Publications by authors named "Philippe Gelisse"

54 Publications

An Electroencephalographic Voyage in Search of TWs-The Sirens and Corsairs on the Encephalopathy/EEG Horizon: A Survey of TWs.

J Clin Neurophysiol 2021 Jun 18. Epub 2021 Jun 18.

Department of Neurology, Johns Hopkins Bayview Medical Center, Baltimore, Maryland, U.S.A.; Department of Neurology, Epilepsy Unit Montpellier, Montpellier, France; Intensive Care Units and Department of Neurology, University Hospital Basel, Basel, Switzerland; and University of Basel, Basel, Switzerland.

Summary: Generalized periodic discharges with triphasic wave (TW) morphology, long referred to as TWs, are typical of many toxic, metabolic, infectious, and cerebral structural problems, often in concert. Identifying TWs has been challenging for the electroencephalographer and clinician, as has been their cause, significance, prognosis, and treatment. This review highlights the many different patterns of TWs with commentary on their various causes and etiologies, characteristics, different morbidities, differentiation from nonconvulsive status epilepticus, and their prognosis. The articles in this Journal of Clinical Neurophysiology special issue on TWs will review the many challenges the clinician face when TWs are sighted.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1097/WNP.0000000000000725DOI Listing
June 2021

Stimulus-Induced Rhythmic or Periodic Intermittent Discharges (SIRPIDs) in patients with triphasic waves and Creutzfeldt-Jakob disease.

Clin Neurophysiol 2021 Aug 20;132(8):1757-1769. Epub 2021 May 20.

Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA.

Since the term Stimulus-Induced Rhythmic, Periodic, or Ictal Discharges (SIRPIDs) was introduced into the vocabulary of electrophysiologists/neurologists, there has been an ongoing debate about its significance, as well as its correlation with outcomes. SIRPIDs are frequently seen in patients who are critically ill from various causes. The literature reflects the findings of triphasic morphology, with the generalized periodic discharge (GPD) classification in many patients with SIRPIDs: toxic/metabolic encephalopathies, septic, and hypoxemic/hypercapnic encephalopathies, but also sharp periodic complexes in Creutzfeldt-Jakob disease and advanced Alzheimer's disease. In these settings, GPDs disappear when patients fall asleep and reappear when patients spontaneously wake up, or are awoken by an external stimulus, or sometimes because of a respiratory event, with the possibility of the appearance of GPDs with a cyclic alternating pattern. SIRPIDs may be seen as a transitional pattern between sleep and waking states, corresponding to a postarousal/awakening phenomenon. As SIRPIDs are a transient phenomenon and can usually be recorded repeatedly with each stimulation, the word "Ictal" could be replaced by "Intermittent": Stimulus-Induced Rhythmic or Periodic Intermittent Discharges. However, considering that SIRPIDs may be "potentially ictal" or on an "ictal-interictal continuum" in some situations, the "plus" modifier may be added: SIRPIDs-plus.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.clinph.2021.05.002DOI Listing
August 2021

Lateralized Periodic Discharges: Which patterns are interictal, ictal, or peri-ictal?

Clin Neurophysiol 2021 Jul 27;132(7):1593-1603. Epub 2021 Apr 27.

Epilepsy and EEG Unit, Johns Hopkins Bayview Medical Center, Baltimore, MD, USA.

There is an ongoing debate if Lateralized Periodic Discharges (LPDs) represent an interictal pattern reflecting non-specific but irritative brain injury, or conversely, is an ictal pattern. The challenge is: how to correctly manage these patients? Between this apparent dichotomous distinction, there is a pattern lying along the interictal-ictal continuum (IIC) that we may call "peri-ictal". Peri-ictal means that LPDs are temporally associated with epileptic seizures (although not necessarily in the same recording). Their recognition should lead to careful EEG monitoring and longer periods of video-EEG to detect seizure activity (clinical and/or subclinical seizures). In order to distinguish which kind of LPDs should be considered as representing interictal/irritative brain injury versus ictal/peri-ictal LPDs, a set of criteria, with both clinical/neuroimaging and EEG, is proposed. Among them, the dichotomy LPDs-proper versus LPDs-plus should be retained. Spiky or sharp LPDs followed by associated slow after-waves or periods of flattening giving rise to a triphasic morphology should be included in the definition of LPDs-plus. We propose defining a particular subtype of LPDs-plus that we call "LPDs-max". The LPDs-max pattern corresponds to an ictal pattern, and therefore, a focal non-convulsive status epilepticus, sometimes associated with subtle motor signs and epileptic seizures. LPDs-max include periodic polyspike-wave activity and/or focal burst-suppression-like patterns. LPDs-max have a posterior predominance over the temporo-parieto-occipital regions and are refractory to antiseizure drugs. Interpretations of EEGs in critically ill patients require a global clinical approach, not limited to the EEG patterns. The clinical context and results of neuroimaging play key roles.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.clinph.2021.04.003DOI Listing
July 2021

Benign EEG variants in the sleep-wake cycle: A prospective observational study using the 10-20 system and additional electrodes.

Neurophysiol Clin 2021 Jun 16;51(3):233-242. Epub 2021 Apr 16.

Gui de Chauliac Hospital, Epilepsy Unit, Montpellier, France; Institut National de la Santé et de la Recherche Médicale (INSERM), Unité de Recherche sur les Comportements et Mouvements Anormaux, Montpellier, France. Electronic address:

Objectives: To study the prevalence of benign EEG variants (BEVs) in the sleep-wake cycle among 1163 consecutive patients.

Methods: Prospective, observational EEG study using the 10-20 system with systematically two additional anterior-temporal electrodes. Depending on clinical indications, other electrodes were added. REM sleep identification was based on its characteristic EEG grapho-elements and rapid eye movements, clearly detectable with the additional anterior-temporal and fronto-polar electrodes due to eye proximity. The video-EEG monitoring duration was between 24hours and eight days.

Results: We identified 710 patients (61%) with BEVs. Positive occipital sharp transients of sleep (POSTs) were observed in 36.4% of participants, mu rhythm in 22.4%, lambda waves in 16.7%, wicket spikes (WS) in 15%, 14- and 6-Hz positive bursts in 8.3%, benign sporadic sleep spikes (BSSS) in 3.3%, rhythmic mid-temporal theta burst of drowsiness (RMTD) in 2.15%, midline theta rhythm in 2.1% and six-Hz spike and wave (SW) bursts in 0.1%. WS and RMTD were present during wakefulness, NREM (14.1%, 1.3%, respectively) and REM sleep (3.3%, 1.1%, respectively). Mu rhythm was also observed during NREM (1.5%) and REM sleep (7.7%). Fourteen- and 6-Hz positive bursts were present during NREM (4.5%) and REM sleep (6.5%). BSSS and six-Hz SW bursts were only observed during NREM sleep.

Conclusions: The prevalence of BEVs is much higher than current estimates. POSTs and WS can no longer be considered as unusual patterns but physiological patterns of NREM sleep. RMTD and mu rhythm may be observed during NREM and REM sleep.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.neucli.2021.03.006DOI Listing
June 2021

Epilepsy with eyelid myoclonias (Jeavons syndrome): An electro-clinical study of 40 patients from childhood to adulthood.

Seizure 2021 Apr 26;87:30-38. Epub 2021 Feb 26.

Epilepsy Unit, Hôpital Gui de Chauliac, Montpellier, France; Research Unit (URCMA: Unité de Recherche sur les Comportements et Mouvements Anormaux), INSERM, U661, Montpellier, F-34000, France. Electronic address:

Purpose: To describe the typical and atypical clinical and electroencephalographic (EEG) features of 40 patients with Jeavons syndrome (JS).

Method: Retrospective analysis from two French tertiary centers.

Results: Forty patients were enrolled (31 females and 9 males; sex ratio F/M = 3.44; mean age at epilepsy onset: 6.2 ± 3.4 years [range: 1-15 years]). A positive family history of generalized genetic epilepsy was reported by 13 patients (32.5 %). Eyelid myoclonias with or without absence were the seizure onset in 29 patients (72.5 %), and generalized tonic-clonic seizures in 11 (27.5 %). Over the course of the disease, all had absences. Intellectual disability and psychiatric disorders were reported in 14 (35 %) and 18 patients (45 %), respectively. Focal EEG abnormalities were observed in 65 % of patients, with a posterior (57.7 %) or anterior (30 %) distribution. Generalized EEG discharges were identified in 37 patients (92.5 %). Epileptiform abnormalities were activated during NREM sleep and increased upon awakening. Response to intermittent light stimulation (ILS) was observed in 34 patients (85 %), with an unusual pattern of epileptiform abnormalities at the same frequency of the flashes in 20 patients. Patients with all seizure types were more likely to have this response (p = 0.017).

Conclusion: JS is a lifelong genetic epileptic syndrome with onset in childhood, female preponderance, and a positive family history of epilepsy in one-third of the cases. Focal EEG abnormalities are frequent. Response to ILS appears different from other photosensitive syndromes, with an unusual pattern of photo-induced abnormal synchronization. Intellectual disability and psychiatric disorders are not rare.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.seizure.2021.02.028DOI Listing
April 2021

Failure to recognize muscular artifacts on the EEG may cause a wrong diagnosis of myoclonic status epilepticus.

Epilepsy Behav Rep 2020 5;14:100362. Epub 2020 Apr 5.

Epilepsy Unit, Hôpital Gui de Chauliac, Montpellier, France.

•Quetiapine may cause myoclonus.•Usually, no muscular artifact on the midline EEG electrodes•The EEG pattern of myoclonic jerks is rather polyspike-waves and not only polyspikes.•Failure to recognize muscular artifacts may cause a wrong diagnosis of polyspikes.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.ebr.2020.100362DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7782929PMC
April 2020

Spikes/spike-waves time-locked to the flash frequency during intermittent light stimulation in Jeavons syndrome.

Clin Neurophysiol 2020 10 6;131(10):2479-2481. Epub 2020 Aug 6.

Epilepsy Unit, Hôpital Gui de Chauliac, Montpellier, France; Research Unit (URCMA: Unité de Recherche sur les Comportements et Mouvements Anormaux), INSERM, U661, Montpellier F-34000, France.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.clinph.2020.07.012DOI Listing
October 2020

How to carry out and interpret EEG recordings in COVID-19 patients in ICU?

Clin Neurophysiol 2020 08 13;131(8):2023-2031. Epub 2020 May 13.

Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.

There are questions and challenges regarding neurologic complications in COVID-19 patients. EEG is a safe and efficient tool for the evaluation of brain function, even in the context of COVID-19. However, EEG technologists should not be put in danger if obtaining an EEG does not significantly advance diagnosis or change management in the patient. Not every neurologic problem stems from a primary brain injury: confusion, impaired consciousness that evolves to stupor and coma, and headaches are frequent in hypercapnic/hypoxic encephalopathies. In patients with chronic pulmonary disorders, acute symptomatic seizures have been reported in acute respiratory failure in 6%. The clinician should be aware of the various EEG patterns in hypercapnic/hypoxic and anoxic (post-cardiac arrest syndrome) encephalopathies as well as encephalitides. In this emerging pandemic of infectious disease, reduced EEG montages using single-use subdermal EEG needle electrodes may be used in comatose patients. A full 10-20 EEG complement of electrodes with an ECG derivation remains the standard. Under COVID-19 conditions, an expedited study that adequately screens for generalized status epilepticus, most types of regional status epilepticus, encephalopathy or sleep may serve for most clinical questions, using simplified montages may limit the risk of infection to EEG technologists. We recommend noting whether the patient is undergoing or has been placed prone, as well as noting the body and head position during the EEG recording (supine versus prone) to avoid overinterpretation of respiratory, head movement, electrode, muscle or other artifacts. There is slight elevation of intracranial pressure in the prone position. In non-comatose patients, the hyperventilation procedure should be avoided. At present, non-specific EEG findings and abnormalities should not be considered as being specific for COVID-19 related encephalopathy.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.clinph.2020.05.006DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7217782PMC
August 2020

Open-label, uncontrolled retrospective study of perampanel in adults with Lennox-Gastaut syndrome.

Seizure 2020 Feb 16;75:66-69. Epub 2019 Dec 16.

Centre Saint Paul-H. Gastaut, Marseille, France.

Purpose: Perampanel (PER) was added to the anticonvulsant regimen of 71 patients with Lennox-Gastaut Syndrome (LGS) to evaluate its efficacy against seizures and its tolerability.

Method: We evaluated at 3-month intervals 62 with pure LGS and 9 with LGS-like epileptic encephalopathy (28 females, 43 males, mean age 40.1 ± 11.5 yrs, median 38, range 20-71) in whom PER was introduced by 2 mg steps at 2- to 4-week intervals up to 6 mg/day, with possible dose reduction or increases after that. The Strengthening the Reporting of Observational Studies in Epidemiology (STROBE) guidelines were followed.

Results: Mean PER exposure was 538.9 days ± 425 (median 429), with 44 patients (62 %) on PER at last follow-up. About 2/3 of patients were responders, including 35.2 % that had a ≥75 % decrease in their seizures. Among these 16.9 % had a ≥90 % decrease. No improvement was seen in 14 patients; 5 had a less than 50 % response, and 6 had seizure aggravation. Therefore, 25 (35.2 %) were considered non-responders. Half of the patients developed at least one side-effect. Significant negative changes in behavior were noted in 1/3 of the cases, including irritability (8.5 %) and aggressivity (7 %). Contrastingly, 4 patients reported positive behavioral and psychological well-being side-effects.

Conclusions: This retrospective, open-label study provides evidence that PER may significantly help in LGS. PER should be tried in LGS patients who are not satisfactorily controlled. Its use may be limited in some patients due to behavioral side-effects occurring, particularly at doses ≥ 6 mg/d.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.seizure.2019.12.012DOI Listing
February 2020

Treatment of Juvenile Myoclonic Epilepsy in Patients of Child-Bearing Potential.

CNS Drugs 2019 03;33(3):195-208

Epilepsy Unit, Hôpital Gui de Chauliac, 80 Avenue Fliche, 34295, Montpellier Cedex 05, France.

Juvenile myoclonic epilepsy (JME) is both a frequent and a very characteristic epileptic syndrome with female preponderance. Treatment of JME in women of childbearing potential must consider multiple factors such as desire for pregnancy, use of contraception, seizure control and previously used antiepileptic drugs (AEDs). Approximately 85% of cases are well controlled with valproate, which remains the reference AED in JME but is nowadays considered unsafe for the expecting mother and her fetus. The prescription of valproate is now severely restricted in women of childbearing potential but may still be considered, at the lowest possible dose and when pregnancies can be reliably planned, with temporary alternatives to valproate prescribed before fertilization. Alternatives have emerged, especially lamotrigine and levetiracetam, but also topiramate, zonisamide, and recently perampanel, but none of these AEDs can be considered fully safe in the context of pregnancy. In special settings, benzodiazepines and barbiturates may be useful. In some cases, combination therapy, especially lamotrigine and levetiracetam, may be useful or even required. However, lamotrigine may have the potential to aggravate JME, with promyoclonic effects. Carbamazepine, oxcarbazepine and phenytoin must be avoided. Valproate, levetiracetam, zonisamide, topiramate if the daily dose is ≤ 200 mg and perampanel if the daily dose is ≤ 10 mg do not affect combined hormonal contraception. Lamotrigine ≥ 300 mg/day has been shown to decrease levonorgestrel levels by 20% but does not compromise combined hormonal contraception. Patients with JME taking oral contraceptive should be counselled on the fact that the estrogenic component can reduce concentrations of lamotrigine by over 50%, putting patients at risk of increased seizures. Pregnancy is a therapeutic challenge, and the risk/benefit ratio for the mother and fetus must be considered when choosing the appropriate drug. Lamotrigine (< 325 mg daily in the European Registry of Antiepileptic Drugs in Pregnancy) and levetiracetam seem to be comparatively safer in pregnancy than other AEDs, especially topiramate and valproate. Plasma concentration of lamotrigine and levetiracetam decreases significantly during pregnancy, and dosage adjustments may be necessary. With persisting generalized tonic-clonic seizures, the combination of lamotrigine and levetiracetam offer the chance of seizure control and lesser risks of major congenital malformations. The risk of malformation increases when valproate or topiramate are included in the drug combination. In one study, the relative risk of autism and autism spectrum disorders (ASD) in children born to women with epilepsy (WWE) treated with valproate were, respectively, 5.2 for autism and 2.9 for ASD versus 2.12 for autism and 1.6 for ASD in WWE not treated with valproate. More studies are needed to assess the risk of autism with AEDs other than valproate. The current knowledge is that the risk appears to be double that in the general population. In patients with JME, valproate remains an essential and life-changing agent. The consequences of a lifetime of poorly controlled epilepsy need to be balanced against the teratogenic risks of valproate during limited times in a woman's life. The management of JME in WWE should include lifestyle interventions, with avoidance of sleep deprivation, and planned pregnancy.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1007/s40263-018-00602-2DOI Listing
March 2019

Perampanel in 12 patients with Unverricht-Lundborg disease.

Epilepsia 2017 04 6;58(4):543-547. Epub 2017 Feb 6.

Centre Saint Paul-H. Gastaut, Marseille, France.

Objective: Perampanel (PER) was used in 12 patients with Unverricht-Lundborg disease (ULD) to evaluate its efficacy against myoclonus and seizures.

Methods: We treated 11 patients with EPM1 mutations (6 F, 5 M, aged 13-62 years) and a 43-year-old man with de novo KCNC1 mutation. PER was introduced by 2 mg steps at 2-4 week intervals until 6 mg/day, with a possible dose reduction or dose increase.

Results: Ten patients had a clear clinical response of myoclonus, and five were able to reduce concomitant therapy. Improvement was noted sometimes as soon as with 2 mg/day. Epileptic seizures stopped on PER in the six patients who still had experienced generalized tonic-clonic or myoclonic seizures (100%). Some abatement of efficacy on myoclonus was seen in two patients who still retained some benefit. Weight gain was reported in six patients (50%). Psychological and behavioral side-effects were observed in six patients (50%) and led to withdrawal of PER in three cases and dose reduction in three, with abatement of the problems.

Significance: This study provides evidence that for ULD patients, PER may show marked efficacy even in severe cases, particularly against myoclonus, but also against seizures. PER should thus be tried in ULD patients whose seizures are not satisfactorily controlled. Its use is limited because of psychological and behavioral side effects, with higher doses of approximately 6 mg/day or greater likely risk factors.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1111/epi.13662DOI Listing
April 2017

Cost-effectiveness analysis of epilepsy surgery in a controlled cohort of adult patients with intractable partial epilepsy: A 5-year follow-up study.

Epilepsia 2016 Oct 5;57(10):1669-1679. Epub 2016 Sep 5.

Department of Functional Neurology and Epileptology and the Institute of Epilepsies, Hospices Civils de Lyon, Lyon, France.

Objective: Despite its well-known effectiveness, the cost-effectiveness of epilepsy surgery has never been demonstrated in France. We compared cost-effectiveness between resective surgery and medical therapy in a controlled cohort of adult patients with partial intractable epilepsy.

Methods: A prospective cohort of adult patients with surgically remediable and medically intractable partial epilepsy was followed over 5 years in the 15 French centers. Effectiveness was defined as 1 year without a seizure, based on the International League Against Epilepsy (ILAE) classification. Clinical outcomes and direct costs were compared between surgical and medical groups. Long-term direct costs and effectiveness were extrapolated over the patients' lifetimes with a Monte-Carlo simulation using a Markov model, and an incremental cost-effectiveness ratio (ICER) was computed. Indirect costs were also evaluated.

Results: Among the 289 enrolled surgery candidates, 207 were operable-119 in the surgical group and 88 in the medical group-65 were not operable and not analyzed here, 7 were finally not eligible, and 10 were not followed. The proportion of patients completely seizure-free during the last 12 months (ILAE class 1) was 69.0% in the operated group and 12.3% in the medical group during the second year (p < 0.001), and it was respectively 76.8% and 21% during the fifth year (p < 0.001). Direct costs became significantly lower in the surgical group the third year after surgery, as a result of less antiepileptic drug use. The value of the discounted ICER was 10,406 (95% confidence interval [CI] 10,182-10,634) at 2 years and 2,630 (CI 95% 2,549-2,713) at 5 years. Surgery became cost-effective between 9 and 10 years after surgery, and even earlier if indirect costs were taken into account as well.

Significance: Our study suggests that in addition to being safe and effective, resective surgery of epilepsy is cost-effective in the medium term. It should therefore be considered earlier in the development of epilepsy.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1111/epi.13492DOI Listing
October 2016

Is there a difference between various presentations of valproate for cognitive outcome after in utero exposure?

Epilepsia 2016 Mar;57(3):523-4

Epilepsy Unit, Gui de Chauliac Hospital, Montpellier, France.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1111/epi.13301DOI Listing
March 2016

Can emotional stress trigger the onset of epilepsy?

Epilepsy Behav 2015 Jul 28;48:15-20. Epub 2015 May 28.

Epilepsy Unit, Hôpital Gui de Chauliac, Montpellier, France; Research Unit (URCMA: Unité de Recherche sur les Comportements et Mouvements Anormaux), INSERM U661, Montpellier, France.

Objective: The aim of this study was to investigate the potential role of an acute adverse stress as "trigger" for the onset of epilepsy.

Methods: Among 4618 consecutive patients, twenty-two reported a major life event within three months before the onset of epilepsy.

Results: All patients had focal epilepsy except one with idiopathic generalized epilepsy. The temporal lobe was involved in 90% of patients with focal epilepsy. More precisely, 13 patients (62% of patients with focal epilepsy) had medial temporal lobe epilepsy (MTLE), two had lateral temporal lobe epilepsy, four had temporoparietooccipital junction epilepsy, and two patients had central lobe epilepsy. The mean age and the median age at onset of epilepsy for patients with MTLE were both 38 years (range: 9.5-65 years). Ten patients had right and three had left MTLE. Among patients with focal epilepsy, MRI was abnormal in 7 (33%) with hippocampal sclerosis in four, periventricular nodular heterotopia in two, and complex cortical dysgenesis in one. The mean age at onset of epilepsy for patients with brain lesions was 26 years (range: 9.5-49). Twelve patients (54%) reported a death as a triggering factor for the onset of their epilepsy. Seven patients (32%) reported that a relationship of trust had been broken. Three patients (14%) had been subjects of violence. No patient reported sexual abuse as a triggering factor.

Conclusion: This study provides evidence that some patients (5/1000 patients) began their seizures in the wake of significant life events. The average age at onset of epilepsy is quite late, around age 30, even in the presence of brain lesions. These patients are emotionally and affectively more prone to have consequences of a stressful life event. The recognition and management of such situations may bring significant relief with improvement of the control of epilepsy.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.yebeh.2015.05.010DOI Listing
July 2015

Mixed myoclonic-absence status epilepticus in juvenile myoclonic epilepsy.

Epileptic Disord 2015 Mar;17(1):95-6

Epilepsy Unit, Hôpital Gui de Chauliac, Montpellier Research Unit URCMA (Unité de recherche sur les comportements et mouvements anormaux), INSERM, U661, Montpellier, France.

Myoclonic status epilepticus or mixed absence-myoclonic status is uncommon in juvenile myoclonic epilepsy (JME), often precipitated by sleep deprivation, withdrawal of medication, or inadequate antiepileptic drugs (Thomas et al., 2006; Crespel et al., 2013). Such episodes respond well to benzodiazepines or valproate (Crespel et al., 2013). We present the video-EEG of a 24-year-old woman with JME and bipolar disorder. She had a confusional state five days after withdrawal of clonazepam (14 mg/d) and introduction of oxazepam (200 mg/d), followed by catatonic stupor with subtle myoclonus of the face and the arms. The EEG showed absence status (figures 1, 2), which stopped after IV injection of clonazepam (1 mg) (figure 3). Consciousness returned to normal [Published with video sequence and figures (1)].
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1684/epd.2014.0719DOI Listing
March 2015

Reversible pseudo-tumoral brain lesion in patient with focal status epilepticus.

J Neuroradiol 2015 Jul 17;42(4):245-7. Epub 2014 Nov 17.

Neurology Department, Montpellier University Hospital Center, Gui-de-Chauliac Hospital, 80, avenue Augustin-Fliche, 34295 Montpellier cedex 5, France.

View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.neurad.2014.10.002DOI Listing
July 2015

Zonisamide for refractory juvenile absence epilepsy.

Epilepsy Res 2014 Sep 14;108(7):1263-6. Epub 2014 May 14.

Epilepsy Unit, Hôpital Gui de Chauliac, Montpellier, France. Electronic address:

Purpose: To examine the clinical effect of zonisamide (ZNS) in patients with drug-resistant juvenile absence epilepsy (JAE).

Methods: Between 2006 and 2010, 13 JAE patients were successively treated with add-on ZNS. Safety and efficacy were assessed according to the patient and caregiver reports at visits every 3 months. Response rate was defined as a 50% or greater reduction in seizure frequency.

Results: Mean age was 42 years. No patient had been seizure free for a period ≥12 months before ZNS. The mean follow-up was 34 months. The mean dosage of ZNS was 388 mg. ZNS was effective for absence seizures (AS) in all patients (more than 50% AS reduction). Four patients reached seizure reduction on 550-600 mg/day. Three (23%) had a reduction in AS frequency >75% and five (38.5%) between 50% and 75%. Seizure freedom was achieved in five patients (38.5%) (three patients with AS only and two with AS plus generalized tonic-clonic seizures (GTCS)). Before ZNS, four patients had AS evolving to absence status. After ZNS, three of them were in the seizure-free group, the later never experienced this type of complication. Among seven patients with AS plus GTCS, two of them did not report any improvement in the frequency of GTCS (29%).

Conclusion: This observational post-marketing study confirms the broad-spectrum activity of ZNS that includes GTCS, myoclonic seizures and now AS. This study provides evidence that add-on ZNS is efficient and well tolerated in adult patients with refractory JAE, even at high doses.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.eplepsyres.2014.04.010DOI Listing
September 2014

Consensus on diagnosis and management of JME: From founder's observations to current trends.

Epilepsy Behav 2013 Jul;28 Suppl 1:S87-90

University of Rome Sapienza II, Rome, Italy.

An international workshop on juvenile myoclonic epilepsy (JME) was conducted in Avignon, France in May 2011. During that workshop, a group of 45 experts on JME, together with one of the founding fathers of the syndrome of JME ("Janz syndrome"), Prof. Dr. Dieter Janz from Berlin, reached a consensus on diagnostic criteria and management of JME. The international experts on JME proposed two sets of criteria, which will be helpful for both clinical and scientific purposes. Class I criteria encompass myoclonic jerks without loss of consciousness exclusively occurring on or after awakening and associated with typical generalized epileptiform EEG abnormalities, with an age of onset between 10 and 25. Class II criteria allow the inclusion of myoclonic jerks predominantly occurring after awakening, generalized epileptiform EEG abnormalities with or without concomitant myoclonic jerks, and a greater time window for age at onset (6-25years). For both sets of criteria, patients should have a clear history of myoclonic jerks predominantly occurring after awakening and an EEG with generalized epileptiform discharges supporting a diagnosis of idiopathic generalized epilepsy. Patients with JME require special management because their epilepsy starts in the vulnerable period of adolescence and, accordingly, they have lifestyle issues that typically increase the likelihood of seizures (sleep deprivation, exposure to stroboscopic flashes in discos, alcohol intake, etc.) with poor adherence to antiepileptic drugs (AEDs). Results of an inventory of the different clinical management strategies are given. This article is part of a supplemental special issue entitled Juvenile Myoclonic Epilepsy: What is it Really?
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.yebeh.2012.11.051DOI Listing
July 2013

Management of juvenile myoclonic epilepsy.

Epilepsy Behav 2013 Jul;28 Suppl 1:S81-6

Epilepsy Unit, Hôpital Gui de Chauliac, Montpellier, France; Research Unit "Movement Disorders" (URMA), Department of Neurobiology, Institute of Functional Genomics, Montpellier, France.

Juvenile myoclonic epilepsy (JME) is a common form of epilepsy and a fairly lifelong disorder that may significantly lower a patient's expectations and potential for a full life. Luckily, it is also a highly treatable disorder, and up to 85% of patients with JME will enjoy satisfactory seizure control. Among anticonvulsants, valproate still stands out as the most efficacious drug, but may be poorly tolerated by some, and is considered unsafe for the fetuses of pregnant women. Alternatives have emerged in recent years, especially levetiracetam, but also topiramate, zonisamide or lamotrigine. In some cases, combination therapy may be useful or even required. One should not forget the potential aggravation induced not only by some commonly used anticonvulsants, especially carbamazepine and oxcarbazepine, but also, in some patients, by lamotrigine. In special settings, older drugs like benzodiazepines and barbiturates may be useful. But the management of JME should also include intervention in lifestyle, with strict avoidance of sleep deprivation and the management of copathologies, including the cognitive and psychiatric problems that are often encountered. With adequate management, there will only remain a small proportion of patients with uncontrolled epilepsy and all of its related problems. Juvenile myoclonic epilepsy is a condition in which the clinician has a fair chance of significantly helping the patient with medication and counseling.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.yebeh.2013.01.001DOI Listing
July 2013

Neurophysiology of juvenile myoclonic epilepsy.

Epilepsy Behav 2013 Jul;28 Suppl 1:S30-9

Center of Sleep Medicine, Neurology Unit, University-Hospital S. Maria della Misericordia, Udine, Italy.

Juvenile myclonic epilepsy (JME) can be firmly diagnosed by a careful interview of the patient focusing on the seizures and by the EEG with the help, if necessary, of long-term video-EEG monitoring using sleep and/or sleep deprivation. Background activity is normal. The interictal EEG shows diffuse or generalized spike-wave (SW) and polyspike-wave (PSW) discharges. In some patients, non-specific changes or misleading features such as focal changes are found. Changes are mostly seen at sleep onset and at awakening. Provoked awakenings are more likely to activate interictal paroxysmal abnormalities than spontaneous awakenings. The presence of a photoparoxysmal response with or without myoclonic jerks (MJ) is common (30% of the cases). Myoclonic jerks are associated with a discharge of fast, irregular, generalized PSWs that predominate anteriorly. Myoclonic jerks appear to be associated with rhythmic EEG (spike) potentials at around 20Hz. These frequencies are in the range of movement-related fast sensorimotor cortex physiological rhythms. The application of jerk-locked averaging technique has provided findings consistent with a cortical origin of MJ. Paired TMS (transcranial magnetic stimulation) studies showed a defective intracortical inhibition, due to impaired GABA-A mediated mechanisms. In this review, we present the EEG characteristics of JME with particular emphasis on the pathophysiology of MJ and on the role of sleep deprivation on interictal and ictal changes.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.yebeh.2012.11.042DOI Listing
July 2013

The history of juvenile myoclonic epilepsy.

Epilepsy Behav 2013 Jul;28 Suppl 1:S2-7

Centre Saint Paul-Hôpital Henri Gastaut, Marseille, France.

Juvenile myoclonic epilepsy (JME) has been the subject of intensive research over the past 25years. It was discovered stepwise in Switzerland and France in the 19th century, adequately described in Germany and Uruguay in the 1950s, and rediscovered in North America in the early 1980s. Juvenile myoclonic epilepsy represents the most common idiopathic epilepsy syndrome. As a tribute to the primary author of the first extensive and detailed clinical description of JME, it is also called the Janz syndrome. Juvenile myoclonic epilepsy is an archetypical epileptic syndrome, with a fairly homogenous presentation and a still largely unknown etiology. Its clinical spectrum now includes cognitive and psychiatric symptoms as significant copathologies, and the elucidation of its probably multiple genetic mechanisms is an ongoing process. Juvenile myoclonic epilepsy may not qualify as a "benign" epilepsy, but seizures in most patients can be managed adequately and patients will not suffer severe limitations in their lifetime expectations.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.yebeh.2013.01.002DOI Listing
July 2013

Dramatic weight loss with rufinamide.

Epilepsia 2013 Jan 10;54(1):e5-8. Epub 2012 Jul 10.

Department of Neurology, Gui de Chauliac Hospital, Montpellier, France.

Rufinamide (RUF) is a novel antiepileptic drug considered as second-line therapy in the treatment of Lennox-Gastaut syndrome. Treatment-emergent adverse events (AEs) have consisted mainly of drowsiness, irritability, vomiting, and loss of appetite. RUF is considered as a "weight-neutral" drug. We found clinically significant weight loss in 7 of 15 consecutive adult patients (47%; 3 male, 4 female, aged 18-31 years) treated with RUF as add-on therapy (800-2,400 mg/day: 23.5-57.1 mg/kg/day). The body mass index (BMI) decreased by 7.3-18.7%. Two patients were obese class I before RUF. Five patients (71%) were underweight before RUF (mild in one case, moderate in two cases, and severe in two cases). Four of these patients stopped RUF because of this adverse effect. RUF was recommenced in two patients using a lower and slower dosing strategy; one patient showed improvement in seizure control and no weight loss but RUF was re-stopped in the second patient because of continued weight loss. Despite of weight loss, RUF was continued in two other patients because it reduced seizure activity. We primarily related weight loss to reduced food intake, that is, loss of appetite and nausea, although in two patients no obvious loss of appetite was reported. RUF can cause clinically significant weight loss in adult patients, even at low dose. This AE can affect patients who are already underweight. There is a possibility that lower starting doses and slower escalation might minimize weight loss, but further information is required to determine whether this is the case.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1111/j.1528-1167.2012.03579.xDOI Listing
January 2013

Sleep before and after temporal lobe epilepsy surgery.

Seizure 2012 May 12;21(4):260-5. Epub 2012 Feb 12.

Epilepsy Unit, Hôpital Gui de Chauliac, Montpellier, France.

Purpose: Patients with epilepsy often complain of non-restorative sleep. This is the consequence of the acute effect of seizures and the chronic effect of epilepsy responsible for disrupting sleep architecture. Other factors such as antiepileptic drugs (AEDs), also play a role in the alteration of sleep organization. The aim of this study was to evaluate the specific effect of seizures and interictal epileptiform abnormalities (IEAs) on sleep, in particular to see whether reducing seizure frequency by epilepsy surgery might improve sleep organization in these patients.

Methods: Eleven patients with refractory mesial temporal lobe epilepsy, who underwent surgical treatment and who were seizure free at the follow-up, were included in the study. Treatment with AEDs was not significantly modified before the second year of follow-up. Patients were evaluated before surgery, at 1-year and 2-year follow-up visits with a videoEEG monitoring (24h/24). At each follow-up visit, interictal epileptiform abnormalities and sleep macrostructure parameters were assessed.

Results: All patients showed a reduction of their IEAs. At 1-year follow-up, total sleep time and REM sleep increased significantly (p=0.032 and p=0.006, respectively). At 2-year follow-up, an important increase of REM sleep was observed (p=0.028). Most significant variations were noted 1 year after surgery. No significant variations were observed between the first and the second year after surgery.

Conclusions: Surgical treatment of temporal lobe epilepsy may improve sleep macrostructure by reducing the number of seizures and of IEAs. These results indirectly confirm the role of epilepsy in disrupting sleep organization chronically.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.seizure.2012.01.007DOI Listing
May 2012

Worsening of negative myoclonus by lamotrigine in a case of idiopathic focal epilepsy of children with long-term follow-up.

Brain Dev 2012 Mar 28;34(3):248-50. Epub 2011 May 28.

Epilepsy Unit, Hôpital Gui de Chauliac, Montpellier, France.

We report the case of a 5-year-old boy, first referred in 1995, with benign epilepsy with centro-temporal spikes (BECTS) with proximal negative myoclonus as the only seizure type who experienced severe aggravation of seizures when lamotrigine (25 mg/d) was prescribed in association to valproate (400 mg/d). Lamotrigine was stopped and progressively the drops of the legs disappeared within 6 months but valproate was continued. The overall prognosis was benign, confirmed by long-term follow-up until age 20, inkeeping with the diagnosis of BECTS. There was a clear relationship between introduction of lamotrigine and the detrimental effect and the condition improved dramatically when lamotrigine was stopped. Thus lamotrigine may aggravate BECTS in certain conditions and should be used with proper care in this indication.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.braindev.2011.05.001DOI Listing
March 2012

Effects of carbamazepine and metabolites on IL-2, IL-5, IL-6, IL-10 and IFN-γ secretion in epileptic patients: the influence of co-medication.

Pharmacol Rep 2011 ;63(1):86-94

Département de Pharmacologie Médicale et Toxicologie, Centre Hospitalier Régional Universitaire de Montpellier, 191, Avenue Gaston Giraud, 34295 Montpellier Cedex 5, France.

Carbamazepine is a widely used anticonvulsive agent. Its metabolic pathway leads not only to the major active metabolite, carbamazepine-10,11-epoxide, but also to minor terminal metabolites such as iminostilbene and acridine. Carbamazepine is usually well-tolerated, but it may lead to rare, but serious, hypersensitive reactions associated with hypereosinophilia. The mechanisms of hypersensitivity reactions to carbamazepine are still largely unknown, and the implications of the cell-mediated immune response (Th1 pathway) or the humoral immune response (Th2 pathway) are still not understood in these reactions. It is also unclear whether the parent drug or its subsequent metabolites are the primary trigger agent. In our study, we performed ex vivo experiments to evaluate the stimulation of cytokine secretion by carbamazepine, carbamazepine-10,11-epoxide, iminostilbene and acridine. IL-5, IL-6 and IL-10 were quantified as markers of the Th2 pathway, and IL-2 and IFN-γ were used as markers of the Th1 pathway. Blood samples (n=24) were obtained from epileptic patients routinely treated with carbamazepine alone or co-treated with lamotrigine or valproate. The concentrations of cytokines in the plasma were determined before and after 3 h stimulation with drugs. We found a significantly positive effect of co-treatment with valproate on the basal level of IL-5 (p<0.01) and IL-10 (p<0.05). IL-5 production increased only in blood stimulated with a high level of acridine (33 μM), whereas IL-6 production was less specifically stimulated (p<0.05). Because IL-5 is the most potent stimulating factor of the eosinophils, we suggest that the potential helper effect of valproate and acridine can lead to hypersensitive reactions to carbamazepine in the context of the humoral immune response.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/s1734-1140(11)70402-9DOI Listing
July 2011

Focal epilepsy as first symptom in CADASIL.

Seizure 2011 Jul 16;20(6):502-4. Epub 2011 Mar 16.

Epilepsy Unit, Gui de Chauliac Hospital, Montpellier, France.

Recurrent transient ischemic attacks, migraine and dementia represent the principal symptoms of cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy (CADASIL). During the course of the disease, about 10% of patients may experience epileptic seizures, mainly related to the presence of an ischemic stroke. We present a 30-year-old woman with new-onset focal epilepsy leading to the diagnosis of CADASIL. The neuropsychological testing revealed no cognitive impairment. Interictal EEG demonstrated spikes and polyspikes with low amplitude over the right occipital region during NREM sleep. MRI showed white-matter hyperintensities on both hemispheres with confluent lesions at the right parieto-occipital junction, with juxtacortical components. Like in multiple sclerosis, we can suppose that this type of white matter lesions, close to the cortex, may be causative of seizures.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.seizure.2011.02.006DOI Listing
July 2011

Valproate treatment after liver transplant in a patient with Lennox-Gastaut syndrome.

Seizure 2011 Jul 3;20(6):500-1. Epub 2011 Mar 3.

Epilepsy Unit, Gui de Chauliac Hospital, Montpellier, France.

Lennox-Gastaut syndrome (LGS) is a well-defined epileptic encephalopathy highly drug resistant. The first-line treatment option is valproate (VPA), usually in combination with lamotrigine. VPA has been linked to serious hepatotoxicity. We report a 22-year-old liver transplanted patient with LGS successfully treated with VPA in combination with phenobarbital (100 mg/d; blood level: 36 mg/l), lamotrigine (125 mg/d; blood level: 4.81 mg/l) and topiramtate (175 mg/d), as well as immunosuppressive, antiviral, anti-anemic, hypo-phosphoric and alkaline medication. On VPA 1000 mg/d, the seizure frequency decreased significantly. Taking into consideration the patient's good tolerance and the normal liver function, VPA was increased to 1500 mg/d. At this dose the daily drop attacks and generalized tonic-clonic seizures totally ceased. The patient presented only some tonic seizures around awakening. During many years, VPA was avoided in this patient because of its potential hepatotoxicity. However the good functioning of the transplanted liver permitted its introduction. VPA can be used safely in liver transplanted patients under the strict control of the hepatic function.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.seizure.2011.02.005DOI Listing
July 2011

Cardiac asystole during a cluster of right temporo-parietal seizures.

Seizure 2011 Mar 26;20(2):181-3. Epub 2010 Nov 26.

Epilepsy Unit, Hôpital Gui de Chauliac, Montpellier, France.

Ictal bradycardia and asystole can appear during an epileptic seizure. They must be promptly recognized and treated due to their potential life threatening consequences. Indeed, they have been implicated in the pathogenesis of sudden unexpected death in epilepsy (SUDEP). We report the case of a 33 year-old woman with a right temporo-parietal lobe epilepsy who presented a 19 s asystole during a cluster of seizures. Careful interview revealed appearance of numerous episodes of fall in the previous years. The patient underwent cardiological investigations including echocardiography and His bundle electrography that resulted to be normal. A pacemaker was immediately implanted and, although she continued presenting seizures, no more episodes of falls occurred. In order to better preview ictal asystole several risk factors need to be searched. Attention should be paid to an accurate medical history (ask for episodes of falls) and a simultaneous ECG/EEG recording. The occurrence of bradycardia during a seizure should lead to further cardiological investigations. This could help preventing the occurrence of dramatic consequences such as traumatic falls or SUDEP.
View Article and Find Full Text PDF

Download full-text PDF

Source
http://dx.doi.org/10.1016/j.seizure.2010.10.031DOI Listing
March 2011
-->