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Dive into the research topics where Edouard Hirsch is active.

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Featured researches published by Edouard Hirsch.


Epilepsia | 2017

ILAE classification of the epilepsies: Position paper of the ILAE Commission for Classification and Terminology

Ingrid E. Scheffer; Samuel F. Berkovic; Giuseppe Capovilla; Mary B. Connolly; Jacqueline A. French; Laura Maria de Figueiredo Ferreira Guilhoto; Edouard Hirsch; Satish Jain; Gary W. Mathern; Solomon L. Moshé; Douglas R. Nordli; Emilio Perucca; Torbjoern Tomson; Samuel Wiebe; Yuehua Zhang; Sameer M. Zuberi

The International League Against Epilepsy (ILAE) Classification of the Epilepsies has been updated to reflect our gain in understanding of the epilepsies and their underlying mechanisms following the major scientific advances that have taken place since the last ratified classification in 1989. As a critical tool for the practicing clinician, epilepsy classification must be relevant and dynamic to changes in thinking, yet robust and translatable to all areas of the globe. Its primary purpose is for diagnosis of patients, but it is also critical for epilepsy research, development of antiepileptic therapies, and communication around the world. The new classification originates from a draft document submitted for public comments in 2013, which was revised to incorporate extensive feedback from the international epilepsy community over several rounds of consultation. It presents three levels, starting with seizure type, where it assumes that the patient is having epileptic seizures as defined by the new 2017 ILAE Seizure Classification. After diagnosis of the seizure type, the next step is diagnosis of epilepsy type, including focal epilepsy, generalized epilepsy, combined generalized, and focal epilepsy, and also an unknown epilepsy group. The third level is that of epilepsy syndrome, where a specific syndromic diagnosis can be made. The new classification incorporates etiology along each stage, emphasizing the need to consider etiology at each step of diagnosis, as it often carries significant treatment implications. Etiology is broken into six subgroups, selected because of their potential therapeutic consequences. New terminology is introduced such as developmental and epileptic encephalopathy. The term benign is replaced by the terms self‐limited and pharmacoresponsive, to be used where appropriate. It is hoped that this new framework will assist in improving epilepsy care and research in the 21st century.


Nature Genetics | 2013

GRIN2A mutations in acquired epileptic aphasia and related childhood focal epilepsies and encephalopathies with speech and language dysfunction

Gaetan Lesca; Gabrielle Rudolf; Nadine Bruneau; Natalia Lozovaya; Audrey Labalme; Nadia Boutry-Kryza; Manal Salmi; Timur Tsintsadze; Laura Addis; Jacques Motte; Sukhvir Wright; Vera Tsintsadze; Anne Michel; Diane Doummar; Karine Lascelles; Lisa J. Strug; Patrick Waters; Julitta de Bellescize; Pascal Vrielynck; Anne de Saint Martin; Dorothée Ville; Philippe Ryvlin; Alexis Arzimanoglou; Edouard Hirsch; Angela Vincent; Deb K. Pal; Nail Burnashev; Damien Sanlaville; Pierre Szepetowski

Epileptic encephalopathies are severe brain disorders with the epileptic component contributing to the worsening of cognitive and behavioral manifestations. Acquired epileptic aphasia (Landau-Kleffner syndrome, LKS) and continuous spike and waves during slow-wave sleep syndrome (CSWSS) represent rare and closely related childhood focal epileptic encephalopathies of unknown etiology. They show electroclinical overlap with rolandic epilepsy (the most frequent childhood focal epilepsy) and can be viewed as different clinical expressions of a single pathological entity situated at the crossroads of epileptic, speech, language, cognitive and behavioral disorders. Here we demonstrate that about 20% of cases of LKS, CSWSS and electroclinically atypical rolandic epilepsy often associated with speech impairment can have a genetic origin sustained by de novo or inherited mutations in the GRIN2A gene (encoding the N-methyl-D-aspartate (NMDA) glutamate receptor α2 subunit, GluN2A). The identification of GRIN2A as a major gene for these epileptic encephalopathies provides crucial insights into the underlying pathophysiology.


Epilepsia | 2017

Operational classification of seizure types by the International League Against Epilepsy: Position Paper of the ILAE Commission for Classification and Terminology

Robert S. Fisher; J. Helen Cross; Jacqueline A. French; Norimichi Higurashi; Edouard Hirsch; Floor E. Jansen; Lieven Lagae; Solomon L. Moshé; Jukka Peltola; Eliane Roulet Perez; Ingrid E. Scheffer; Sameer M. Zuberi

The International League Against Epilepsy (ILAE) presents a revised operational classification of seizure types. The purpose of such a revision is to recognize that some seizure types can have either a focal or generalized onset, to allow classification when the onset is unobserved, to include some missing seizure types, and to adopt more transparent names. Because current knowledge is insufficient to form a scientifically based classification, the 2017 Classification is operational (practical) and based on the 1981 Classification, extended in 2010. Changes include the following: (1) “partial” becomes “focal”; (2) awareness is used as a classifier of focal seizures; (3) the terms dyscognitive, simple partial, complex partial, psychic, and secondarily generalized are eliminated; (4) new focal seizure types include automatisms, behavior arrest, hyperkinetic, autonomic, cognitive, and emotional; (5) atonic, clonic, epileptic spasms, myoclonic, and tonic seizures can be of either focal or generalized onset; (6) focal to bilateral tonic–clonic seizure replaces secondarily generalized seizure; (7) new generalized seizure types are absence with eyelid myoclonia, myoclonic absence, myoclonic–atonic, myoclonic–tonic–clonic; and (8) seizures of unknown onset may have features that can still be classified. The new classification does not represent a fundamental change, but allows greater flexibility and transparency in naming seizure types.


Epilepsia | 1990

Landau‐Kleffner Syndrome: A Clinical and EEG Study of Five Cases

Edouard Hirsch; Christian Marescaux; Pierre Maquet; N. Metz-Lutz; M. Kiesmann; Eric Salmon; Georges Franck; D. Kurtz

Summary: : In five children with normal initial psychomotor development, a Landau‐Kleffner syndrome appeared at age 3–7 years. No neuroanatomy; lesions were noted. Aphasia and hyperkinesia were isolated in three patients and associated with global regression of higher cortical functions in one patient. Massive intellectual deterioration and psychotic behavior were associated with transient aphasia in one patient. The epilepsy (focal motor and generalized tonic‐clonic seizures, subclinical EEG focal seizures during sleep, and atypical absences) always regressed spontaneously or with antiepileptic drug (AED) treatment. The EEG in waking patients snowed focal and generalized spike‐wave discharges on a normal background rhythm. Discharge topography and pattern changed frequently. During sleep, discharges always increased. At some time during syndrome development, all patients had bilateral spike‐waves for 7gt;85% of the sleep period, while at other times the discharges were discontinuous or continuous but focal or unilaterally hemispheric. Discharge topography and abundance changed from night to night. The abnormal EEG and the impaired higher functions developed and regressed together, but not with strict temporal correlation. Our own experience suggests that the Landau‐Kleffner syndrome and epilepsy with continuous spike‐wave activity in slow‐wave sleep cannot be clearly differentiated. They may be different points on the spectrum of a single syndrome.


Epilepsia | 1990

Landau-Kleffner Syndrome: A Pharmacologic Study of Five Cases

Christian Marescaux; Edouard Hirsch; S. Finck; Pierre Maquet; E. Schlumberger; F. Sellal; M. N. Metz-Lutz; Y. Alembik; E. Salmon; G. Franck; D. Kurtz

Summary: : Five children with Landau‐Kleffner syndrome (epilepsy, acquired aphasia, and continuous spike‐wave discharges during sleep), were treated with antiepileptic drugs (AEDs), sleep‐modifying drugs, and corticosteroids. The pharmacologic profiles differed from those observed in focal epilepsies, resembling instead those of certain generalized epilepsies, such as West or Lennox‐Gastaut syndromes. Phenobarbital (PB), carbamazepine (CBZ), and phenytoin (PHT) were ineffective or worsened the EEG and neuropsychological symptoms, whereas valproate (VPA), ethosuximide (ESM), and benzodiazepines were partially or transiently efficacious. Dextroamphetamine produced a dramatic but transient improvement in waking and sleep EEG in one of two children; aphasia did not change. Corticosteroid treatment resulted in improved speech, suppression of seizures, and normalization of the EEG in three of three children. Our own experience and data from the literature suggest that corticosteroids should be given in high doses as soon as the diagnosis is firmly established and should be continued in maintenance dose for several months or years to avoid escape. Early diagnosis, before mutism or global deterioration develops, appears to be essential for effective therapy with minimal neuropsychological sequelae.


Epilepsia | 2006

Nocturnal Hypermotor Seizures, Suggesting Frontal Lobe Epilepsy, Can Originate in the Insula

Philippe Ryvlin; Lorella Minotti; Geneviève Demarquay; Edouard Hirsch; Alexis Arzimanoglou; Dominique Hoffman; Marc Guénot; Fabienne Picard; Sylvain Rheims; Philippe Kahane

Summary:  Purpose: To report three patients with drug‐resistant nocturnal hypermotor seizures (NHSs), no detectable brain lesion, and clinically defined nocturnal frontal lobe epilepsy (NFLE) or autosomal dominant NLFE (ADNFLE), whose intracerebral EEG ictal onset primarily involved the insula, rather than the mesial or orbital frontal cortex.


Epilepsy Research | 1995

Effects of levetiracetam, a novel antiepileptic drug, on convulsant activity in two genetic rat models of epilepsy

A.T. Gower; Edouard Hirsch; Any Boehrer; M. Noyer; Christian Marescaux

The anticonvulsant effects of levetiracetam were assessed in two genetic rat models. In the audiogenic-seizure prone rat, levetiracetam, 5.4 to 96 mg/kg i.p. dose-dependently inhibited both wild running and tonic-clonic convulsions. In the GAERS model of petit mal epilepsy, levetiracetam markedly suppressed spontaneous spike-and-wave discharge (SWD) but left the underlying EEG trace normal. The effects were already marked at 5.4 mg/kg and did not increase significantly up to 170 mg/kg although more animals were completely protected. Levetiracetam produced no observable effects on behaviour apart from slight reversible sedation at 170 mg/kg. In contrast, piracetam, a structural analogue of levetiracetam, significantly and consistently suppressed SWD in GAERS rats only at the high dose of 1000 mg/kg with some slight effects at lower doses. The effect of piracetam appeared to be due to increased sleeping rather than to a direct antiepileptic effect. The results with levetiracetam argue for a clinical application in both petit mal, absence epilepsy and in treating generalised tonic-clonic and partial seizures.


Neurosurgery | 2002

Antiepileptic effect of high-frequency stimulation of the subthalamic nucleus (corpus luysi) in a case of medically intractable epilepsy caused by focal dysplasia: a 30-month follow-up: technical case report.

Alim-Louis Benabid; Lorella Minotti; Adnan Koudsie; Anne de Saint Martin; Edouard Hirsch; Patrick J. Kelly; Nicholas M. Barbaro; Philip A. Starr; Imad Najm; Ali R. Rezai; Andres M. Lozano

OBJECTIVE AND IMPORTANCE Currently, some forms of epilepsy are resistant to both pharmacological and surgical interventions. As a result, there is a need for new therapeutic strategies. Because the nigral system modulates neuronal excitability in animal models of epilepsy, we considered therapeutic high-frequency stimulation of the subthalamic nucleus (STN). We were encouraged by the known relationship between the STN and the nigral system, as well as by our experience with high-frequency stimulation of the STN in Parkinsonian patients. CLINICAL PRESENTATION A 5-year-old girl with pharmacologically resistant, inoperable epilepsy caused by focal centroparietal dysplasia underwent implantation with a permanent electrode in the left STN and was chronically stimulated. To date, we have followed up this patient for 30 months postoperatively. TECHNIQUE High-frequency stimulation of the STN induced a significant voltage-dependent reduction (by 80%) in the number and severity of seizures. In addition, consistent improvement in both motor and cognitive functions was noted as a result of reduced postictal states. The effect was more prominent for seizures occurring in clusters (89% reduction) and during the day (88% reduction) than for those that occurred during sleep (53% reduction). CONCLUSION This is the first report of epilepsy control using chronic high-frequency stimulation of the STN. Preliminary observations in three other operated patients (at 2, 12, and 18 mo) confirm these data. We think that high-frequency stimulation of the STN may hold significant future potential as a treatment for epilepsy, similar to its established role in the treatment of Parkinson’s disease. This finding opens completely new experimental and therapeutic avenues for the treatment of surgically and medically intractable epilepsy.


Nature Genetics | 2013

Mutations of DEPDC5 cause autosomal dominant focal epilepsies

Saeko Ishida; Fabienne Picard; Gabrielle Rudolf; Eric Noé; Guillaume Achaz; Pierre Thomas; Pierre Genton; Emeline Mundwiller; Markus Wolff; Christian Marescaux; Richard B. Miles; Michel Baulac; Edouard Hirsch; Eric LeGuern; Stéphanie Baulac

The main familial focal epilepsies are autosomal dominant nocturnal frontal lobe epilepsy, familial temporal lobe epilepsy and familial focal epilepsy with variable foci. A frameshift mutation in the DEPDC5 gene (encoding DEP domain–containing protein 5) was identified in a family with focal epilepsy with variable foci by linkage analysis and exome sequencing. Subsequent pyrosequencing of DEPDC5 in a cohort of 15 additional families with focal epilepsies identified 4 nonsense mutations and 1 missense mutation. Our findings provided evidence of frequent (37%) loss-of-function mutations in DEPDC5 associated with a broad spectrum of focal epilepsies. The implication of a DEP (Dishevelled, Egl-10 and Pleckstrin) domain–containing protein that may be involved in membrane trafficking and/or G protein signaling opens new avenues for research.


Epilepsia | 2012

Epileptic encephalopathies of the Landau-Kleffner and continuous spike and waves during slow-wave sleep types: genomic dissection makes the link with autism.

Gaetan Lesca; Gabrielle Rudolf; Audrey Labalme; Edouard Hirsch; Alexis Arzimanoglou; Pierre Genton; Jacques Motte; Anne de Saint Martin; Maria-Paola Valenti; Clotilde Boulay; Julitta de Bellescize; Pascale Kéo-Kosal; Nadia Boutry-Kryza; Patrick Edery; Damien Sanlaville; Pierre Szepetowski

Purpose:  The continuous spike and waves during slow‐wave sleep syndrome (CSWSS) and the Landau‐Kleffner (LKS) syndrome are two rare epileptic encephalopathies sharing common clinical features including seizures and regression. Both CSWSS and LKS can be associated with the electroencephalography pattern of electrical status epilepticus during slow‐wave sleep and are part of a clinical continuum that at its benign end also includes rolandic epilepsy (RE) with centrotemporal spikes. The CSWSS and LKS patients can also have behavioral manifestations that overlap the spectrum of autism disorders (ASD). An impairment of brain development and/or maturation with complex interplay between genetic predisposition and nongenetic factors has been suspected. A role for autoimmunity has been proposed but the pathophysiology of CSWSS and of LKS remains uncharacterized.

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Julia Scholly

University of Strasbourg

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Anne de Saint Martin

French Institute of Health and Medical Research

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Pierre Kehrli

University of Strasbourg

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Arielle Crespel

University of Montpellier

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