Learning disabilities: analysis of 69 children

With this article we intend to demonstrate the importance of evaluation and follow up of children with learning disabilities, through a multidisciplinary team. As well as to establish the need of intervention. We evaluate 69 children, from Aline Picheth Public School, in Curitiba, attending first or second grade of elementary school, through general and evolutionary neurological examination, pediatric checklist symptoms, and social, linguistic and psychological (WISC-III, Bender Infantile and WPPSI-figures) evaluation. The incidence was higher in boys (84,1 percent), familiar history of learning disabilities was found in 42 percent, and writing abnormalities in 56,5 percent. The most frequent diagnosis was attention deficit and hyperactivity disorder, in 39,1 percent. With this program, we aimed to reduce the retention taxes and stress the importance of this evaluation, and, if necessary, multidisciplinar intervention in the cases of learning disabilities

Síndrome de Rett: estudo retrospectivo e prospectivo de 28 pacientes / Rett syndrome: retrospective and prospective study of 28 patients

No período entre Novembro 1982 e Maio 1999, 28 crianças com Síndrome de Rett foram seguidas por um período médio de 6 anos e 2 meses.O início da regressäo do desenvolvimento psicomotor ocorreu entre 5 e 20 meses.Os 19 casos de síndrome de Rett típica apresentavam períodos pré e perinatal normais,e evoluíram com perda das habilidades previamente adquiridas, retardo psicomotor e estereotipias de mäos; 16 tinham desaceleraçäo do crescimento craniano e 12 tinham marcha anormal. Nove pacientes foram casos atípicos: 2 formas frustras, 2 congênitas, 3 com crises precoces, 1 com fala preservada e 1 sendo do sexo masculino. A epilepsia esteve presente em 21 pacientes com crises predominantemente parciais e a droga de escolha foi a carbamazepina (15 pacientes). Na avaliaçäo inicial a maioria dos pacientes estava distribuída em estágios II e III da síndrome e evolutivamente passaram aos estágios III e IV, sendo que 3 faleceram

Epilepsy in children with cerebral palsy

OBJECTIVE: To describe the prevalence and characteristics of epilepsy in patients with cerebral palsy in a tertiary center. METHODS: a total of 100 consecutive patients with cerebral palsy were retrospectively studied. Criteria for inclusion were follow-up period for at least 2 years. Types and incidence of epilepsy were correlated with the different forms of cerebral palsy. Other factors associated with epilepsy such as age of first seizure, neonatal seizures and family history of epilepsy were also analysed. RESULTS: follow-up ranged between 24 and 151 months (mean 57 months). The overall prevalence of epilepsy was 62 percent. Incidence of epilepsy was predominant in patients with hemiplegic and tetraplegic palsies: 70.6 percent and 66.1 percent, respectively. First seizure occurred during the first year of life in 74.2 percent of patients with epilepsy. Generalized and partial were the predominant types of epilepsy (61.3 percent and 27.4 percent, respectively). Thirty-three (53.2 percent) of 62 patients were seizure free for at least 1 year. Neonatal seizures and family history of epilepsy were associated with a higher incidence of epilepsy. CONCLUSIONS: epilepsy in cerebral palsy can be predicted if seizures occur in the first year of life, in neonatal period and if there is family history of epilepsy

Imunoglobulina endovenosa em crianças com síndrome de Guillain-Barré / Intravenous immunoglobulin in children with Guillain-Barré syndrome

Relatamos nossa experiência com imunoglobulina endovenosa (IGEV), plasmaferese e terapêutica de suporte no tratamento de 13 pacientes com síndrome de Guillain-Barré (SGB). Dos 13 pacientes, 7 receberam IGEV, 2 plasmaferese e 4 terapêutica de suporte. No 15 dia após a administraçäo da IGEV, todos os pacientes deste grupo apresentaram melhora de pelo menos 1 grau na escala de Hughes et al. modificada. Dos 2 pacientes submetidos a plasmaferese, 1 apresentou melhora de 1 grau 5 dias após o procedimento. Entre os 4 pacientes que receberam tratamento de suporte, 2 apresentaram melhora dentro de 20 dias de evoluçäo. No grupo que recebeu IGEV os escores finais foram menores e näo houve recidivas. Assim, estes resultados sugerem que a IGEV diminui o tempo necessário para a melhora clínica quando comparado com tratamento suportivo

Síndrome de West: evoluçao clínica e eletrencefalográfica de 70 pacientes e resposta ao tratamento com hormônio adrenocorticotrófico, prednisona, vigabatrina, nitrazepam e ácido valpróico / West syndrome: clinical and eletroencephalographic follow up of 70 patients and response to the adrenocorticotropic hormone, prednisone, vigabatrin, nitrazepam and valproate

Em estudo retrospectivo avaliamos a evoluçao clínica e eletrencefalográfica das formas criptogênica e sintomática da síndrome de West e analisamos a eficácia do hormônio adrenocorticotrófico, vigabatrina, prednisona, ácido valpróico e nitrazepam no controle dos espasmos. Participaram do estudo 70 pacientes, acompanhados por período maior que 2 anos. Doze (17 por cento) eram criptogênicos e 58 (83 por cento) sintomáticos. O grupo criptogênico apresentou percentagem significativamente maior de pacientes que frequentavam escola regular e desenvolvimento motor normal, melhor controle das crises epilépticas, tendência menor a evoluir para síndrome de Lennox Gastaut e 83,3 por cento tiveram controle completo dos espasmos (72,4 por cento dos pacientes do grupo sintomático obtiveram controle completo dos espasmos). O hormônio adrenocorticotrófico e a vigabatrina foram as drogas mais eficazes, controlando 68,75 por cento e 60 por cento dos espasmos, respectivamente, quando utilizados como droga de primeira escolha e 75 por cento e 50 por cento, respectivamente, como drogas de segunda escolha

Cerebellar vermis hypoplasia - non progressive congenital ataxia: clinical and radiological findings in a pair of siblings

We describe the clinical and radiological findings of a pair of siblings with cerebellar vermis hypoplasia and compare them with the literature. Both of them present pregnancies and deliveries uneventful and both presented some grade of hypotonia, ataxia, ocular motor abnormalities and mild motor delay and slurred speech. These siblings meet many of the criteria described in non-progressive congenital ataxia in which can occur familial cases with cerebellar atrophy, including vermis hypoplasia. As differential diagnosis we compare them with related syndromes and with Joubert's syndrome which main radiological finding on MRI is vermis hypoplasia associated with "molar tooth" appearance. The correct answer for these cases will only be possible by molecular genetics