Hemiparetic cerebral palsy: clinical data compared with neuroimaging

OBJETIVO: Este estudo correlacionou o desempenho da função motora fina (FMF) e Sensorial (FS) na paralisia cerebral hemiparética (PC-H) à classificação da ressonância magnética (RM). MÉTODO: Utilizaram-se os protocolos específicos para avaliar FMF, FS e lesões encefálicas quanto à sua extensão e localização no hemisfério. Foram avaliados 46 sujeitos sendo 23 com PC-H, grupo 1, e 23 crianças normais, grupo 2. A idade variou entre 07 a 16 anos, idade média de 12 anos e 8 meses. RESULTADOS:O desempenho das FMF e FS demonstrou ser significantemente pior no grupo 1 quando comparado ao grupo controle. PC-H que apresentavam lesões atingindo uma única estrutura encefálica demonstraram melhores resultados que aqueles com comprometimento atingindo duas estruturas maiores que 10 mm. O desempenho dos sujeitos com comprometimento cortical e subcortical, uni ou bilateral, foi inferior quando comparados aos com lesões subcorticais.CONCLUSÃO: As disfunções motoras e sensoriais necessitam ser identificadas e compreendidas para prover melhor treinamento de rotina e cuidados especiais a essas crianças.

Doença cerebrovascular: aquisição de linguagem em pré-escolares / Cerebrovascular disease: language acquisition in preschool children

We describe ten children, aging 5 years and 1 month until 5 years and 11 months, when the phonoaudiological assessment was conducted. They are divided according to cerebrovascular disease, in CVD group (CVD-G) and control group (cG). Children were seen and CVD was confirmed in the acute phase at UNICAMP hospital. Audiologic assessment, protocol for Infant language assessment, and Peabody picture vocabulary test were used in the evaluations. The qualitative analysis of the subjects from a phonoaudiological and neurological point of view has shown the recovery of acquired language disorder (ALD) with no influence whatsoever in the development of 2 subjects and subtle language and/or learning process alterations for 3 subjects. The cases study has revealed that all aspects of language development in preschool children should be analyzed in an individual, quantitative, and qualitative basis to lead to conclusive findings.

São descritos 10 casos de crianças com idade cronológica entre 5 anos e 1 mês e 5 anos e 11 meses, divididas em grupo de doença cerebrovascular, (G-DCV) e grupo controle (Gc). As crianças do G-DCV apresentaram DCV confirmada na fase aguda através de exame neurológico clínico e de imagem na UNICAMP. Nas avaliações utilizou-se triagem audiológica, protocolo de avaliação da linguagem infantil, teste de vocabulário por imagens Peabody, com o objetivo de avaliar os diversos subsistemas lingüísticos e as noções perceptivo-cognitivas. Na análise qualitativa do G-DCV, do ponto de vista fonoaudiológico e neurológico, quando comparado ao Gc, mostrou recuperação completa do distúrbio adquirido de linguagem (DAL) em 2 crianças e alterações de linguagem em 3. O estudo dos casos revelou que os diversos aspectos que constituem a linguagem em desenvolvimento na criança pré-escolar devem ser analisados de forma individual, quantitativa e qualitativamente para achados conclusivos.

Epileptic manifestations induced by midazolam in the neonatal period.

Antiepileptic drugs may cause worsening of epilepsy by aggravating pre-existing seizures or by triggering new seizure types. There are several reports of adverse effects related to midazolam, but only a few authors reported epileptic manifestations. We report four newborns seen at the Neonatal Intensive Care Unit of our University Hospital, who developed seizures a few seconds after the administration of midazolam. It is difficult to identify the patients at risk, but it is important to be aware and recognize this situation.

Hemiparetic cerebral palsy: etiological risk factors and neuroimaging.

The purpose of this paper, which was conducted on 175 children with hemiparetic cerebral palsy (H-CP), was to verify the etiological risk period for this disease. Etiological risk factors (ERF) were detected through anamnesis: 23% in the prenatal period, 18% in the perinatal period and 59% of the patients the period was undefined (ERF in the prenatal and perinatal period was 41% and no ERF was 18% of the cases. The computerized tomographic scan (CT) and MRI were performed on all the patients, who were then classified according to their etiopathogenic data: CT1= normal (18%); CT 2= unilateral ventricular enlargement (25%); CT 3= cortical/ subcortical cavities (28%); CT4= hemispheric atrophy and other findings (14%); CT 5= malformations (15%). CT 5 was associated with physical malformations beyond the central nervous system and with prenatal ERF's, while CT 2 was associated with the perinatal ERF's, mainly in premature births. Magnetic resonance imaging was performed on 57 patients and demonstrated a good degree of concordance with the CT. Etiology remained undefined in only 37% of the cases after neuroimaging was related to ERF. A high perinatal RF frequency (59%) was observed and emphasized the need for special care during this period.

[Medical residence in pediatric neurology in Brazil]. / Residência médica em neurologia infantil no Brasil.

We present the results of a research on Medical Residence in Pediatric Neurology, classifying present information on the teaching and training in the several centers of formation in Brazil. It was possible to contact 17 Institutions with organized services, being 6 accredited by CNRM (National Council of Medical residence), 10 non accredited, and one under diligence. The program content is developed in 3 or 4 years, including the pre-qualification, being the annual schedule load variable, from 1900 to 2880 hours / year.

Fibrodysplasia ossificans progressiva: case report.

Fibrodysplasia ossificans progressiva is a rare genetic disease characterized by widespread soft tissue ossification and congenital stigmata of the extremities. We report on a male child followed for ten years since the age of 3 years and 9 months, when the diagnosis was made. He was born with bilateral hypoplasic hallux valgus and ventricular septal defect, corrected by trans-sternal approach when 32 months old. Restriction of neck mobility followed and foci of ectopic ossification appeared. Four crises of disease exacerbation were treated with oral prednisone and/or other antiinflammatory drugs. Sodium etidronate 5 to 10 mg/kg/day was prescribed intermittently during about six years but was discontinued due to osteopenia. The disease course has been relentless, with severe movement restriction including the chest wall. A review showed few similar case reports in the Brazilian literature. We revisit the criteria for diagnosis and the essentials of management and treatment.

Neurological evaluation of neonates with intraventricular and periventricular hemorrhage.

We studied the clinical aspects of 100 consecutive premature newborns with and without intraventricular and periventricular hemorrhage (IPVH). The diagnosis of IPVH was obtained by ultrasonic scans of the skull during the first week of life and at the age of one month. Forty eight percent of newborns with IPVH had abnormal results, and there was a significant correlation with the neurological evaluation in 85% of the infants. The probability of normality for a child with no associated brain abnormalities was 72%, whereas for a child of the same gestational age with associated brain abnormalities was 48.7%.

[Acute bacterial meningoencephalitis in children. Complications and neurologic sequelae]. / Meningencefalites bacterianas agudas em crianças. Complicações e sequelas neurológicas.

We studied 271 children under age of 15 with diagnosis of acute bacterial meningencephalitis treated at Medical School in Ribeirão Preto, University of São Paulo, between 1980 and 1990. The patients were divided in two groups: 1) those who had not received previous antibiotics treatment (NTP), with 153 cases; and 2), those who had received previous antibiotics treatment (PT), with 118 cases. The etiological agent was more frequently identified in NPT group, while ventriculitis was more frequent in PT group. Mortality rate accounted for 19.5% of all cases, and 29.7% of children under 12 months of age. Acute meningitis caused by Streptococcus pneumoniae was frequently followed by increased mortality. Convulsive disorders and hemiparesis predominante among children under 12 months of age. On the neurosurgical point of view, ventriculitis, subdural hygroma, hydrocephalus, subdural empyema and brain abscess were identified and treated.

Cerebrovascular disease in neonates. Evaluation of four cases.

The clinical and neurological study in four neonates infants with cerebral infarction are reported. The purpose of this study is to call attention for the clinical course, cranial ultrasound, computed tomography and laboratories tests, in order to evaluate the neurological sequelae. A careful evaluation has be taken in order to determine the significance of clinical and laboratory tests for syndromic, topographic and etiologic diagnosis after one year ambulatorial follow-up.

[Video-polygraphic-EEG study in the full-term newborn with low birthweight for their gestational age]. / Estudo vídeo-polissonográfico em recém-nascidos de termo pequenos para a idade gestacional.

Video-polygraphic-EEG studies were performed in the first 24 life-hours of 26 healthy full-term newborns without perinatal injuries. The neurological examination and cranial ultrasonography were normal. The newborns were divided into two groups: one, with full-term appropriate--birth weight 11 newborns (control group) and the other with full-term low-birth weight 15 newborns. Thirteen newborns of the second group had video-polygraphic-EEG study abnormalities. The most frequent abnormalities were found in 11 cases, as far as sleep architecture is concerned. Also, when compared with the control group, 8 cases of an excessive amount of startles and 2 cases of low behavior activities were found. The results demonstrate the usefulness of video-polygraphic-EEG study in the full-term newborns with intra-uterine growth retard. This examination was sensitive to detect behavior, sleep architecture and EEG standard differences in the low birth-weight newborns as to the control group.

[Cerebrovascular disease in children: I. Epileptic manifestations]. / Doença cerebrovascular na infância. I. Manifestações epilépticas.

Seizures may occur as a complication of cerebrovascular disease (CVD) and its prevalence, clinical presentation, risk factors and evolution have been reported by few authors. We evaluated 39 children with CVD and analyzed the association with seizures. Seizures occurred in 24 (61.5%) patients and were classified as partial (29.2%), generalized (54.2%) and secondarily generalized (16.6%). Infants had a significantly higher prevalence of seizures (p = 0.0362) than children at other ages. Cortical localization was associated with a significantly higher prevalence of seizures (p = 0.0101). There were no differences between ischemic and hemorrhagic strokes. Fourteen patients had no seizures after the acute phase of the CVD, the 2 previously epileptic patients had their seizures controlled with antiepileptic drugs, 3 developed epilepsy, 2 died during the acute phase and in 3 patients there was not enough time yet to make a clear diagnosis of epilepsy.

[Cerebrovascular disease in children: II. Clinical aspects in 42 cases]. / Doença cerebrovascular na infância. II. Aspectos clínicos em 42 casos.

We report the findings recorded in 42 children suffering cerebrovascular disease and assisted at the Hospital das Clínicas FCM-UNICAMP, over a 8 years period (January 1990 until April 1998). The ischemic type was the most common, and involvement of the middle cerebral artery, sudden onset of clinical manifestation with seizures and motor disability were more common in early aged children. Motor sequelae predominated in the follow-up of these children.

Cerebrovascular disease in newborn infants. Report of three cases with clinical follow-up and brain SPECT imaging.

The clinical and neurological findings of three neonates with the diagnosis of cerebrovascular disease are reported. The neuropsychological evaluation disclosed impairment of fine motor function, coordination, language, perception and behavioral disturbances. Brain SPECT imaging revealed perfusional deficits in the three cases.

[Comparison of the neuropsychological assessment in a girl with bilateral cerebrovascular disease (moyamoya) before and after surgical intervention]. / Comparação das avaliações neuropsicológicas em menina com doença cerebrovascular bilateral (moyamoya) antes e após a intervenção cirúrgica.

Moyamoya is a chronic progressive cerebrovascular disease with characteristic angiographic findings and a clinical picture with episodes of transient ischemic attacks, headache, seizures, hemiparesis, which may resolve after surgical treatment. We describe the case of a girl with the typical findings of the disease, comparing them before and after surgery with the use of neuropsychological tests, neurological examination and laboratory tests.

Fragile X syndrome. Clinical, electroencephalographic and neuroimaging characteristics.

We studied 11 patients (9 males) with cytogenetic diagnosis of fragile X syndrome (FXS) with the purpose of investigating the neural circuitry involved in this condition. The ages ranged from 8 to 19. All the individuals presented large ears, elongated faces and autistic features. Ten patients had severe mental retardation. Attention disorder was found in 10 individuals. Electroencephalographic recordings were abnormal in 6 of 10 patients examined, showing focal epileptiform discharges predominantly in frontal and parietal areas. All patients underwent magnetic resonance imaging studies which were abnormal in 8 of them. The most important abnormalities were reduction of the cerebellar vermis and enlargement of the IV ventricle. Single photon emission computerized tomography (SPECT) was performed in 7 patients and was abnormal in all of them, the most frequent finding being a hypoperfusion of the inferior of the frontal lobes. Based on the clinical picture, neuropsychological findings and functional and structural imaging studies we suggest that FXS presents with a dysfunction involving a large area of the central nervous system: cerebellum-basal frontal regions-parietal lobes. The literature points to a disturbance involving the same neural circuitry in patients with autism.

Mumps meningoencephalitis. An epidemiological approach.

The aim of this study was to analyse distribution of meningoencephalitis caused by mumps virus in children related to sex, age and seasonal influences. Thirty seven children were evaluated, ages ranging from 2 to 14 years. They were seen at Emergency Unit of Faculdade de Medicina do Triângulo Mineiro and at Hospital da Criança, in Uberaba-MG, Brazil, from March 1st 1991 to February 1st 1993 and they were hospitalized for about 5 days. Through a protocol findings were studied during hospitalization and clinical course stressing epidemiology, symptomatology, cerebrospinal fluid studies, electroencephalogram and cortical function analysis. Only epidemiological data were considered in the present study. Data analysis revealed male predominance, at a range from 5 to 9 years and great number of occurrences at the last quarter of the year.

Polysomnographic quantification of bioelectrical maturation in preterm and fullterm newborns at matched conceptional ages.

We analyzed the relationship between normal neonatal EEG features and gestational age and conceptional age, and evaluated the normal aspects of EEG maturation in preterm babies compared to term babies. We report 46 newborns, divided into two groups. Group I consisted of 11 newborns with gestational age between 30 and 32 weeks, followed with weekly polysomnograms until they reach 42 weeks' conceptional age. Group II (control) consisted of 35 newborns with gestational ages of 34 weeks (n = 5), 36 weeks (n = 10), 38 weeks (n = 10) and 40 weeks (n = 10) evaluated with one polysomnogram in their first 24-48 h of life. In each examination one 5 min epoch in REM and NREM sleep was analyzed to quantify the number of delta brushes, the presence of frontal and temporal sharp transients, the presence of delta frontal rhythmic activity, the grade of concordance between EEG patterns and sleep stages, the percent of interhemispheric synchrony and the duration of interburst interval. The age dependent variability of the EEG patterns was evaluated during the subsequent weeks with group comparisons at weeks 34, 36, 38 and 40. Our results show that the number of delta brushes and the duration of the interburst interval decrease as gestational and conceptional age increase. The percent of interhemispheric synchrony increases with gestational and conceptional age. The presence of frontal sharp transients and delta frontal rhythmic activity suggest that the newborn is fullterm. The presence of temporal sharp transients suggest a preterm newborn. The degree of concordance between behavioral sleep patterns and EEG was more helpful in recognizing sleep stages than in estimating gestational or conceptional age. Although the EEG patterns were comparable between the groups at the same age, analyses of the behavioral patterns of concordance in NREM sleep showed that newborns in Group I had a more immature behaviour than newborns in Group II. Our results also suggest that extrauterine life of preterm babies does not seem to accelerate EEG maturation but may influence the acquisition of behavioral patterns during NREM sleep.

[Hypoxic-ischemic encephalopathy in newborn infants. Acute period and outcome]. / Encefalopatia hipóxico-isquêmica em recém-nascidos a termo. Aspectos da fase aguda e evolução.

Ninety four neonates with hypoxic ischemic encephalopathy HIE attended at the University of Ribeirão Preto since 1982 were studied in terms of the neurological alterations during the acute phase and outcome over a mean period of 47 months. From 43 newborns with HIE I, 40 recovered within 96 hours and 3 died. Among 40 infants with HIE II, 37.5% recovered within the first week, and the others continued abnormal beyond the 7th day. All 11 infants with HIE III died before the second month of life. The HIE I group had no motor sequelae. Among the HIE II group, 34.5% showed cerebral palsy and 17.7% neuromotor retardation. 80.0% of those with sequelae persisted abnormal beyond 7th day of life, during the acute phase of the HIE. Epilepsy occurred in 17.5% of cases with HIE grade II, only among those with neuromotor sequelae. The IQ test did not show statistically significant difference between the HIE I, II without motor sequelae and the control groups. The authors reaffirm the value of the findings in the acute phase of HIE on the outcome of these patients.

[Attention deficit disorder: treatment with methylphenidate]. / Distúrbio do déficit de atenção: tratamento com metilfenidato.

The authors present the results of the treatment with methylphenidate in 24 children with attention deficit disorder based on DSM-III. The prescribed dose was 5 to 10 mg per day, once or twice a day. The results were good in 79.1% of the patients and poor in 16.6%. The drug was withdrawn in one patient because of side effects and the evaluation of efficacy was not possible. Side effects occurred in 2 children (headache and/or nausea). The therapeutic response and the side effects are compared with the literature. The authors claim attention to the efficacy and safety of the treatment with methylphenidate.

Recurrent meningitis in a case of congenital anterior sacral meningocele and agenesis of sacral and coccygeal vertebrae.

A rare case of recurrent meningitis due to congenital anterior sacral meningocele and agenesis of the sacral and coccygeal vertebrae is described. An autosomal dominant inheritance is demonstrated for lower cord malformation, and environmental factors (chromic acid or fumes) are discussed.