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1/9. Psychogenic seizures mimicking juvenile myoclonic epilepsy: case reports.

    We describe two patients with psychogenic seizures of rare semiology. Both patients (a 23-year-old man and a 26-year-old woman) attended the emergency department because status epilepticus with myoclonic seizures had been diagnosed. seizures were documented with video-electroencephalography. Semiology of seizures were brief myoclonia of both arms resulting in a short elevation of both arms without impairment of consciousness. Ictal EEG registration was without abnormal finding. Psychiatric diagnostic assessment suggested a dissociative disorder and mild depression, respectively. During psychiatric treatment seizures occurred only rarely within a 3-5 months follow-up. One should be aware that juvenile myoclonic epilepsy may be mimicked by psychogenic seizures copyright 2001 BEA Trading Ltd.
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ranking = 1
keywords = status epilepticus, epilepticus, status
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2/9. Myoclonic status epilepticus: video presentation.

    A young woman with juvenile myoclonic epilepsy had recurrent attacks of myoclonic status epilepticus related to a long history of limited compliance and irregular sleep. The diagnosis of this clinical pattern is based mainly on clinical description. A home video captured an attack.
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ranking = 5
keywords = status epilepticus, epilepticus, status
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3/9. Source analysis of polyspike and wave complexes in juvenile myoclonic epilepsy.

    We applied dipole modeling and brain distributed source analysis to find current sources comprising spikes and slow waves of polyspike and wave complexes (PSWC) in patients with juvenile myoclonic epilepsy (JME). The dipoles were localized in frontal, parietal and temporal lobes. The frontal dipoles were clustered in the frontal medial gyrus and fronto-orbital region. A midsagittal frontal current source was observed using brain distributed source analysis in all patients. When the slow wave was analyzed, multiple sources in different cortical regions were detected using dipole modeling and brain distributed analysis. These results show pre-frontal medial current sources corresponding to spikes and many diffuse sources in cortical regions corresponding to wave components of PSWC in patients with JME.
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ranking = 2.5393036051986E-5
keywords = complex
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4/9. Nonconvulsion status epilepticus in patients with juvenile myoclonic epilepsy: types and frequencies.

    Juvenile myoclonic epilepsy (JME) is an idiopathic, age-related generalized epileptic syndrome, featuring generalizedtonic-clonic and absence seizures as well as myoclonic jerks. Except for some case reports, little is known about type and frequency of nonconvulsive status epilepticus (NCSE) in adult patients with JME. In a retrospective study we therefore reviewed all patients with JME, who had been referred to our hospital between 1994 and 1999 for the occurrence of NCSE. Of the 69 patients with JME seen within these 6 years three women had typical absence status-one of them two times-and one woman had impulsive-petit-mal status. All four patients had a history of myoclonic, absence and generalized tonic-clonic seizures. Because of the extreme rarity of impulsive-petit-mal status the corresponding case history is presented in detail. According to our findings the prevalence of NCSE in JME can be estimated at 5.8%, the incidence at 1.2% per year with a clear preponderance of female gender.
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ranking = 5.0138951429615
keywords = status epilepticus, epilepticus, status
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5/9. Non-convulsive status epilepticus: a rare presentation of juvenile myoclonic epilepsy.

    We present a case of a boy with juvenile myoclonic epilepsy (JME) who presented with features of non-convulsive status epilepticus (NCSE). This case highlights the fact that NCSE, even though not a common occurrence in JME, should be kept in mind when a patient with previous history of seizures presents with subtle changes in sensorium with no obvious cause.
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ranking = 5
keywords = status epilepticus, epilepticus, status
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6/9. Worsening of seizures by oxcarbazepine in juvenile idiopathic generalized epilepsies.

    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. methods: We retrospectively studied all patients with IGE first referred to our epilepsy department between January 2001 and June 2003 and treated with OXC. RESULTS: During this period, six patients were identified. All had an aggravation of their epilepsy in both clinical and EEG activities. OXC had been used because of an incorrect diagnosis of focal epilepsy or generalized tonic-clonic seizures (GTCSs) of undetermined origin (no syndromic classification of the epilepsy). Before OXC, only one patient had experienced a worsening of seizures with an inadequate drug (CBZ). Four had juvenile myoclonic epilepsy, one had juvenile absence epilepsy, and one had IGE that could not be classified into a precise syndrome. OXC (dosage range, 300-1,200 mg/day) was used in monotherapy in all of them except for one patient. Aggravation consisted of a clear aggravation of myoclonic jerks (five cases) or de novo myoclonic jerks (one case). Three patients had exacerbation of absence seizures. One patient had worsened dramatically and had absence status, and one had de novo absences after OXC treatment. The effects of OXC on GTCSs were less dramatic, with no worsening in frequency in three and a slight increase in three. CONCLUSIONS: OXC can be added to the list of antiepileptic drugs that can exacerbate myoclonic and absence seizures in IGE.
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ranking = 0.0046317143205092
keywords = status
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7/9. Severe myoclonic epilepsy in infancy: toward an optimal treatment.

    Severe myoclonic epilepsy in infancy, or Dravet syndrome, is one of the catastrophic epilepsy syndromes. In the past, treatment was mainly based on valproate and phenobarbital. Recently, some of the new antiepilepsy drugs, such as topiramate and stiripentol, have been shown to be promising in the treatment of this epilepsy syndrome. The treatment regimen of 12 children with Dravet syndrome and proven mutations in the alpha subunit of the sodium channel SCN1A is reported here. Five patients on the "traditional" treatment regimen are compared with seven children on an "optimal" treatment regimen based on a combination of valproate and topiramate. With respect to the literature and our own experience, we propose guidelines for "optimal" treatment of children with severe myoclonic epilepsy in infancy. This includes prevention of hyperthermia, rigorous treatment of fever, avoiding stressful situations, maintenance treatment based on a combination of only two antiepilepsy drugs (ie, valproate and topiramate), and a strict acute seizure treatment based on benzodiazepines. To prevent long-lasting periods of status epilepticus, this acute seizure treatment must be taught to parents and caregivers.
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ranking = 1
keywords = status epilepticus, epilepticus, status
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8/9. Refractory calculation-induced idiopathic generalized epilepsy: a case report and review of the literature.

    We report a case with calculation-induced idiopathic generalized epilepsy (IGE) that, unlike most patients with IGE, was refractory to medications. This patient had a family history of (1) a similar condition in a relative of hers who, however, did not have identical manifestations, and (2) a mother who had migraine. Our observations illustrate that the occurrence of IGE in families usually follows rather complex patterns of inheritance and that some of them can be refractory to therapy.
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ranking = 5.0786072103972E-6
keywords = complex
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9/9. Oligogenic inheritance in photosensitive juvenile myoclonic epilepsy?

    The interplay of multiple genetic factors, as opposed to monogenic inheritance, is suspected to play a role in many idiopathic generalized epilepsies. This leads to a digenic or oligogenic inheritance model, which although rather simplified, may explain at least some of the clinical observations. Here we describe a family in which the clinical phenotype in the offspring can be explained by a combination of photosensitivity and epilepsy traits that segregated independently of each other. This case history demonstrates the need to evaluate family histories in more detail in order to uncover potential clinical markers for genetic factors in complex epilepsies.
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ranking = 5.0786072103972E-6
keywords = complex
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