Epilepsy and stigma: An approach to understanding through the life and works of the Brazilian writer Machado de Assis (1839-1908)

Machado de Assis (1839-1908)-novelist, short story writer, essayist, and poet-is a fascinating personality. Had he written in French, English, German, or Italian, he would have achieved universal fame and would be in the same company as Balzac, Tolstoy, Dickens, and Dostoevsky. This article discusses stigma in epilepsy through Machado de Assis' life, literary work, and letters to other Brazilian writers. Founder of the Brazilian Academy of Letters, Machado offers an insoluble enigma to psychologists and essayists. Born in stark poverty, feeble, and ugly, he had to fight the taint of epilepsy. the documentation of epilepsy in Machado de Assis' texts and letters and the testimony of his contemporaries is unique, allowing the comprehension of scientific concepts and stigma related to epilepsy in the 19th century, when the positivist ideas of the Italian neuropsychiatrist Cesare Lombroso (1835-1909) permeated nascent Brazilian neuropsychiatry. Much of the stigma associated with epilepsy we witness today emerged from these concepts. Even today in Brazil, when barbaric crimes are committed, headlines in newspapers produced by forensic psychiatrists often attribute the conduct of the criminal to epileptic behavior. (C) 2011 Elsevier Inc. All rights reserved.Universidade Federal de São Paulo UNIFESP, Dept Neurol & Neurosurg, São Paulo, BrazilUniversidade Federal de São Paulo UNIFESP, Dept Neurol & Neurosurg, São Paulo, BrazilWeb of Scienc

Juvenile myoclonic epilepsy: A system disorder of the brain

The prevailing understanding of generalized epilepsy is shaped by the traditional definition that the responsible neuronal discharge takes place, if not throughout the entire grey matter, then at least in the greater part of it and simultaneously on both sides. This view is no longer tenable since concurrent findings using multiple methods have accumulated to reveal the role of bilateral networks of distributed and selective cortical and subcortical structures in so-called generalized ictogenesis. Most of this research has been focused on juvenile myoclonic epilepsy (JME), which today is commonly considered the archetypical syndrome of the idiopathic generalized epilepsies. Based upon recent research in the fields of clinical epileptology, neuropsychology and psychiatry, clinical neurophysiology, neuroimaging and epilepsy genetics this article, for the first time, unites these new findings into a comprehensive nosological view. Genetically determined dysfunctions of important cognitive systems like visuomotor coordination and linguistic communication appear now as key mechanisms of seizure generation in JME. This review suggests a new paradigm to consider JME as a system disorder of the brain analogous to other neurological system disorders. (C) 2015 Elsevier B.V. All rights reserved

Miopatia nemalinica: relato de um caso com estudo histoquimico e microscopia eletrônica

Relato do caso de criança do sexo masculino, com hipotonia e dificuldades de deglutição desde os primeiros dias de vida, necessitando gastrostomia permanente. Apresentava retardo no desenvolvimento motor. A eletromiografia sugeria envolvimento muscular primário e a biópsia muscular, processada a fresco, histoquímica e pela microscopia eletrônica, revelou corpos nemalínicos clássicos e deficiência de fibras do tipo II. São feitos comentários a respeito dos sintomas e formas clínicas, origem e patogenia dos corpos nemalínicos, sendo o caso relatado caracterizado como miopatia nemalínica do tipo congênito

Surgical treatment of refractory epilepsy associated with space occupying lesions experience and review Tratamento cirúrgico da epilepsia refratária associada a lesões expansivas: experiência e revisão

Surgery for space occupying lesions of the brain associated with intractable epilepsy represents a special problem because relief of the epilepsy is as much an operative goal as excision of the space occupying lesion itself. This study concerns 32 patients with space occupying lesions and intractable epilepsy who underwent excision of the lesion with acute intraoperative electrocorticography guided resection of the epileptogenic focus. Of the 32 patients, 16 formed a subgroup of gangliogliomas alone. The remaining were mixed lesions, predominantly benign. The duration of seizures in these patients ranged from 2 to 30 years, and the seizure frequency varied from 1 to 300 convulsions per month. The operative procedures included temporal corticectomy, amygdalo-hippocampectomy, and extratemporal corticectomies. Twenty nine patients were in Engel class I postoperatively, and three patients were in Engel class II. The findings with gangliogliomas are also considered in a separate group. This study strongly suggests that the operative procedure under electrocorticography guidance improves seizure outcome in space occuping lesions related intractable epilepsy.O tratamento cirúrgico das lesões que ocupam espaço (LOE) do sistema nervoso associadas a epilepsia intratável representa um problema clínico especial, já que tanto o tratamento da lesão como o da epilepsia são relevantes. Estudaram-se 32 pacientes com LOEs que foram submetidos a lesionectomia com margens guiadas por eletrocorticografia. Destes, 16 possuíam gangliogliomas e os restantes lesões variadas, predominantemente benignas. A duração das crises nesses pacientes variou de 2 a 30 anos e a frequência das crises de 1 a 300 por mês. Os procedimentos cirúrgicos incluíram corticectomia temporal, amigdalo-hipocampectomia e corticectomias extra-temorais. Vinte e nove pacientes estvam em grau I de Engel pós-operatoriamente e 3 em grau II. Os achados em relação aos gangliogliomas foram também estudados separadamente. Este estudo sugere que a inclusão da eletrocorticografia e da ressecção das margens epileptogênicas em pacientes com LOEs e epilepsia refratária melhoram os resultados obtidos em relação às crises

Are psychiatric disorders exclusion criteria for video-EEG monitoring and epilepsy surgery in patients with mesial temporal sclerosis?

Epilepsy surgery (ES) is a treatment option for patients with refractory temporal lobe epilepsy (TLE). However, psychiatric disorders (PDs) have been a contraindication for presurgical evaluation in many epilepsy centers. the aim of this study was to evaluate the safety of video-EEG (VEEG) and surgical outcome in patients with refractory TLE and mesial temporal sclerosis (TLE-MTS) associated with PDs. We retrospectively analyzed the clinical, sociodemographic, and VEEG data and surgery outcome of patients with refractory TLE-MTS who underwent ES over the period of 2002 to 2011 and compared data between those with and without PDs. Psychiatric evaluation was performed through DSM-IV and ILAE criteria. Safety during presurgical evaluation was analyzed by the rate of adverse events (AEs). Patients' quality of life (QOL) was measured through ESI-55 and the surgical outcome through Engel's classification. Data from 145 patients were included. the mean VEEG length (93 h) was not affected by PDs. Among patients with PDs, 4.91% (3/61) had AEs, and 13.11% (8/61) had psychogenic nonepileptic seizures (PNESs). Among patients without PDs, 4.76% (4/84) had AEs, and 5.95% (5/84) had PNESs. in the first two follow-up years, of the 94 patients who underwent ES, 65.85% (27/41) with PDs and 67.92% (36/53) without PDs became free of disabling seizures (Engel I). No significant differences were observed in the patients' QOL between both groups after surgery. the rate of AEs and seizure outcome did not differ significantly between both groups, reinforcing the idea that PDs should not be absolute exclusion criteria for VEEG monitoring and epilepsy surgery among patients with TLE-MTS. (C) 2013 Elsevier Inc. All rights reserved.Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES)Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP)Universidade Federal de São Paulo UNIFESP, Dept Neurol & Neurosurg, São Paulo, BrazilUniversidade Federal de São Paulo UNIFESP, Dept Psychiat, Lab Interdisciplinar Neurociencias Clin LiNC, São Paulo, BrazilUniversidade Federal de São Paulo UNIFESP, Dept Neurol & Neurosurg, São Paulo, BrazilUniversidade Federal de São Paulo UNIFESP, Dept Psychiat, Lab Interdisciplinar Neurociencias Clin LiNC, São Paulo, BrazilWeb of Scienc

Cognitive performance in juvenile myoclonic epilepsy patients with specific endophenotypes

Purpose: Juvenile myoclonic epilepsy (JME) is a heterogeneous syndrome in which seizures can be precipitated not only by non-specific factors, such as sleep deprivation and stress, but also by specific stimuli, such as photic stimuli, eye-closure, praxis, and language. The presence of these reflex traits may indicate the hyperexcitability of different cortical areas, which may be reflected in patients' neuropsychological deficit profile. The objective of our study is to investigate the possible relations between JME endophenotypes and patients' cognitive performance. Methods: 61 JME patients were divided into four groups: no reflex traits (group 1, 20 patients)praxis induction (group 2, 13)eye-closure and/or photosensitivity (group 3, 17)and a combination of different reflex traits (group 4, 11). Neuropsychological performance was compared between JME subgroups. 60 healthy controls were used to calculate z-scores. Patients also underwent psychiatric assessment. We controlled the clinical variables, e.g. age at epilepsy onset, frequency of myoclonic seizures, total and sedative drug load, setting them as covariables for the ANOVA analysis. Results: Praxis induction was more common in males (p = 0.018) and groups with reflex traits (2, 3, and 4) presented higher rates of persistent myoclonia, polytherapy, clonazepam use (group 3), and more frequent psychiatric comorbidities. Group 4 patients performed worse in Trail Making Test B than the patients in group 1. These findings were independent of clinical variables. Conclusion: JME patients with a combination of praxis induction and eye-closure/photosensitivity had greater executive dysfunction, revealing an association between reflex ictogenic mechanisms and cognitive performance. (C) 2016 British Epilepsy Association. Published by Elsevier Ltd. All rights reserved.Univ Fed Sao Paulo UNIFESP, Dept Neurol & Neurosurg, Unidade Pesquisa & Tratamento Epilepsias, Sao Paulo, BrazilDanish Epilepsy Ctr, Dianalund, DenmarkUniv Fed Santa Catarina, Neurol Serv, Dept Clin Med, Florianopolis, SC, BrazilUniv Fed Sao Paulo UNIFESP, Dept Neurol & Neurosurg, Unidade Pesquisa & Tratamento Epilepsias, Sao Paulo, BrazilWeb of Scienc