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The epileptologically informed in-depth analysis of auto- and heteroanamnestic historical sources supports the hypothesis that Hector Berlioz (1803-1869) might have suffered from a photosensitive idiopathic generalized epilepsy syndrome with absences, absence status, myoclonic seizures, and bilateral tonic-clonic seizures. The study outlines the possible influence of the composer's probable epilepsy on his literary and musical oeuvre, including the Symphonie fantastique en cinq parties.

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INTRODUCTION: Dom Pedro II, the Prince Heir and Emperor under regency, in a delicate period of the construction of the Brazilian nation, had convulsive seizures. OBJECTIVE: To investigate the convulsive seizures and related syndromes of Dom Pedro II and his family, besides the physicians in charge of the health care. METHOD: Narrative review based on primary and secondary sources. CONCLUSION: The scattered and self-limited convulsive seizures associated with physical and mental integrity favored a benign prognosis. Dom Pedro and his family presented rich history of epileptic seizures and febrile convulsion. This variety resembles the diagnosis of generalized epilepsy with febrile seizures plus that seems to be a combination of several syndromes with shared genetic susceptibility.

INTRODUÇÃO: Dom Pedro, o príncipe herdeiro e imperador sob regência, em período delicado da formação da nação brasileira, apresentou crises convulsivas que geraram preocupação para o país. OBJETIVO: Investigar a história da epilepsia de Dom Pedro II e da sua família e procurar identificar quais tipos de crises epilépticas estavam presentes, além dos médicos envolvidos com os cuidados de saúde. MÉTODO: Revisão narrativa baseada em fontes primárias e secundárias. CONCLUSÃO: As crises convulsivas esparsas e auto-limitadas associadas a higidez física e mental de Dom Pedro II sugerem um prognóstico benigno. A história de epilepsia idiopática e convulsões febris no imperador e em outros membros da sua família aponta para o diagnóstico mais provável de Epilepsia Generalizada com Convulsões Febris Plus que é determinada por uma combinação de alguns tipos de manifestações epilépticas com suscetibilidade genética compartilhada.

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INTRODUCTION: Dom Pedro II, the Prince Heir and Emperor under regency, in a delicate period of the construction of the Brazilian nation, had convulsive seizures. OBJECTIVE: To investigate the convulsive seizures and related syndromes of Dom Pedro II and his family, besides the physicians in charge of the health care. METHOD: Narrative review based on primary and secondary sources. CONCLUSION: The scattered and self-limited convulsive seizures associated with physical and mental integrity favored a benign prognosis. Dom Pedro and his family presented rich history of epileptic seizures and febrile convulsion. This variety resembles the diagnosis of generalized epilepsy with febrile seizures plus that seems to be a combination of several syndromes with shared genetic susceptibility.

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PURPOSE: To describe Victor Horsley's contribution to John Hughlings Jackson's understanding of the mechanisms involved in the generalization of convulsive epileptic seizures. METHODS: I reviewed Horsley's writings and other relevant late 19th century medical literature. RESULTS: Horsley's combination of strategically sited surgical lesions and cerebral cortex stimulation studies in experimental animals showed that, contrary to Hughlings Jackson's earlier belief, epileptic activity arising in one cerebral hemisphere had to spread to the contralateral hemisphere before bilateral convulsing could occur. CONCLUSIONS: On the basis of well-designed experiments, Horsley made a major contribution to the understanding of epileptic seizure propagation mechanisms.

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Early in these proceedings, the origin of the three terms in the title, "idiopathic generalized epilepsy," is discussed with respect to their significance over time, and typical misunderstandings. In the mid-20th century, a rather chaotic use of a multitude of often loosely defined terms had developed, which increasingly became an obstacle to a meaningful international discussion. The International League against Epilepsy (ILAE) took the initiative to develop an internationally accepted terminology with a classification system consisting of a classification of seizures (1981) and a classification of syndromes (1989). The Idiopathic Generalized Epilepsies are one of its four major groups emerging from a double dichotomy of generalized versus localization-related and idiopathic versus symptomatic. The inclusion of biologic aspects such as syndrome-specific ages of onset ("age-related syndromes") or syndrome-specific relations of seizure occurrence to the sleep-wake cycle ("Epilepsy with Grand Mal on Awaking") meant that the syndrome classification merged the more biological views of the German school with the more neurophysiological ones of the French. Apart from establishing a common international language concerning epilepsy, the International Classification of Epilepsies and Epileptic Syndromes became an important stimulator of research, especially concerning the idiopathic epilepsies. In particular, genetic and functional imaging investigations aim at a better understanding of these conditions. It is now understood that most idiopathic syndromes have a--sometimes complex--genetic background, but we are also becoming aware of the inappropriateness of the time-honored term "generalized" and part of our dichotomies. Both localization-related and "generalized" idiopathic epilepsies seem to share a principle of ictogenesis based on functional anatomic pathogenic networks, and we seem to move toward understanding them as functional system disorders of the brain.

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Bilateral medial temporal lobe resection in man results in a persistent impairment of recent memory whenever the removal is carried far enough posteriorly to damage portions of the anterior hippocampus and hippocampal gyrus. This conclusion is based on formal psychological testing of nine cases (eight psychotic and one epileptic) carried out from one and one-half to four years after operation. The degree of memory loss appears to depend on the extent of hippocampal removal. In two cases in which bilateral resection was carried to a distance of 8 cm posterior to the temporal tips the loss was particularly severe. Removal of only the uncus and amygdala bilaterally does not appear to cause memory impairment. A case of unilateral inferior temporal lobectomy with radical posterior extension to include the major portion of the hippocampus and hippocampal gyrus showed no lasting memory loss. This is consistent with Milner and Penfield's negative findings in a long series of unilateral removals for temporal lobe epilepsy. The memory loss in these cases of medial temporal lobe excision involved both anterograde and some retrograde amnesia, but left early memories and technical skills intact. There was no deterioration in personality or general intelligence, and no complex perceptual disturbance such as is seen after a more complete bilateral temporal lobectomy. It is concluded that the anterior hippocampus and hippocampal gyrus, either separately or together, are critically concerned in the retention of current experience. It is not known whether the amygdala plays any part in this mechanismi, since the hippocampal complex has not been removed alone, but always together with uncus and amygdala.

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