, such as acute disseminated encephalomyelitis, infectious myelitis, myasthenia gravis, stroke, alternating hemiplegia of childhood, periodic paralyses, brainstem encephalitis, and functional muscle weakness. Algorithms for acute localized or diffuse weakness investigation in the emergency setting are also presented. Conclusions: The clinical skills to obtain a complete history and to perform a detailed physical examination are emphasized. An organized, logical, and stepwise diagnostic and therapeutic management is essential to eventually restore patient's well-being and full health. Resumo: Objetivos: O objetivo deste artigo de revisão é apresentar uma abordagem clínica, enfatizando a investigação diagnóstica, voltada para crianças e adolescentes no pronto socorro com fraqueza muscular de surgimento agudo. Fontes: Foi realizada uma pesquisa sistemática na base de dados PubMed entre abril e maio de 2017, utilizando os seguintes termos de pesquisa em várias combinações: “agudo”, “fraqueza”, “déficit motor”, “paralisia flácida”, “criança”, “pediátrico” e “emergência”. Os trabalhos escolhidos para esta revisão foram publicados nos últimos dez anos, de 1997 a 2017. Este trabalho aborda a faixa etária pediátrica, de 0 a 18 anos. Resumo dos dados: O déficit motor agudo é uma causa razoavelmente comum para crianças e adolescentes procurarem o pronto socorro. Os pacientes podem ser classificados como apresentando deficiência motora localizada ou difusa, e uma descrição precisa das características clínicas é essencial para possibilitar um diagnóstico diferenciado completo. As duas causas mais comuns de paralisia flácida aguda no pronto socorro pediátrico são síndrome de Guillain-Barré e mielite transversa, independentemente de outras etiologias serem consideradas, como encefalomielite disseminada aguda, mielite infecciosa, miastenia grave, derrame, hemiplegia alternante da infância, paralisia periódica, encefalite do tronco encefálico e fraqueza muscular funcional. Os algoritmos da investigação de fraqueza aguda localizada ou difusa na configuração de emergência também são apresentados. Conclusões: São enfatizadas as habilidades clínicas para obter um histórico completo e realizar um exame físico detalhado. Um manejo diagnóstico e terapêutico organizado, lógico e por etapas é essencial para eventualmente restaurar o bem-estar e a saúde total do paciente. Keywords: Acute weakness, Motor deficit, Guillain–Barré syndrome, Transverse myelitis, Child, Palavras-chave: Fraqueza aguda, Déficit motor, Síndrome de Guillain–Barré, Mielite transversa, Criança
ABSTRACT
PURPOSE
This study aims to assess cerebral metabolites in school‐aged autistic patients through proton magnetic resonance spectroscopy.
METHODS
This case‐control study included 10 ...right‐handed male children (median age, 9.53 years ± 1.80) with autism according to DSM‐IV criteria, and 10 healthy age‐ and sex‐matched healthy controls (median age, 8.52 years ± 1.42). Imaging was performed on a 1.5‐T scanner utilizing a single voxel point‐resolved spectroscopy (PRESS) technique (TR = 1,500 ms, TE = 30 ms). Four cerebral areas were evaluated: bilateral anterior cingulate, left striatum, left cerebellar hemisphere, and left frontal lobe. Peak areas and ratios to creatine (Cr) of N‐acetylaspartate (NAA), choline (Cho), and myo‐inositol (mI) were analyzed.
RESULTS
Compared with controls, autistic children showed a significant increase in mI (P= .021) and Cho (P= .042) peak areas in anterior cingulate and in mI/Cr ratio in anterior cingulate (P= .037) and left striatum (P= .035). The remaining metabolites and ratios were not significantly different between the 2 groups.
CONCLUSIONS
This study found a statistically significant increase in myo‐inositol and choline in anterior cingulate and left striatum of autistic children compared with controls. In contrast to previous studies, NAA peak area and NAA/Cr and NAA/Cho ratios had no statistically significant decrease in any of the 4brain regions.
This review article aimed to present a clinical approach, emphasizing the diagnostic investigation, to children and adolescents who present in the emergency room with acute‐onset muscle weakness.
A ...systematic search was performed in PubMed database during April and May 2017, using the following search terms in various combinations: “acute,” “weakness,” “motor deficit,” “flaccid paralysis,” “child,” “pediatric,” and “emergency”. The articles chosen for this review were published over the past ten years, from 1997 through 2017. This study assessed the pediatric age range, from 0 to 18 years.
Acute motor deficit is fairly common presentation in the pediatric emergency room. Patients may be categorized as having localized or diffuse motor impairment, and a precise description of clinical features is essential in order to allow a complete differential diagnosis. The two most common causes of acute flaccid paralysis in the pediatric emergency room are Guillain‐Barré syndrome and transverse myelitis; notwithstanding, other etiologies should be considered, such as acute disseminated encephalomyelitis, infectious myelitis, myasthenia gravis, stroke, alternating hemiplegia of childhood, periodic paralyses, brainstem encephalitis, and functional muscle weakness. Algorithms for acute localized or diffuse weakness investigation in the emergency setting are also presented.
The clinical skills to obtain a complete history and to perform a detailed physical examination are emphasized. An organized, logical, and stepwise diagnostic and therapeutic management is essential to eventually restore patient's well‐being and full health.
Apresentar uma abordagem clínica, enfatizar a investigação diagnóstica, voltada para crianças e adolescentes no pronto‐socorro com fraqueza muscular de surgimento agudo.
Foi feita uma pesquisa sistemática na base de dados PubMed entre abril e maio de 2017, com os seguintes termos de pesquisa em várias combinações: “agudo”, “fraqueza”, “déficit motor”, “paralisia flácida”, “criança”, “pediátrico” e “emergência”. Os trabalhos escolhidos para esta revisão foram publicados nos últimos dez anos, de 1997 a 2017. Este trabalho aborda a faixa etária pediátrica, até 18 anos.
O déficit motor agudo é uma causa razoavelmente comum para crianças e adolescentes procurarem o pronto‐socorro. Os pacientes podem ser classificados como com deficiência motora localizada ou difusa e uma descrição precisa das características clínicas é essencial para possibilitar um diagnóstico diferenciado completo. As duas causas mais comuns de paralisia flácida aguda no pronto‐socorro pediátrico são síndrome de Guillain‐Barré e mielite transversa, independentemente de outras etiologias serem consideradas, como encefalomielite disseminada aguda, mielite infecciosa, miastenia grave, derrame, hemiplegia alternante da infância, paralisia periódica, encefalite do tronco encefálico e fraqueza muscular funcional. Os algoritmos da investigação de fraqueza aguda localizada ou difusa na configuração de emergência também são apresentados.
São enfatizadas as habilidades clínicas para obter um histórico completo e fazer um exame físico detalhado. Um manejo diagnóstico e terapêutico organizado, lógico e por etapas é essencial para eventualmente restaurar o bem‐estar e a saúde total do paciente.
Objectives: This review article aimed to present a clinical approach, emphasizing the diagnostic investigation, to children and adolescents who present in the emergency room with acute‐onset muscle ...weakness. Sources: A systematic search was performed in PubMed database during April and May 2017, using the following search terms in various combinations: “acute,” “weakness,” “motor deficit,” “flaccid paralysis,” “child,” “pediatric,” and “emergency”. The articles chosen for this review were published over the past ten years, from 1997 through 2017. This study assessed the pediatric age range, from 0 to 18 years. Summary of the data: Acute motor deficit is fairly common presentation in the pediatric emergency room. Patients may be categorized as having localized or diffuse motor impairment, and a precise description of clinical features is essential in order to allow a complete differential diagnosis. The two most common causes of acute flaccid paralysis in the pediatric emergency room are Guillain‐Barré syndrome and transverse myelitis; notwithstanding, other etiologies should be considered, such as acute disseminated encephalomyelitis, infectious myelitis, myasthenia gravis, stroke, alternating hemiplegia of childhood, periodic paralyses, brainstem encephalitis, and functional muscle weakness. Algorithms for acute localized or diffuse weakness investigation in the emergency setting are also presented. Conclusions: The clinical skills to obtain a complete history and to perform a detailed physical examination are emphasized. An organized, logical, and stepwise diagnostic and therapeutic management is essential to eventually restore patient's well‐being and full health.
Abstract The recent alarming statements concerning the newborn ZIKV-induced microcephaly epidemics in the Northeast of Brazil, released by the Brazilian Ministry of Health, as well as important ...international health agencies, such as the World Health Organization and the Pan American Health Organization, raised many “why and how” questions so far, that will hopefully be scientifically answered, as more researches in that regard come up in the long term. In this paper, we describe another potentially ZIKV-induced central nervous system and musculoskeletal disorder that has accompanied microcephaly in these children: atrhogryposis multiplex congenita. The goal is to bring up some hypotheses for possible underlying molecular mechanisms based on published data taken from animal models, such as ovine and cattle, which once infected by other types of arboviroses and viruses that also belong to the Flaviviridae family, presented, too, with the full-blown CNS spectrum of malformations at birth.
Angelman syndrome is a neurogenetic disorder that severely affects global neurodevelopment due to modifications in the structure or functioning of
UBE3A
gene. Its prevalence ranges from 1:10,000 to ...1:40,000. There are four main genetic types of AS transmission. A maternal deletion in 15q11.2-q13 is the most common type. There are three well-established electroencephalogram (EEG) patterns used as an ancillary tool for AS diagnosis. The main objectives are to scrutinize the EEG patterns in Angelman syndrome, their correlation to different types of seizures and to review the role of the EEG as an ancillary screening tool in the diagnosis of clinically suspected patients. Forty-three patients’ charts and their previously recorded EEGs were reviewed. A set of 34 patients with deletion type, paternal uniparental disomy type and imprint defect type AS were enrolled. AS diagnosis was confirmed either by fluorescent in situ hybridization test or Methylation Specific–Multiplex Ligation Probe Amplification test. Sequencing of
UBE3A
was not available. Frequencies and Chi-square tests were used for statistic analysis. Pattern I type EEG was observed in 22 (64.7 %) individuals. Pattern II accounted for 6 (17.6 %); Pattern III was evident in 11 (32.4 %). The three distinguished EEG patterns, more frequently Pattern I, when observed in the appropriate clinical setting, may heighten the index of suspicion for selecting patients who will need a molecular biology test to confirm the diagnosis of AS.
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