China
Africa
Explore chapters and articles related to this topic
Sulthiame
Published in Stanley R. Resor, Henn Kutt, The Medical Treatment of Epilepsy, 2020
Wolfgang K. Baier, Herman Doose
As mentioned above, Ingram and Ratcliffe (10) and Bray and Brower (11) in 1963 had reported favorable results using sulthiame to treat patients with myoclonic seizures. In the early 1970s Lerman and Nussbaum (16) studied 52 patients with myoclonic seizures (Lennox-Gastaut syndrome, myoclonic petit mal, juvenile myoclonic epilepsy). The seizures of 83% of this heterogeneous sample were controlled or considerably improved on sulthiame used alone or in combination with other AEDs. Among these 52 patients were 12 with juvenile myoclonic epilepsy. With the addition of sulthiame, all 12 were ultimately rendered seizure-free, 8 on sulthiame alone. This remarkably positive response led the authors to propose sulthiame as the treatment of choice for juvenile myoclonic epilepsy, a recommendation not widely followed.
Seizures
Published in Michael B O’Neill, Michelle Mary Mcevoy, Alf J Nicholson, Terence Stephenson, Stephanie Ryan, Diagnosing and Treating Common Problems in Paediatrics, 2017
Michael B O’Neill, Michelle Mary Mcevoy, Alf J Nicholson, Terence Stephenson, Stephanie Ryan
Juvenile myoclonic epilepsy, also known as Janz’s syndrome, is one of the most common epilepsy syndromes. Approximately 5% of people with epilepsy have juvenile myoclonic epilepsy. The seizures typically begin in early adolescence and persist into adulthood. The seizures are characterised by early-morning jerks of the head, neck and upper limbs that usually occur shortly after waking. Myoclonic jerks are characterised by brief, bilateral, usually symmetrical synchronous muscle contractions. Seizures can be triggered by sleep deprivation, stress, menstruation, photic stimulation or alcohol consumption. Other seizure types can also occur, including generalised tonic–clonic and absence seizures. Typical EEG features are 3 Hz spike and wave discharges. Juvenile myoclonic epilepsy is inherited; however, the exact mode of inheritance is unclear. In 50% of cases there may be a positive family history.
The Problems
Published in John Greene, Ian Bone, Understanding Neurology a problem-orientated approach, 2007
True petit mal seizures: Are associated with a generalized spike-wave discharge.Usually last 30–60 seconds.Usually originate in the temporal lobe.May occur many times per day.May occur in juvenile myoclonic epilepsy.
Retinal ganglion cell complex and visual evoked potentials in levetiracetam treatment
Published in Cutaneous and Ocular Toxicology, 2020
Dicle Hazirolan, Melih Duman, Selda Keskin Guler, Guner Uney, Firdevs Ornek
Levetiracetam is a broad-spectrum, second-generation AED. It is most commonly approved as adjunctive treatment or monotherapy of partial onset seizures with or without secondary generalisation. Other approved indications include adjunctive treatment of myoclonic seizures associated with juvenile myoclonic epilepsy and primary generalised tonic-clonic seizures associated with idiopathic generalised epilepsy1. It is a pyrolidone derivative and has a unique mechanisms of action. Unlike other AEDs, the mechanisms of action of levetiracetam appear to involve neuronal binding to synaptic vesicle protein 2 A, inhibiting calcium release from intraneuronal stores, opposing the activity of negative modulators of GABA- and glycin-gated currents and inhibiting excessive synchronised activity between neurons1. In addition, levetiracetam also inhibits N-type calcium channels1.
New discoveries in progressive myoclonus epilepsies: a clinical outlook
Published in Expert Review of Neurotherapeutics, 2018
Shweta Bhat, Subramaniam Ganesh
Clinical evaluation for the presence of positive symptoms exclusive to ULD and the absence of specific characteristics and markers of other PMEs are, at best, indicative. These include the history of the age of disease onset, examination for lack of significant cognitive impairment along with sensory problems, observation of video documentation of myoclonus, and proper evaluation of electro encephalogram (EEG) recordings over time among others [26]. Differential diagnosis of ULD from Juvenile myoclonic epilepsy (JME) can be difficult in early years, which is overcome by thorough neurologic consideration and evaluation of EEG. EEG of ULD patients will show characteristic abnormal, disorganized with the disturbed background unlike JME [29]. Currently, the accurate diagnosis of ULD, including carrier testing and prenatal diagnosis, is exclusively based on genotyping for the CSTB gene for the presence of a pathogenic mutation. In the absence of defects in the CSTB gene, the persistent clinical features resembling EPM1, reassessment for the existence of other disorders like EPM1-like PME-ataxia AMRF, EPM6, myoclonus epilepsy, and ataxia due to pathogenic variants in the potassium channel (MEAK) should be considered [18].
Withdrawal seizures: possible risk factors
Published in Expert Review of Neurotherapeutics, 2020
Sara Matricardi, Francesca Felicia Operto, Giovanni Farello, Giangennaro Coppola, Alberto Verrotti
Epilepsy syndrome should be carefully evaluated in the decision process at the time of treatment discontinuation. An initial tailored ASM therapy should improve outcomes, and the choice of the ASM should be based on its efficacy and effectiveness for specific epileptic seizures and syndromes [12]. Many epilepsy syndromes, as juvenile myoclonic epilepsy, are drug-dependent conditions, reaching seizure freedom under medications but with a higher relapse rate after drug withdrawal. The absence of age-dependent genetic syndrome may be related to a higher risk of relapse [13,14]. Moreover, a structural etiology is associated with an increased risk of seizure recurrence after discontinuation [15].