Journal ArticleDOI
Multilobar polymicrogyria, intractable drop attack seizures, and sleep-related electrical status epilepticus
Renzo Guerrini,P. Genton,Michelle Bureau,Antonia Parmeggiani,X. Salas-Puig,Margherita Santucci,Paolo Bonanni,Giovanni Ambrosetto,C. Dravet +8 more
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TLDR
Age-related secondary bilateral synchrony underlying ESES may be facilitated in multilobar polymicrogyria, and the good seizure outcome contrasts with that usually found in the presence of cortical malformations.Abstract:
Background and Objective: Patients with cortical malformations often have intractable seizures and are candidates for epilepsy surgery. Within an unselected series of patients with various forms of cortical malformation, nine patients with multilobar polymicrogyria had electrical status epilepticus during sleep (ESES) accompanied by infrequent focal motor seizures. Eight patients also had intractable atonic drop attack seizures. Because ESES usually is accompanied by a good long-term seizure prognosis, the objective of this study was to examine ESES outcome among patients with a structural lesion that is usually highly epileptogenic and has a low seizure remission trend. Methods: The nine patients had follow-up periods lasting 4 to 19 years. All underwent brain MRI, serial sleep EEG recordings, and cognitive testing during and after ESES. Results: ESES and drop attack seizures appeared between the ages of 2 and 5 years(mean, 4 years) and ceased between the ages of 5 and 12 years (mean, 8 years). At the last visit patients were 8 to 23 years of age (mean, 14.5 years) and were either seizure free or had very infrequent focal motor seizures during sleep. Three patients were free from antiepileptic drugs. In no patient was definite cognitive deterioration apparent after ESES in comparison with earlier evaluations. Conclusions: Age-related secondary bilateral synchrony underlying ESES may be facilitated in multilobar polymicrogyria. The good seizure outcome contrasts with that usually found in the presence of cortical malformations. For children with polymicrogyria and drop attack seizures, surgical treatment of the epilepsy should be considered cautiously, and sleep EEG recordings should be performed systematically.read more
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Journal ArticleDOI
Terminology and classification of the cortical dysplasias.
André Palmini,Imad Najm,Giuliano Avanzini,Thomas L. Babb,Renzo Guerrini,Nancy Foldvary-Schaefer,Graeme D. Jackson,Hans Lüders,Richard A. Prayson,Roberto Spreafico,Harry V. Vinters +10 more
TL;DR: The panel proposed a specific terminology for the different types of abnormal cells encountered in the cerebral cortex of patients with FCD, a reappraisal of the different histopathologic abnormalities usually subsumed under the term “microdysgenesis,” and a more detailed yet straightforward classification of the various histopathological features that usually are included under the heterogeneous term of “focal cortical dysplasia.”
Book
Epilepsy in Children
TL;DR: Treatment and quality of life have improved because the syndrome-specific efficacy profile of drugs is better known, and there is heightened awareness that compounds with severe cognitive side-effects and heavy polytherapies should be avoided.
Journal ArticleDOI
Malformations of cortical development: clinical features and genetic causes
TL;DR: Because of substantial genotypic and phenotypic heterogeneity for most of these genes, a comprehensive analysis of clinical, imaging, and genetic data is needed to properly define these disorders.
Encephalopathy with electrical status epilepticus during slow sleep
C. A. Tassinari,Guido Rubboli,Lilia Volpi,Stefano Meletti,E. Gardella,Giuseppe d'Orsi,Anna Zaniboni,F. Rondelli,C. Cagnetti,Roberto Michelucci +9 more
TL;DR: Encephalopathy with electrical status epilepticus during sleep or ESES is an age-dependent and self-limited syndrome whose distinctive features include a characteristic age of onset (with a peak around 4-5 years), heterogeneous seizures types (mostly partial motor or unilateral seizures during sleep and absences or falls while awake), a typical EEG pattern (with continuous and diffuse paroxysms occupying at least 85% of slow wave sleep) and a variable neuropsychological regression consisting of IQ decrease, reduction of language (as in acquired aphasia or Landau-Kleff
Journal ArticleDOI
Encephalopathy with electrical status epilepticus during slow sleep or ESES syndrome including the acquired aphasia.
Carlo Alberto Tassinari,Guido Rubboli,Lilia Volpi,Stefano Meletti,Giuseppe d'Orsi,Michele Franca,Annarita Sabetta,Patrizia Riguzzi,Elena Gardella,Anna Zaniboni,Roberto Michelucci +10 more
TL;DR: No specific treatment has been advocated for this syndrome, but valproate sodium, benzodiazepines and ACTH have been shown to control the seizures and the SES pattern in many cases, although often only temporarily.
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Journal ArticleDOI
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