Magda Lahorgue Nunes

Distúrbios da aquisição da linguagem e da aprendizagem

OBJETIVO: O objetivo deste artigo e instrumentalizar os profissionais da saude, em especial o pediatra, para que possam agir no diagnostico e na prevencao primaria dos disturbios de linguagem oral e escrita. FONTES DOS DADOS: Foi realizada revisao da literatura sobre o tema proposto na MEDLINE nos ultimos 5 anos e foram incluidas referencias de livros-texto considerados como base para a compreensao dos disturbios da linguagem e do aprendizado na infância. SINTESE DOS DADOS: Sabe-se que as causas de alteracoes de linguagem e de dificuldades de aprendizagem podem ser variadas, apesar de existirem muitos estudos indicando fatores neurologicos para tais problemas. CONCLUSAO: Ressalta-se a importância de uma adequada investigacao buscando um correto diagnostico para, entao, direcionar o melhor tratamento indicado para cada caso.

Developmental regulation of regional functionality of substantia nigra GABAA receptors involved in seizures

GABAergic (gamma-aminobutyric acid) transmission in the substantia nigra pars reticulata is critical for seizure control. We tested the hypothesis that there is a differential regional distribution and functionality of nigral GABAA receptor sites that is developmentally regulated. In adult rats, we determined the effects on flurothyl seizures of (Z)-3-[(aminoiminomethyl)thio]prop-2-enoic acid (ZAPA, a presumed agonist of the low-affinity GABAA receptor site), bicuculline (an antagonist of the low-affinity GABAA receptor site) and gamma-vinyl-GABA (a GABA-transaminase inhibitor), infused bilaterally in anterior or posterior substantia nigra pars reticulata. ZAPA infusions (8 micrograms) were anticonvulsant in anterior substantia nigra but proconvulsant in posterior substantia nigra. Bicuculline infusions (100 ng) were proconvulsant in anterior substantia nigra but ineffective in posterior substantia nigra. An anticonvulsant dose of gamma-vinyl-GABA, when infused in anterior substantia nigra, was proconvulsant when infused in posterior substantia nigra. In 15 day old rats, the effects of ZAPA, were biphasic: 2 micrograms was anticonvulsant while 8 micrograms was proconvulsant. There was no regional specificity. The data suggest that with maturation there is functional segregation of specific GABAA receptor subtypes involved in substantia nigra-mediated seizure control.

Clinical assessment of language development in children at age 3 years that were born preterm

OBJECTIVE To evaluate the influence of gestational age and birth weight on language development and neurodevelopmental outcome at age 3 years in children born preterm. METHOD Cross sectional study including 69 children followed in our developmental outpatient clinic. Patients were consecutively included at the time of the 3 years of age appointment and stratified for birth weight (<1500 grams and between 1500-2500 grams). All patients were assessed for receptive and expressive language , Denver II and Bayley II tests and clinical neurological examination. For analysis patients were divided in two groups normal language acquisition (NLA) and delay in language acquisition (DLA). RESULTS NLA children had higher scores on mental and psychomotor (p=<0.01, p=0.012) indexes of Bayley II. Newborns with less than 1500 grams had lower scores on all Bayley scale at age 36 months (p=0.002, p=0.007 and p<0.001). Multivariate analysis suggests an association between gestational age (p=0.032), abnormal behavior (p<0.001) and delay in language acquisition. Denver test at 12 and 24 months of age was a good predictor of delayed receptive and expressive language at three years of age (p=<0.01 and p=<0.01). CONCLUSION Children born prematurely with low birth weight had an increased risk of language acquisition delay, and those had also lower cognitive and behavior scores when compared to NLA.

Evaluation of sleep habits in children with epilepsy

Sleep disturbances are a common complaint among patients with epilepsy. Studies assessing the relationship between sleep and epilepsy during childhood are scarce. The purpose of this study was to evaluate sleep habits in children with epilepsy. This cross-sectional study of children with and without epilepsy employed two questionnaires to evaluate sleep habits. Characteristics of both sleep habits and epilepsy (type of seizure, epileptic syndrome, number of seizures, use of anticonvulsant drugs) were collected from parental interviews and medical records. Our results indicate that children with epilepsy have a greater incidence of sleep problems compared with children without epilepsy. In those with epilepsy, we observed that nocturnal seizures, polytherapy, developmental delay, refractory epilepsy, generalized seizures, and epileptic syndromes with an unfavorable outcome are associated with poor sleep habits.

Sleep Organization in Children With Partial Refractory Epilepsy

Although it is currently known that sleep can influence epilepsy and epilepsy can influence sleep organization, few data have been published on this mutual interaction concerning the pediatric population. The objective of this study was to verify the eventual presence of sleep alterations in children with partial refractory epilepsy. Seventeen patients with partial refractory epilepsy were submitted to whole-night polysomnography as part of their epilepsy investigation. Polysomnographic recordings were performed on a digital video-electroencephalography (EEG) system and consisted of the registration of EEG (24 channels), electro-oculogram, electromyogram, electrocardiogram, and nasal airflow and abdominal respiratory movements. Sleep stages were visually scored following standard criteria, and ictal events were classified according to the international classification of seizures. The patients were also subdivided into two subgroups based on the presence or absence of ictal episodes during the recording night. The results concerning sleep organization were compared with those obtained from a normal control group. The analysis of the sleep parameters showed a reduction of total time in bed and total sleep time in both subgroups of epileptic children; there was a higher number of stage shifts per hour in the control group than in both epileptic subgroups. The percentage of stage 2 shifts is significantly reduced in patients with epilepsy and seizures during the night and the percentage of stage 3 to 4 shifts is increased. Nonsignificant differences are evident for the number of awakenings per hour and the percentage of stage 1 shifts. The percentage of rapid eye movement (REM) sleep is reduced, and first REM latency is increased in both epileptic subgroups, compared with normal controls, without statistical significance. Nine of 17 patients had seizures during the polysomnographic recording; nocturnal ictal events occurred mostly during non-REM sleep stage 2. Our results show that patients with partial refractory epilepsies have only mild sleep structure abnormalities, and this can be considered as an effect of the epileptic syndrome per se or as a result of the chronic antiepilepsy drug treatment. (J Child Neurol 2003;18:763—766).

Duration of rhythmic EEG patterns in neonates: new evidence for clinical and prognostic significance of brief rhythmic discharges.

OBJECTIVE This study aimed at identifying the characteristics - especially the duration - of rhythmic discharges in neonatal EEG, and their association with clinical neonatal problems. Specifically, we aimed at testing the diagnostic and prognostic validity of using 10 s as minimal duration for defining electroencephalographic seizures. DESIGN AND METHODS The polysomnographies of 340 neonates were reviewed, and episodes of rhythmic discharges were identified, analyzed, and quantified. The study sample was divided into 3 groups: one in which the maximal duration of rhythmic discharges was shorter than 10 s (brief rhythmic discharges, BRD), a second one in which there were rhythmic discharges longer than 10 s (long rhythmic discharges, LRD), and finally a group in which no rhythmic discharge was found (No-RD). These 3 groups of subjects were compared for the baseline and outcome clinical data. RESULTS From the 340 neonates studied, 210 did not present any form of rhythmic discharge, 67 (19. 7%) had only BRD episodes, and 63 (18.5%) had at least one LRD episode. Prevalence of rhythmic discharges was low among healthy full term newborns, and was significantly higher among preterm and high-risk newborns. Electrophysiological characteristics of rhythmic discharges did not differ between healthy neonates and high-risk ones. Accompanying clinical manifestations were present in 26.3% of the LRD group, but also in 15.9% of the BRD group. The presence of BRD was significantly associated with leukomalacia and with hypoglycemia in the cross-sectional analysis of baseline data, and with an increased risk for abnormal neurodevelopmental outcome after a mean follow-up period of 47 months (adjusted relative risk=4.90, P<0.01). CONCLUSIONS The present data demonstrate an association between BRD and clinical history of hypoxic-ischemic encephalopathy, especially when complicated by leukomalacia, and also with a prognosis of increased risk for abnormal neurodevelopmental outcome. The clinical and prognostic significance of isolated BRD justifies the need to include these brief episodes in future studies of neonatal seizures.

Heart rate variability during sleep in children with partial epilepsy.

Alterations in autonomic control of cardiac activity in epileptic patients have been reported by several studies in the past, and both ictal and interictal modifications of heart rate regulation have been described. Alterations of autonomic control of cardiac activity can play an important role in sudden unexplained death in patients with epilepsy (SUDEP). However, the presence of specific changes in heart rate variability (HRV) during sleep, not correlated with seizures, has not been assessed in children with epilepsy; for this reason, we evaluated features of cardiac autonomic function during sleep without ictal epileptiform electroencephalogram (EEG) activity in a group of children with partial epilepsy. Eleven patients (five males and six females; mean age 11.5 years, SD: 3.65 years) affected by partial epilepsy were admitted to this study; 11 normal subjects (five males and six females; mean age 12.9 years, SD: 2.72 years) served as a control group. All subjects slept in the laboratory for two consecutive nights. The data were analyzed during the second night. Sleep was polygraphically recorded [including one electrocardiography (ECG) channel] and signals were digitally stored. A series of 5‐min ECG epochs were chosen from each sleep stage, during periods without evident ictal epileptiform activity in the EEG. Electrocardiography signals were analyzed for automatic detection of R‐waves and, subsequently, a series of time‐ and frequency‐domain measures were calculated. Epileptic subjects tended to show an overall lower HRV in both time‐ and frequency‐domain parameters, principally during rapid eye movement (REM) sleep and, to a lesser extent, during sleep stage 2. Among the different bands, this decrease was most evident for the high‐frequency band (HF) absolute power. For this reason, the ratio between the low‐frequency band (LF) and HF was always higher in epileptic patients than in normal controls and the difference was statistically significant during sleep stages 3 and/or 4 and REM sleep. Our results indicate that during sleep, a particular condition of basal modification in autonomic characteristics occurs (mostly during REM sleep) in partial epilepsy patients. This finding might represent an important factor contributing to the complex mechanism of SUDEP which takes place most often during sleep and supports the need of studying HRV specifically during this state in subjects with seizures.

Sono e envelhecimento

OBJETIVO: descrever aspectos atualizados sobre as alteracoes e os disturbios do sono no processo de envelhecimento normal e usual. METODOS: procedeu-se a revisao da arquitetura do sono e ritmo circadiano de sono e vigilia para estabelecer o padrao de normalidade e as alteracoes fisiologicas no processo de envelhecimento, descrevendo-se os disturbios de sono mais prevalentes no idoso, sua avaliacao diagnostica e o manejo nao-farmacologico. O texto foi elaborado a partir da consulta as publicacoes cientificas indexadas no Medline, em outras de acesso on line e em livros textos das areas de geriatria, psiquiatria geriatrica e medicina do sono. RESULTADOS E CONCLUSAO: a maioria dos idosos tem queixas relacionadas ao sono decorrentes de mudancas fisiologicas especificas do processo de envelhecimento ou de doencas que podem causar disturbios secundarios de sono. A linha divisoria entre a normalidade e o disturbio pode ser estabelecida por meio de criteriosa avaliacao diagnostica, a qual necessariamente precede e orienta a conduta terapeutica. A maioria dos disturbios de sono sao clinicamente importantes e trataveis com medidas nao-farmacologicas, que incluem a orientacao sobre rotinas e rituais de sono, atividades de vida diaria e condicoes ambientais.AIM: to describe the up-to-date aspects on sleep changes and disturbances in the normal and usual aging process. METHODS: the sleep architecture and the sleep and vigil circadian rhythm were reviewed in order to establish the normalcy pattern and physiological changes in the aging process, describing the most prevalent sleep disturbances of the elderly, their diagnostic evaluation and non-pharmacological management. The text was drawn up based on consulting scientific publications indexed in Medline, in other on-line access publications and in textbooks in the fields of geriatrics, geriatric psychiatry and sleep therapy. RESULTS AND CONCLUSION: Most of the elderly have sleep-related complaints resulting from specific physiological changes that are specific to the aging process or to diseases that may cause secondary sleep disturbances. The borderline between normalcy and disturbance may be established by means of a judicious diagnostic evaluation that necessarily precedes and guides the therapeutic management. Most sleep disturbances are clinically significant and can be treated with non-pharmacological measures, which include guidance concerning sleep routines and rituals, everyday life activities and environmental conditions.

Cognitive and behavioral status of low birth weight preterm children raised in a developing country at preschool age

Objective: To assess cognitive and behavioral development at preschool age of children born preterm and with low birth weight and raised in a developing country. Methods: Prospective cross-sectional study of 80 neonates born in a university hospital in southern Brazil. Neuropsychological assessment at age 4-5 years included the Wechsler Preschool and Primary Scale of Intelligence (WPPSI), Conners’ Parent Rating Scale-Revised, Denver test and neurological examination. Perinatal and early neuropsychomotor development information was collected from the database during follow-up. Results were related to birth weight, sex and gestational age. Results: WPPSI scores were: total intelligence quotient (IQ) 88.00±16.96, verbal IQ 89.72±16.72, and executive IQ 88.12±15.71 for the group with less than 1,500 grams; and total IQ 91.11±14.73, verbal IQ 93.36±12.65, and executive IQ 90.20±16.06 for the group between 1,500 and 2,500 grams. The best scores were obtained in tests that evaluated capacity of abstraction and symbolization, picture completion and common perception, in which only 5% and 6.3% of the children had abnormal results, respectively. The lowest scores were obtained in tests that evaluated visual-motor coordination and flexibility-speed of reasoning, in which 27.5% and 16.3% of the children had abnormal results, respectively. A total of 32.5% had abnormal results in the arithmetic tests. Behaviors related to attention deficit/ hyperactivity disorder (ADHD) were observed in 48% of the sample. Low score in the Bayley Mental Scale and abnormal result in the Denver test were significantly correlated to ADHD (p = 0.017 an dp=0 .004). Abnormal results in the Bayley Mental Scale (p < 0.001), Denver test (p < 0.001) and neurological examination (p = 0.002) were associated with lower IQ.

Predictive value of sequential electroencephalogram (EEG) in neonates with seizures and its relation to neurological outcome.

The aim of this study was to evaluate the relationship of sequential neonatal electroencephalography (EEG) and neurological outcome in neonates with seizures to identify polysomnographic features predictive of outcome. Sequential EEGs recordings of 58 neonates that belonged to 2 historical cohorts of newborns with seizures from the same neonatal intensive care unit and who had follow-up at the Neurodevelopment Clinic of the Hospital São Lucas, Pontifícia Universidade Católica do Rio Grande do Sul (PUCRS) in Porto Alegre, Brazil, were analyzed and classified into 4 groups: normal-normal, abnormal-normal, abnormal-abnormal, normal-abnormal. In patients with more than 2 recordings, during the neonatal period, the first EEG was compared with the following more abnormal. A total of 58 pairs of 2 sequential EEGs were analyzed. Considering the first EEG, a statistically significant difference was observed between the relationship of the result of this exam, if it was abnormal, with developmental delay (P = .030) and postnatal death (P = .030). Abnormal background activity was also related to neurodevelopment delay (P = .041). EEG sequences abnormal-abnormal and normal-abnormal significantly correlated to the outcome epilepsy ( P = .015). Abnormal sequential background activity was associated with neurodevelopment delay (P = .006) and epilepsy (P = .041). The burst suppression pattern when present in any EEG correlated with epilepsy (P = .013) and postnatal death (P = .034). Sequential abnormal background patterns in the first and second EEG increased the risk for epilepsy (relative risk [RR] = 1.8; 95% confidence interval [CI] = 1.03-3.0) and neurodevelopment delay (RR = 2.20; 95% CI = 1.3-3.0). Abnormal background activity only in the second electroencephalogram increased the risk for neurodevelopment delay (RR = 2.20; 95% CI = 1.3-3.0). All the neonates (n = 33) with seizures related to probable hypoxic ischemic encephalopathy had abnormalities in the first EEG (P = .030). Postnatal epilepsy was diagnosed in 24 infants (41.4%). Five (20.8%) presented West syndrome, 7 (29.2%) focal symptomatic epilepsy, 6 (25%) generalized symptomatic epilepsy, 2 (8.3%) early myoclonic encephalopathy, 1 (4.2%) early infantile epileptic encephalopathy, and in 3, the epileptic syndrome was undefined (12.5%). All infants (n = 5) with West syndrome had some degree of neurodevelopment delay. In conclusion, our findings suggest that sequential EEG in neonates with seizures has more predictive value to estimate the outcomes of neurodevelopment delay, epilepsy, and postnatal death than a single EEG recording. The abnormal background activity in even 1 EEG of the sequential recordings was more significant to determine neurological outcome than abnormal ictal activity or abnormalities in the organization of sleep state.