Alexandra Prufer de Queiroz Campos Araújo

Vascular Perfusion Abnormalities in Infants with Spinal Muscular Atrophy

Spinal muscular atrophy (SMA) is an important cause of death in children and SMA type I, also known as Werdnig-Hoffman disease, is the most severe form of this disease. We report 2 cases of infants with SMA I in whom a distal necrosis developed, a feature not previously reported. Poor perfusion, autonomic dysfunction, and position-dependent factors may all play a role in the development of this complication.

Zika virus-associated neurological disorders: a review

Zika virus, an arbovirus transmitted by mosquitoes of the Aedes species, is now rapidly disseminating throughout the Americas and the ongoing Brazilian outbreak is the largest Zika virus epidemic so far described. In addition to being associated with a non-specific acute febrile illness, a number of neurological manifestations, mainly microcephaly and Guillain-Barré syndrome, have been associated with infection. These with other rarer neurological conditions suggest that Zika virus, similar to other flaviviruses, is neuropathogenic. The surge of Zika virus-related microcephaly cases in Brazil has received much attention and the role of the virus in this and in other neurological manifestations is growing. Zika virus has been shown to be transmitted perinatally and the virus can be detected in amniotic fluid, placenta and foetus brain tissue. A significant increase in Guillain-Barré syndrome incidence has also been reported during this, as well as in previous outbreaks. More recently, meningoencephalitis and myelitis have also been reported following Zika virus infection. In summary, while preliminary studies have suggested a clear relationship between Zika virus infection and certain neurological conditions, only longitudinal studies in this epidemic, as well as experimental studies either in animal models or in vitro, will help to better understand the role of the virus and the pathogenesis of these disorders.

Desempenho escolar e transtorno do déficit de atenção e hiperatividade

OBJETIVO: esta revisao visa fornecer aos pediatras, neurologistas infantis e psiquiatras infantis nocoes atualizadas sobre a relacao entre desempenho escolar e transtorno do deficit de atencao e hiperatividade (TDAH). FONTE DE DADOS: revisao abrangente nao-sistematica da literatura medica sobre a relacao entre desempenho escolar e TDAH. SINTESE DE DADOS: os dados da literatura demonstram que o TDAH, principalmente o tipo desatento esta relacionado a mau desempenho escolar. CONCLUSOES: criancas com TDAH estao sob risco de mau-desempenho escolar e devem receber cuidados especiais.

Prevalência do transtorno do déficit de atenção e hiperatividade e suas comorbidades em uma amostra de escolares

ABSTRACT - Attention deficit hyperactivity disorder (ADHD) is a frequent condition in school-age children and commonly presents in comorbidity with other psychiatric diseases. In Brazil, there are few studies con-cerning non-clinical samples. Purpose: The present study aims to calculate the prevalence of this disorder and its comorbidities in a sample of school-age children. Method: Cross-sectional analytic study was con-ducted on a non-clinical sample of children and adolescents registered in 2003 in the elementary school of the Federal University of Rio de Janeiro. A screening questionnaire was used and parents of those possible affected children were invited for a clinical structured interview. Results: The prevalence of ADHD was 8.6%. Comorbidities were present in 58% of the cases and oppositional-defiant disorder was the most com-mon, found in 38.5%. Conclusions: The prevalence of the ADHD and its comorbidities in this sample is sim-ilar to that observed in the literature. KEY WORDS: attention deficit disorder with hyperactivity, child; adolescent, comorbidity, prevalence.A prevalencia do transtorno do de fi cit de atencao e hiperatividade (TDAH) varia entre 3 e 5% das crian-cas em idade escolar

Transtorno do déficit de atenção e hiperatividade (TDAH): aspectos relacionados à comorbidade com distúrbios da atividade motora

This paper addresses the physiopathological and clinical aspects related to Attention-Deficit Hyperactivity Disorder in particular the ones concerning the association of this disorder with the Coordination Development Disorder. The Medline database was used to survey indexed articles from 1965 thru 2004. As a result of the articles selected, others were obtained through the relevance assigned to them in the initial sources. Hereditary predisposal of this condition is unquestionable as well as the presence of noradrenergic and dopaminergic dysfunction in the pre-frontal cortex and connections. Notwithstanding this condition diagnosis is based on clinical data. Morbid associations occur in approximately half of the individuals and the prevailing comorbidities as of psychiatric nature. The presence of co-morbidity may modify the therapy and prognosis. The Coordination Development Condition, also stemming from clinical diagnosis is cause for a worse prognosis to children sharing the same conditions. An active search of associated conditions should be performed in each of the children diagnosed.

Juvenile Human T Lymphotropic Virus Type 1-Associated Myelopathy

We report the cases of 5 adolescents with human T lymphotropic virus type 1-associated myelopathy/tropical spastic paraparesis, acquired in all but 1 case from the mother. The first symptom in all patients was difficulty in running, which was present for many years before the final diagnosis was made. Follow-up showed an indolent progression, regardless of treatment strategy.

O retardo mental na distrofia muscular de Duchenne

OBJETIVO: Fazer um levantamento da literatura medica destinada ao estudo das disfuncoes cognitivas nos pacientes com distrofia muscular de Duchenne, atraves da descricao dos marcos do desenvolvimento neuropsicomotor e dos testes psicometricos para quantificacao da inteligencia. FONTES DOS DADOS: Revisao nao sistematica sobre os aspectos da cognicao na distrofia muscular de Duchenne nas principais bases medicas cientificas: MEDLINE, LILACS, Biblioteca Cochrane e SciELO. SINTESE DOS DADOS: Os pacientes com distrofia muscular de Duchenne apresentaram atraso para marcha e desenvolvimento da linguagem, os quais se correlacionaram a menores pontuacoes nos testes de inteligencia no futuro. Ha marcante disfuncao nos subtestes das habilidades verbais. CONCLUSOES: A media do coeficiente de inteligencia encontra-se com um desvio padrao abaixo da media populacional. Quanto maior a disfuncao cognitiva, piores serao os aspectos relacionados a morbidade e mortalidade na doenca.OBJECTIVE To survey the medical literature directed to the study of cognitive dysfunction in patients with Duchenne muscular dystrophy through description of the milestones of neurological development and psychometric tests for quantifying intelligence. SOURCES Non-systematic review of aspects of cognition in Duchenne muscular dystrophy in the major medical scientific bases: MEDLINE, LILACS, SciELO and Cochrane Library. SUMMARY OF THE FINDINGS Patients with Duchenne muscular dystrophy exhibited delay in walking and language development, which correlated with lower scores on future intelligence tests. There is marked impairment in the verbal subtests. CONCLUSIONS Average IQ has standard deviation below the average of the population. The greater the cognitive impairment, the worse aspects related to morbidity and mortality in the disease will be.

Self-reported quality of life has no correlation with functional status in children and adolescents with spinal muscular atrophy ☆

BACKGROUND Spinal muscular atrophy is one of the most common neuromuscular disorders in children. Associated with progressive muscular weakness, it may assume a chronic course. In chronic disorders it is of utmost importance to determine the quality of life level. OBJECTIVE To determine the level of quality of life in a cohort of SMA children and adolescents, and study its relation to motor ability. METHODS From the children and adolescents with confirmed SMA diagnosis (presence of deletion) followed at a University Hospital, we selected those that were 4 years or older. They were classified as SMA type II or III according to their best motor ability, evaluated according to the modified Hammersmith functional score, and undertook the AUQEI Portuguese version to determine quality of life. This is an Institutional Review Board approved study and consent was given by all those included. RESULTS Thirty-three children and adolescent with a mean age of 10.28 (± 4.71) took part of the study. The fourteen SMA type II had a mean Hammersmith score of 11 (± 9.50) and AUQEI of 55.85 (± 7.16), while the nineteen SMA type III scored 31.10 (± 12.30) and 52.94 (± 4.85). No significant difference was found when quality of life scores was compared among those groups. CONCLUSION On a self-reported scale it seems that regardless the functional status an SMA child and adolescent has a perception of good quality of life.

Academic Performance in ADHD When Controlled for Comorbid Learning Disorders, Family Income, and Parental Education in Brazil

Objective: Scholastic achievement in a nonclinical sample of ADHD children and adolescents was evaluated taking into consideration variables such as comorbid learning disorders, family income, and parental education which may also be associated with poor academic performance. Method: After screening for ADHD in 396 students, the authors compared academic performance of 26 ADHD individuals and 31 controls paired for gender, age, and intelligence level considering both mathematics and Portuguese language scores. Learning disorders were investigated and the Diagnostic and Statistical Manual of Mental Disorders (4th ed.; DSM-IV ) criteria were met using structured interviews. Results: The prevalence of academic underachievement was 2.98 times higher in students with ADHD, the most frequent subtype being predominantly inattentive. Parental educational level, family income, and comorbid learning disorders could not explain the discrepancies between ADHD students and controls. Conclusions: ADHD seems to be associated with poor academic performance even in the absence of comorbid learning disorders, lower family income, and parental educational level. (J. of Att. Dis. 2009; 12(5) 469-473)

Management of rheumatic chorea: an observational study

BACKGROUND Rheumatic chorea (RC) has recently been linked to an antibody-mediated immune mechanism. OBJECTIVE/METHOD To verify if this knowledge reflected in management changes we conceived a descriptive study. RESULTS The medical charts of 20 children (13 females) aged 6 to 12 years (mean 8 years), diagnosed as RC from June 1996 to June 1999, were reviewed. All patients received some medical treatment. Haloperidol was the most prescribed medication (15 patients - 75 %). Sulpiride, diazepam and valproate were also used as symptomatic treatment. Immune-modulating therapy with prednisone was prescribed for seven children. The shortest course of chorea (16 days) occurred in a patient treated with prednisone. CONCLUSION Prednisone has been prescribed for rheumatic chorea besides the traditional symptomatic approach. A great variety of antichoreic drugs are being employed.