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Juvenile Myoclonic Epilepsy

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A disorder characterized by the onset of myoclonus in adolescence, a marked increase in the incidence of absence seizures (see EPILEPSY, ABSENCE), and generalized major motor seizures (see EPILEPSY, TONIC-CLONIC). The myoclonic episodes tend to occur shortly after awakening. Seizures tend to be aggravated by sleep deprivation and alcohol consumption. Hereditary and sporadic forms have been identified. (From Adams et al., Principles of Neurology, 6th ed, p323)

Also Known As:

Myoclonic Epilepsy, Juvenile; Impulsive Petit Mal Epilepsy; JME (Juvenile Myoclonic Epilepsy); Janz Impulsive Petit Mal; Janz Juvenile Myoclonic Epilepsy; Juvenile Myoclonic Epilepsy of Janz; Myoclonic Epilepsy, Adolescent; Myoclonic Epilepsy, Juvenile, 1; Petit Mal, Impulsive; Petit Mal, Impulsive, Janz; Adolescent Myoclonic Epilepsies; Adolescent Myoclonic Epilepsy; Epilepsies, Adolescent Myoclonic; Epilepsies, Juvenile Myoclonic; Epilepsy, Adolescent Myoclonic; Epilepsy, Juvenile Myoclonic; JMEs (Juvenile Myoclonic Epilepsy); Juvenile Myoclonic Epilepsies; Myoclonic Epilepsies, Adolescent; Myoclonic Epilepsies, Juvenile; Petit Mals, Impulsive; Syndrome, Janz; Epilepsy, Myoclonic, Juvenile; Impulsive Petit Mal, Janz; Janz Syndrome

Networked: 255 relevant articles (15 outcomes, 39 trials/studies)

Relationship Network

Disease Context: Research Results

Nervous System Diseases: 6158
- Central Nervous System Diseases: 1649
  - Brain Diseases: 2114
    - Epilepsy: 22953
      - Myoclonic Epilepsies: 522
        Juvenile Myoclonic Epilepsy: 255

Related Diseases

1.	Epilepsy (Aura)
2.	Seizures (Seizure)
3.	Generalized Epilepsy
4.	Myoclonic Epilepsies (Myoclonic Encephalopathy)
5.	Rolandic Epilepsy (Centrotemporal Epilepsy)

Experts

1.	Sander, Thomas: 8 articles (05/2010 - 04/2002)
2.	Lerche, Holger: 7 articles (07/2014 - 04/2003)
3.	Delgado-Escueta, Antonio V: 7 articles (11/2011 - 09/2005)
4.	Heils, Armin: 7 articles (04/2007 - 04/2002)
5.	Sander, T: 6 articles (02/2005 - 04/2000)
6.	Berkovic, Samuel F: 5 articles (04/2007 - 04/2003)
7.	Giallonardo, Anna Teresa: 4 articles (01/2016 - 09/2006)
8.	Gambardella, Antonio: 4 articles (04/2015 - 04/2006)
9.	Macdonald, Robert L: 4 articles (08/2010 - 06/2004)
10.	Stephani, Ulrich: 4 articles (05/2010 - 10/2006)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Juvenile Myoclonic Epilepsy:

1.	Valproic Acid (Valproate, Semisodium)FDA LinkGeneric 01/01/2005 - "Valproate can be regarded as the recommended first-line treatment for juvenile myoclonic epilepsy based on case series reports. " 09/01/2009 - "The current review presents evidence and arguments supporting the use of a new AED and those supporting the use of valproate as the first-line treatment in a girl with newly diagnosed juvenile myoclonic epilepsy. " 09/01/2009 - "[Juvenile myoclonic epilepsy with recurrent myoclonic status: efficacy of valproate]." 10/01/2015 - "Differential improvement of the sleep quality among patients with juvenile myoclonic epilepsy with valproic acid: A longitudinal sleep questionnaire-based study." 06/01/2006 - "Therefore, we aimed to study the effects of 1 Hz rTMS on corticospinal excitability in patients with juvenile myoclonic epilepsy (JME) in two different conditions: low- or high-plasma valproate levels. "
2.	etiracetam (levetiracetam)FDA LinkGeneric 04/01/2008 - "A multicenter, prospective, long-term, open-label study to evaluate the effects of levetiracetam on electroencephalogram (EEG) abnormalities and photoparoxysmal response (PPR) of patients affected by juvenile myoclonic epilepsy (JME). " 01/01/2008 - "The aim of this study was to evaluate the efficacy and tolerability of levetiracetam (LEV) monotherapy in juvenile myoclonic epilepsy (JME). " 09/01/2006 - "Open label, long-term, pragmatic study on levetiracetam in the treatment of juvenile myoclonic epilepsy." 11/08/2013 - "Levetiracetam is a good option as monotherapy for females with juvenile myoclonic epilepsy. " 01/01/2009 - "Rapid effect of levetiracetam in a case of juvenile myoclonic epilepsy."
3.	Immunoglobulin E (IgE)IBA 07/01/2014 - "We sought to clarify which structural alterations are present in IGE and the most frequent subsyndrome juvenile myoclonic epilepsy (JME). " 06/01/2014 - "A specific deficit in executive function in a subgroup of IGE, juvenile myoclonic epilepsy (JME) has been a particular focus of recent research. " 04/01/2007 - "It has recently been suggested that variation in the BRD2 gene confers increased risk of juvenile myoclonic epilepsy (JME), which accounts for around a quarter of all IGE. " 01/01/2005 - "(d) Myoclonic SE is also uncommon in IGE but occurs in juvenile myoclonic epilepsy. " 09/01/2003 - "In family I, the mother had juvenile myoclonic epilepsy (JME), and the father had IGE. "
4.	lamotrigine (Lamictal)FDA LinkGeneric 12/01/2013 - "Controlled randomized studies recommending the clinical use of lamotrigine in adult populations with the diagnosis of Juvenile Myoclonic Epilepsy are still lacking. " 05/01/2005 - "Effects of lamotrigine monotherapy in patients with newly diagnosed juvenile myoclonic epilepsy: An open-label study." 12/01/2013 - "Efficacy and tolerability of lamotrigine in juvenile myoclonic epilepsy in adults: a prospective, unblinded randomized controlled trial." 07/01/2011 - "To determine the response rate of patients with juvenile myoclonic epilepsy (JME) to lamotrigine (LTG) and identify predictive factors for treatment response. " 07/01/2011 - "Outcome of lamotrigine treatment in juvenile myoclonic epilepsy."
5.	Anticonvulsants (Antiepileptic Drugs)IBA 07/01/2008 - "To analyze background EEG activity of patients with juvenile myoclonic epilepsy (JME) with and without antiepileptic drugs. " 07/01/2007 - "JME (Juvenile Myoclonic Epilepsy) is relatively common and usually responds well to treatment with appropriate anticonvulsants. " 06/01/1993 - "Juvenile myoclonic epilepsy usually responds well to treatment with appropriate anticonvulsants, and misdiagnosis often results in unnecessary morbidity. " 01/01/2005 - "Approximately 15% of patients with juvenile myoclonic epilepsy (JME) are resistant to antiepileptic drugs (AEDs) and may require extensive study to make a differential diagnosis from symptomatic epilepsies. " 02/01/2013 - "Juvenile myoclonic epilepsy (JME) is often successfully managed with anticonvulsants; however, some patients may have medically resistant seizures. "
6.	zonisamide (Zonegran)FDA LinkGeneric 04/01/2007 - "Potential efficacy of zonisamide in refractory juvenile myoclonic epilepsy: retrospective evidence from an Irish compassionate-use case series." 04/01/2007 - "To retrospectively evaluate the efficacy of zonisamide as adjunctive therapy in the treatment of refractory juvenile myoclonic epilepsy. " 04/01/2007 - "In this retrospective study, zonisamide was effective and well-tolerated as adjunctive therapy in patients with refractory juvenile myoclonic epilepsy." 04/01/2007 - "We retrospectively reviewed the records of seven patients with refractory juvenile myoclonic epilepsy, commenced on a compassionate-use basis on zonisamide as adjunctive treatment between October 2001 and September 2004. " 12/01/2004 - "This case study focuses on the effects of zonisamide on the EEG of a patient with juvenile myoclonic epilepsy. "
7.	lacosamide FDA Link 04/01/2012 - "In conclusion, although, the data from our three patients' suggest that lacosamide may be effective in the treatment of juvenile myoclonic epilepsy, larger studies are needed to explore efficacy and role of lacosamide in the treatment of this disorder." 04/01/2012 - "We present three patients with classic clinical and electrographic features of juvenile myoclonic epilepsy that were maintained on lacosamide (one on monotherapy and two as adjuvant therapy). " 04/01/2012 - "Lacosamide treatment of juvenile myoclonic epilepsy." 04/01/2012 - "The role of lacosamide in treatment of idiopathic generalized epilepsy including juvenile myoclonic epilepsy is unknown. "
8.	Ethosuximide (Zarontin)FDA LinkGeneric 08/01/1990 - "We report 3 patients with epilepsy with myoclonic absences and good outcome, to emphasize the importance of a precise diagnosis by means of the polygraphic recording of the attack, the fact the association of sodium valproate and ethosuximide is the most useful therapy and, finally, the possibility that some patients with epilepsy with myoclonic absence may develop Janz's juvenile myoclonic epilepsy." 01/01/2005 - "Voltage-gated Ca2+ channels are involved in neurotransmitter release, in the sustained depolarisation-phase of PDSs, and in the generation of absence seizures; their mutations are a substrate for juvenile myoclonic epilepsy and the absence-like pattern seen in some mice; the antiabsence effect of ethosuximide is due to the inhibition of thalamic T-type Ca2+ channels. " 01/16/2004 - "As regards idiopathic generalised epilepsies: 1. VPA in monotherapy is recommended in all the forms, 48% were controlled; 18% were controlled with VPA + lamotrigine (LTG); 2. Childhood absence epilepsy is controlled up to 50% with VPA and 85% with VPA + ethosuximide (ESM); 3. LTG, CLB, topiramate (TPM) and Rivotril (CLN) are alternatives to be considered in all types of epilepsies and syndromes that are resistant to medication, and 4. In GCTS, VPA should be chosen in low doses in juvenile myoclonic epilepsy of Janz." 12/01/1998 - "The corresponding recommendations are: in typical absences, valproate, ethosuximide and lamotrigine; in atypical absences, valproate and lamotrigine; in juvenile myoclonic epilepsy, valproate, lamotrigine and clobazam; in infantile spasms vigabatrin, ACTH and valproate; in Lennox-Gastaut syndrome, valproate, lamotrigine and vigabatrin; in atonic seizures, valproate and lamotrigine; in simple and complex partial seizures with or without secondary generalization, oxcarbazepine/carbamazepine, valproate/ vigabatrin and lamotrigine; and in status epilepticus lorazepam, diazepam and clonazepam together with phenytoin or fosphenytoin. "
9.	Clonazepam (Rivotril)FDA LinkGeneric 09/01/1989 - "We studied the efficacy of clonazepam (CZP) in control of juvenile myoclonic epilepsy (JME) in 17 patients. " 09/01/1989 - "Clonazepam in juvenile myoclonic epilepsy." 01/16/2004 - "As regards idiopathic generalised epilepsies: 1. VPA in monotherapy is recommended in all the forms, 48% were controlled; 18% were controlled with VPA + lamotrigine (LTG); 2. Childhood absence epilepsy is controlled up to 50% with VPA and 85% with VPA + ethosuximide (ESM); 3. LTG, CLB, topiramate (TPM) and Rivotril (CLN) are alternatives to be considered in all types of epilepsies and syndromes that are resistant to medication, and 4. In GCTS, VPA should be chosen in low doses in juvenile myoclonic epilepsy of Janz." 12/01/1998 - "The corresponding recommendations are: in typical absences, valproate, ethosuximide and lamotrigine; in atypical absences, valproate and lamotrigine; in juvenile myoclonic epilepsy, valproate, lamotrigine and clobazam; in infantile spasms vigabatrin, ACTH and valproate; in Lennox-Gastaut syndrome, valproate, lamotrigine and vigabatrin; in atonic seizures, valproate and lamotrigine; in simple and complex partial seizures with or without secondary generalization, oxcarbazepine/carbamazepine, valproate/ vigabatrin and lamotrigine; and in status epilepticus lorazepam, diazepam and clonazepam together with phenytoin or fosphenytoin. "
10.	Piracetam (Nootropil)IBA 07/01/2005 - "We have treated a patient with juvenile myoclonic epilepsy who had frequent and inconvenient morning myoclonus with 3,200 mg of piracetam daily. " 08/01/2008 - "Levetiracetam, the alpha-ethyl analogue of the nootropic piracetam, is a widely used antiepileptic drug (AED) that provides protection against partial seizures and is also effective in the treatment of primary generalized seizure syndromes including juvenile myoclonic epilepsy. "

Therapies and Procedures

Drug Therapy (Chemotherapy)

12/01/2012 - "Although most patients with juvenile myoclonic epilepsy (JME) achieve remission on antiepileptic drug therapy, <20% appear to remain in remission without treatment. "
05/01/2005 - "It is important that drug therapy for juvenile myoclonic epilepsy(JME), a lifelong disorder requiring long-term therapy, is effective and well tolerated with long-term use. "
11/01/1996 - "Clinical and electroencephalographic data and response to drug therapy of 3 boys and 3 girls aged 14-19 years, mean 16, with juvenile myoclonic epilepsy (JME) are described. "
02/04/2014 - "Although distinction from more common forms of genetic generalized epilepsy, particularly juvenile myoclonic epilepsy, may be challenging early on, the presence or evolution of 1) progressive neurologic disability, 2) failure to respond to antiepileptic drug therapy, and 3) background slowing on EEG should suggest PME. "
01/01/1989 - "Data from 50 patients with juvenile myoclonic epilepsy (JME) were analyzed retrospectively to assess the response to drug therapy--long-term seizure control, relapse rates, and confounding factors in seizure recurrence. "