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Both the rheumatic fever and Sydenham's chorea groups were associated with a higher risk of developing neuropsychiatric disorders than the comparison group. ADHD appears to be a risk factor for Sydenham's chorea in children with rheumatic fever.

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Misoprostol, a synthetic analog of prostaglandin, has been widely used in Brazil as an abortifacient. Abortion is illegal in Brazil. An uncertain number of these abortion attempts are unsuccessful and the pregnancy continues. We report on 7 patients whose mothers attempted to abort using this drug in the first trimester of gestation without success. The 7 patients presented with limb defects and in 4 of them a diagnosis of Möbius sequence was made.

Summary:Purpose: This study aimed to evaluate the sensitivity of the EEG in Angelman syndrome (AS), to verify the age at onset of suggestive EEGs and to study EEG patterns, analyzing variations and comparing our findings with nomenclature previously used.Methods: Seventy EEG and 15 V-EEGs of 26 patients were analyzed. Suggestive EEG patterns of AS were classified in delta pattern (DP), theta pattern (TP), and posterior discharges (PDs). Generic terms were used to simplify the analysis.Results: Suggestive EEGs were observed in 25 (96.2%) patients. DP occurred in 22 patients with four variantshypsarrhythmic-like: irregular, high-amplitude, generalized delta activity (DA) with multifocal epileptiform discharges (EDs); slow variant: regular, high-amplitude, generalized DA with rare EDs; ill-defined slow spike-and-wave: regular, high-amplitude, generalized DA with superimposed EDs characterizing a slow wave, with notched appearance; triphasic-like: rhythmic, moderate-amplitude DA over anterior regions with superimposed EDs. TP was observed in eight patients, as generalized or over the posterior regions. PDs were seen in 19 patients as runs of sharp waves or runs of high-amplitude slow waves with superimposed EDs. TP was the only age-related pattern (younger than 8 years) and observed only in patients with deletion. In 15 patients who had an EEG before the clinical diagnosis, 60% had a suggestive tracing.Conclusions: Although some EEG descriptions are not very detailed, and every author describes findings in a slightly different manner, obviously a common denominator must exist. In this context, EEG seems to be a very sensitive method for the diagnosis of AS, offering an opportunity to corroborate this etiologic diagnosis. Conversely, we do not believe that these patterns may be accounted as specific, except for the delta pattern, which seems to be extremely unusual in other syndromes. Other EEG patterns observed in AS, such as theta activity and PDs, occur in a wide variety of disorders. Nonetheless, their importance for the EEG diagnosis of AS is supported by the fact that they are associated with other features and may be helpful in a proper clinical setting. Key Words: Angelman syndrome-EEG-VariantsEpilepsy-Genetic mechanisms.Angelman syndrome (AS) was originally described in 1965 by a British pediatrician named Harry Angelman, who reported three children with peculiar traits and introduced the term puppet children to name them (1). AS did not attract much attention in medical literature until the 1980s, when advances in the field of genetics led to renewed interest in this syndrome. According to the Consensus for Diagnostic Criteria (2), AS demonstrates, in all cases, impairment of neurologic development, poor or no language acquisition, a peculiar behavioral profile (unprovoked laughter, happy demeanor, hand flapping, hyperactivity, and attention-deficit disorder), and a wide-based AS is caused by multiple genetic mechanisms, all of which affect the maternal chromosome 15q11-q13, a known imprinted region. The kn...

RESUMO -O objetivo deste estudo é analisar dados clínicos e laboratoriais de 80 casos de esquistossomose medular (EM) que possam contribuir para melhorar o diagnóstico e tratamento dessa doença. Em 59 pacientes o estudo foi de maneira prospectiva, em 79 pacientes o diagnóstico foi catalogado como altamente provável, presuntivo, baseado em critérios clínico e laboratoriais. Em um paciente houve confirmação anatomo-patológica. Houve predominância do sexo masculino (63,7%), faixa etária entre 21 e 40 anos (63,7%), procedência nordestina (85%), trabalhador em construção civil (31,2%), esforço abdominal prévio (57,5%), início subagudo (61,2%), forma clínica mielorradiculítica e lesão no cone medular e cauda equina (72,5%). O estudo do líquido cefaloraquidiano mostrou pleocitose e taxas elevadas de proteínas em 100% dos casos, da gamaglobulina em 76.5%, positividade de reações imunológicas específicas (imunofluorescência e/ou ELISA) em 100% dos casos (titulo médio de 1/16 e 61u/dl, respectivamente). Costicosteróides e drogas esquistossomicidas foram administrados em todos os pacientes com evolução satisfatória em 80% dos casos. ABSTRACT -To outline through cinical-laboratorial analysis a profile of schistosomiasis of the spinal cord (SSC) that contributes to the diagnosis and treatment of this disease. 80 patients were studied (59 prospectively), and epidemiological, clinical,laboratorial, treatment and outcome data extracted. In 79 patients the diagnosis was presumptive and obeyed rigorous criteria. There was a predominance of male sex (68.7%), age group from 21 to 40 years (63.7%), Northeasterners (85%), building construction workers (31.2%), previous abdominal effort (57.5%), subacute beginning (61.2%), myeloradiculitis form and lesion in conus and cauda equina (72.5%). Cerebral spinal fluid showed lymphomononuclear pleocytosis and protein increase in 100% of the cases as well as gamma globulin in 76.5%, positiveness of immunofluorescence reaction and/or ELISA for schistosomiasis in 100% of the cases with average titles of 1/16 and 61 u/dl, respectively. Corticosteroids and antischistosomal drugs were given to all patients with a satisfactory outcome in 80% of the cases. We emphasize the importance of a precocious treatment to avoid irreversible deficits such as paraplegia or sexual impotence. PALAVRAS-CHAVE

The association between Sydenham's chorea and OCD supports suggestions that similar mechanisms involving the basal ganglia underlie both disorders. Obsessive-compulsive symptoms occurred at the beginning of rheumatic fever, so early psychopathological assessments are essential.