Philippe Gelisse

Worsening of Seizures by Oxcarbazepine in Juvenile Idiopathic Generalized Epilepsies

Summary:  Purpose: Several studies have shown that carbamazepine (CBZ) may aggravate idiopathic generalized epilepsy (IGE). Oxcarbazepine (OXC) is a new drug chemically related to CBZ. We report six cases of juvenile IGE with a clear aggravation by OXC.

Antimyoclonic Effect of Levetiracetam in 13 Patients with Unverricht–Lundborg Disease: Clinical Observations

Summary:  Purpose: Disabling myoclonus is the main symptom in long‐standing Unverricht–Lundborg disease (ULD), and levetiracetam (LEV) appears to be an effective anticonvulsant with promising short‐term antimyoclonic properties.

Incidence of drug-induced aggravation in benign epilepsy with centrotemporal spikes.

Summary:  Purpose: Benign epilepsy with centrotemporal spikes (BECTS) is characterized by an excellent prognosis. Drug therapy is necessary in only a minority of patients. Carbamazepine (CBZ) and phenobarbital (PB) have been reported to cause electroclinical aggravation in some cases. The incidence of drug‐induced aggravation in BECTS has never been established.

Abnormal neuroimaging in patients with benign epilepsy with centrotemporal spikes.

Summary:  Purpose: Neuroimaging procedures are usually unnecessary in benign epilepsy of childhood with centrotemporal spikes (BECTS) but are often performed before a specific diagnosis has been reached. By definition, BECTS occurs in normal children; however, recent reports have shown that it also can affect children with static brain lesions. We evaluated the prevalence of abnormal neuroimaging in BECTS and assessed whether the lesions had influenced the clinical and EEG expression of this epilepsy.

Senile myoclonic epilepsy: Delineation of a common condition associated with Alzheimer's disease in Down syndrome

In Down syndrome (DS), epilepsy is frequent in all age classes and is recognized as a significant cause of additional handicap and morbidity. Longer life expectancy has led to the recognition of the high incidence of both Alzheimers disease and seizures in elderly persons with DS. Neuropathological markers of AD are found in all DS brains and clinical symptoms of AD become apparent by the age of 60 years and above in over 50% of DS subjects. Following preliminary description of myoclonic seizures and/or myoclonic epilepsy in isolated cases or small series, we wish to report the diagnostic criteria, treatment and prognosis of a specific and recognizable form of epilepsy associated with AD in a larger group of middle-aged to elderly DS patients. This markedly under-recognized entity may indeed concern an already large and steadily increasing number of patients. We reviewed all medical records of patients with DS referred to our centers (Centre Saint Paul-Gastaut, Marseille; Epilepsy Unit, Montpellier University Hospital; Department of Neurology, Hospital General de Asturias, Oviedo) since 1995. DS had been diagnosed in all at birth, and all presented with the typical morphological changes associated with DS. We selected all cases (18) referred as adults with new onset of myoclonic jerks (MJ) and/or behavioral or cognitive deterioration (CD).

Lack of efficacy and potential aggravation of myoclonus with lamotrigine in Unverricht-Lundborg disease.

Summary:  Purpose: Unverricht‐Lundborg disease (ULD) is a progressive myoclonus epilepsy with tonic–clonic seizures, action myoclonus, mild ataxia, without dementia. Persistence of invalidating action myoclonus is a major problem. Drugs like phenytoin can aggravate ULD. In this study, we retrospectively analyzed the effect of add‐on lamotrigine (LTG) in the five patients under our care who received LTG.

Parasagittal transinsular electrodes for stereo-EEG in temporal and insular lobe epilepsies.

Objective: Direct invasive EEG recordings of the insula – due to its particular anatomical position, deeply seated between both opercula – can only be performed with intracerebral electrodes. To date, the technique most commonly used for insular stereoelectroencephalography (SEEG) is the orthogonal-transopercular electrode approach with the Talairach methodology. We propose another technique utilizing MRI with transinsular parasagittal electrodes and a posterior entry point. This avoids passing through the opercula and sylvian vessels running over the insular surface. Methods: Nine patients, whose seizures implicated the insula, underwent brain surgery. Under general anesthesia with a Leksell frame, 3D-T1 SPGR MRI with gadolinium enhancement was achieved. Surgical planning was performed using the StealthStation with an entry point in the parieto-occipital junction setting the target for the first contact of the lead at the most anterior part of the insula. The trajectory was manipulated in order to have at least 4 contacts per electrode track within the insular cortex. All patients had a postoperative MRI to verify the exact position of each contact. Results: Insular seizures were recorded in all patients. There was neither intracranial bleeding nor infection. In all of the cases, except 1, the seizures recorded were found to be propagations of the primary epileptic zone located in the temporal lobe, either in the mesial structures or the superior temporal gyrus. Eight patients were operated, 7 with an antero-mesial temporal lobectomy and 1 with dysplasia of the superior temporal gyrus. No insular resections were performed. Conclusion: We report a novel technique for insular SEEG with parasagittal electrodes, parallel to the insular cortex, with an entry point at the parieto-occipital junction. This technique is based entirely on use of MRI, and avoids passing through the opercula and sylvian vessels.

Are there generalised spike waves and typical absences in benign rolandic epilepsy

Generalised 3 Hz spike wave (SW) discharges with or without absences have been described in children with benign epilepsy with centrotemporal spikes (BECTS), leading to speculations about a continuum between childhood absence epilepsy (CAE) and BECTS. We thus decided to evaluate the prevalence of absence seizures (AS) and generalised 3 Hz SW in patients with BECTS. All patients with BECTS first referred since 1986 have been identified prospectively. Their medical and electroencephalograph (EEG) records were analysed retrospectively, in the search for AS and generalised SW discharges. Over a period of 11 years, we found typical rolandic spikes in 66 newly referred patients; 64 had seizures typical for the condition (18 female, 46 male), two were asymptomatic and were not further analysed. All had routine waking EEG recordings, and 49 children (76%) had at least one sleep EEG. AS or classical generalised 3 Hz SW were never recorded from history or EEG data, respectively. However, 17 patients had some diffuse SW discharges, lasting 1-5 s, which appeared as grossly symmetrical in only seven children, with a clearly asymmetrical aspect in the others. Among these seven patients, the discharges were only seen on awakening in one, both during waking and nREM sleep stage I or II in one and only during nREM sleep stage I or II in five. They were apparently subclinical in all. We thus found neither AS nor classical 3 Hz SW discharges among 64 consecutive patients with BECTS. Brief bursts of bilateral abnormalities occur in about 25% of the cases, mostly with sleepiness. Such findings do not substantiate the existence of a continuum between CAE and BECTS.

Lennox‐Gastaut syndrome with late‐onset and prominent reflex seizures in trisomy 21 patients

Purpose:  Lennox‐Gastaut syndrome (LGS) is a severe epileptic condition characterized by multiple seizure types including tonic seizures, slow spike‐and‐wave discharges on electroencephalography (EEG), and cognitive impairment. LGS can occur in apparently healthy subjects or in patients with preexisting brain damage. The onset peaks between 3 and 5 years of age and the prognosis is usually poor. Herein we report 13 subjects with trisomy 21 who developed LGS.

Benign childhood epilepsy with centrotemporal spikes and hippocampal atrophy.

Summary: A boy without significant family or personal history had three consecutive nocturnal seizures at 1‐month intervals at age 10 years, all simple focal seizures with motor and sensory symptoms, the last with secondary generalization. Waking and napping EEG showed focal sharp changes typical of benign epilepsy with centrotemporal spikes (BECTS). A magnetic resonance imaging documented a marked right hippocampal atrophy (HA). After valproic acid (VPA) therapy, there were no more seizures, and there were fewer EEG changes. An EEG performed in the younger, fully asymptomatic 8‐year‐old sister documented unilateral right focal sharp waves. This case shows that HA, as well as other central nervous system lesions, can be found fortuitously in patients with BECTS.