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-Status Dystonicus (SD) is characterized by generalized muscle contractions in dystonic patients. We report 5 cases of SD, two of which in patients with dystonic cerebral palsy, one in a patient with primary segmental dystonia, one in a patient with Hallervorden-Spatz syndrome and one in a patient with Wilson's disease (WD). Three patients were admitted to an intensive care unit and treated with propofol and midazolam, and two were submitted to neurosurgical procedures (bilateral pallidotomy and bilateral pallidal deep brain stimulation). Triggering factors were identified in three patients as follows: infection, stress-induced and zinc therapy for WD. On follow-up, two patients presented with significant improvement of dystonia, whereas the other three cases the clinical picture ultimately returned to baseline pre-SD condition.

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.

No abstract

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.

Conflict of interest: noneObjective: to determine the incidence and associations of attention deficit-hyperactivity disorder (ADHD), conduct disorder (CD), and substance abuse disorder (SAD) in adolescents in conflict with the law in a Brazilian cohort. Methods: the Brazilian version of the Schedule for Affective Disorders and Schizophrenia for School Aged-Children (K-SADS-PL) was administered to 69 adolescent boys who were incarcerated for 45 days in the city of Curitiba, Brazil. Results: mean age was 15.5 years (range, 12-16.9 years) and most adolescents originated from disadvantaged social classes (87%). They resided in neighborhoods on the outskirts of the city or towns in the greater metropolitan area. Truancy and low educational achievement were common, with 73.9% not currently attending school and 43.4% not having finished the 5 th grade. The great majority lived in single-parent families and many had relatives who themselves had problems with the law. Psychiatric disorders were apparent in 81.1% of the subjects, with the most common disorders being CD (59.4%), SAD (53.6%), and ADHD (43.5%). Both ADHD (p <0.001) and CD (p <0.01) had significant associations with substance abuse. Conclusion: in male adolescents in conflict with the law, ADHD, CD, and SAD were all found to be associated with delinquency.