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%. Incidence of epilepsy was predominant in patients with hemiplegic and tetraplegic palsies: 70.6% and 66.1%, respectively. First seizure occurred during the first year of life in 74.2% of patients with epilepsy. Generalized and partial were the predominant types of epilepsy (61.3% and 27.4%, respectively). Thirty-three (53.2%) 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. KEY WORDS: epilepsy, cerebral palsy, neonatal seizures. Epilepsia em crianças com paralisia cerebral RESUMO-Objetivo: descrever sobre a prevalência e características da epilepsia em crianças com paralisia cerebral atendidas num serviço terciário. Método: um total de 100 pacientes com paralisia cerebral foi estudado retrospectivamente, tendo como critérios de inclusão o seguimento regular por pelo menos 2 anos. Os tipos e a incidência de epilepsia foram correlacionados com as diferentes formas de paralisia cerebral. Outros fatores associados com a ocorrência de epilepsia como idade da primeira crise, crises neonatais e história familiar de epilepsia também foram analisados. Resultados: o tempo de seguimento variou de 24 a 151 meses (média 57 meses). A prevalência total de epilepsia foi 62%. Os pacientes com as formas hemiplégicas e tetraplégicas de paralisia cerebral tiveram a maior incidência de epilepsia: 70,6% e 66,1%, respectivamente. A primeira crise ocorreu durante o primeiro ano de vida em 74,2% dos pacientes com epilepsia. As epilepsias do tipo generalizada e parcial foram as predominantes (61,3% e 27,4%, respectivamente). Trinta e três (53,2%) de 62 pacientes permaneciam há pelo menos um ano livres das crises. Crises neonatais e história familiar de epilepsia estiveram associadas com maior incidência de epilepsia. Conclusões: epilepsia na paralisia cerebral pode ser prevista se ocorrerem crises no primeiro ano de vida e no período neonatal, e se existe história familiar de epilepsia. Dr. Isak Bruck-CENEP – Rua Floriano Essenfelder 81-80060-270 Curitiba PR-Brasil. Fax: (41) 264 9101 / 362 9380 Cerebral palsy (CP) is a chronic disorder of movement and posture. It is the result of a non-progressive damage of the immature nervous system caused by several...

There is a higher frequency of TMD in pediatric patients with headaches; thus, it is important to look for TMD signs and symptoms in this population.

Sudden unexpected, unexplained death in epilepsy (SUDEP) has been reported to be responsible for 2 to 17% of all deaths in patients with epilepsy. This study was conducted to determine the circumstances of SUDEP and the autopsy findings in these patients. Fifty-three individuals whose cause of death was related to epilepsy were identified and in 30 cases relatives or friends were interviewed about the circumstances of death and other information which allowed to classify the patients as SUDEP or not. The death certificates were also reviewed. We found 20 cases of SUDEP. Most of them were found dead lying on the bed with no evidence of seizure event, and most of them had pulmonary and/or cerebral edema as the cause of death. The incidence and the risk of SUDEP can only be fully ascertained if all sudden deaths had postmortem examination. Consensus in certifying SUDEP cases would allow better accuracy in national mortality rate.KEY WORDS: sudden death, epilepsy, circumstances of death, pulmonary edema.Morte súbita, inexplicada e inesperada em epilepsia: pacientes autopsiados RESUMO Morte súbita inexplicada e inesperada em epilepsia (SUDEP) é responsável por 2 a 17% das mortes em pacientes epilépticos. Este estudo visa determinar as circunstâncias de SUDEP e os achados de autópsia destes pacientes. Foram identificados 53 pacientes cuja causa de morte foi associada a crise epiléptica; em 30 destes, parentes ou amigos foram entrevistados quanto às circunstâncias das mortes e outros aspectos. Também foram revisados os certificados de óbito destes pacientes. Foram encontrados 20 casos de SUDEP. A maioria foi encontrada morta na sua cama por parentes, sem evidência de crise convulsiva, e a causa de morte foi edema cerebral e\ou pulmonar. A incidência e o risco relativo de SUDEP só podem ser adequadamente definidos se houver autópsia em todas as pessoas que morrem subitamente. Um consenso sobre SUDEP permitiria maior acurácia aos dados de mortalidade nacional. PALAVRAS-CHAVE: morte súbita, epilepsia, circunstâncias de morte, edema pulmonar.

The aim of the study was to detect neurological abnormalities in human immunodeficiency virus (HIV) infected children. This was achieved by a prospective evaluation, from November/1995 to April/2000, of 43 HIV infected children (group I) and 40 HIV seroreverters children (group II) through neurological exam and neurodevelopmental tests: Denver Developmental Screening Test (DDST) and Clinical Adaptive Test / Clinical Linguistic and Auditory Milestone Scale (CAT/CLAMS). A control group (III), of 67 children, were evaluated by CAT/CLAMS. Hyperactivity, irritability and hypotonia were the findings on neurological examination, without statistical differences between group I and II. On CAT/CLAMS, the group I developmental quotient (DQ) was significantly lower than the other groups. The same occurred in DDST, with group I presenting significantly more failures than group II. Nineteen HIV children of group I had brain computed tomographic scan, with abnormalities in three of them (basal ganglia calcification, white matter hypodensity and asymmetry of lateral ventricles). We conclude that in HIV infected children a neurodevelopment delay occur early in the disease, and it can be detected by screening tests.

No abstract

We analyzed 31 children with myelomeningocele born between July 1990 and July 2000. Follow-up median was 24 months (6-68months). Only 2 mothers had a known etiologic factor (diabetes mellitus). Twelve had the correct prenatal diagnosis. All children were born at term; 23 by cesarean; 13 had rupture of the membrane. Surgical correction had a 4 days median (1 to 44 days). Lumbosacral lesions were the most frequent (46%). Thirty patients were hydrocephalic, shunt was placed in 27. Meningitis was 4 times more frequent in shunted patients. Seven became epileptic (19.4%). Denver II test showed significant delay in gross motor development . Neurogenic bladder was diagnosed in 12 patients. Congenital clubfoot was the main orthopedic malformation (53%). Six infants died. Nowadays, 17 patients are being followed. A multidisciplinary approach probably helps for a better quality of life.

This study evaluated the degree of neurological compromise in HIV-infected children accompanied by the outpatient clinic of infectious diseases and pediatric neurology of the Clinical Hospital of the Federal University of Paraná (UFPR) starting in 1995. Long-term progressive prospective and cross sectional study of 88 children infected by HIV and 84 seroreverter children, using data from general neurological examinations, neuroimaging procedures (brain CT scan) and neurodevelopmental tests (CAT/CLAMS and DENVER I and II). Neurological and neurodevelopmental alterations were found in 82% of the HIV-infected patients and in 36% of the HIV-seroreverter group (P <0.01). In the CAT/ CLAMS test, the development quotient (DQ) of the HIV-infected group was significantly lower than that of the HIV-seroreverter group. CAT/CLAMS scores lower than 70 (mental deficiency) were found in 31% of the HIV-infected patients during the first year of life and in only 1% of the patients of the HIV-seroreverter group, demonstrating the validity of this screening test for precocious detection of alterations in the neurodevelopment of infected patients. The same occurred with the Denver I and II tests, as the HIV-infected group failed more frequently than the HIV-seroreverter group. Nine HIVinfected children presented altered brain CT scans; calcification of basal ganglia was the main finding (five cases). Encephalopathy due to HIV causes early arrest of neurodevelopment, which can be detected with screening tests during the first year of life.

-Septo-optic dysplasia (SOD) is a syndrome composed by optic nerve and septum pellucidum dysgenesis. It has been classified into two subsets according to the embryogenesis and the neuropathological findings. Basically, the difference between these two groups is the presence or not of schizencephaly. The term SOD-Plus was recently proposed to describe SOD associated with cortical dysplasia. We report a 6-month-old female patient who presented absent visual fixation since 4 months of age and delayed psychomotor development. Neurological examination demonstrated spastic left hemiparesis and ophtalmological evaluation revealed bilateral optic disc hypoplasia. The head computed tomography (CT) scan showed absence of the septum pellucidum, ventricular asymmetry and schizencephaly. The magnetic resonance imaging (MRI) showed complete absence of the septum pellucidum associated to optic nerves and chiasma atrophy, schizencephaly and cortical dysplasia. The patient underwent an evoked potential examination with flash stimulation, which revealed bilateral absence of cortical evoked potential. She was referred to visual stimulation and physiotherapy. We emphasize the neuroimaging of this syndrome and stress the importance of the clinical investigation for patients with septum pellucidum dysgenesis on MRI or CT scans.KEY WORDS: magnetic resonance imaging, computed tomography, septo-optic dysplasia, septum pellucidum. Displasia septo-óptica plus: relato de casoRESUMO -A displasia septo-óptica (DSO) é síndrome composta por disgenesia do nervo óptico e do septo pelúcido, que pode ser dividida em dois subgrupos de acordo com sua embriogênese e achados neuropatológicos. A diferença básica entre estes dois grupos é a presença ou não de esquizencefalia. O termo DSO-plus foi proposto recentemente para descrever DSO associada a displasia cortical. Apresentamos uma paciente de 6 meses de idade com ausência de fixação visual desde os 4 meses e atraso do desenvolvimento psicomotor. O exame neurológico demonstrou hemiparesia espástica esquerda e a avaliação oftalmológica revelou hipoplasia do disco óptico bilateralmente. A tomografia computadorizada (TC) de crânio demonstrou ausência de septo pelúcido, assimetria ventricular e esquizencefalia. A ressonância magnética (RM) revelou ausência completa de septo pelúcido associada a atrofia dos nervos e quiasma ópticos, esquizencefalia e displasia cortical. A paciente foi submetida a exame de potencial evocado com estimulação por flashes que revelou ausência bilateral de potencial evocado cortical. Terapia paliativa foi iniciada com estimulação visual e fisioterapia. Os autores enfatizam os achados de neuro-imagem desta síndrome e a importância da investigação clínica e por métodos de imagem (TC e RM) em pacientes com disgenesia do septo pelúcido. PALAVRAS-CHAVE: ressonância magnética, tomografia computadorizada, displasia septo-óptica, septo pelúcido.