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Juvenile Myoclonic Epilepsy

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; Adolescent Myoclonic Epilepsy; Epilepsy, Myoclonic 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; Epilepsy, Adolescent Myoclonic; Epilepsy, Juvenile Myoclonic; JMEs (Juvenile Myoclonic Epilepsy); Juvenile Epilepsy, Myoclonic; Myoclonic Juvenile Epilepsy; Epilepsy, Myoclonic, Juvenile; Impulsive Petit Mal, Janz; Janz Syndrome
Networked: 361 relevant articles (26 outcomes, 65 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Epilepsy (Aura)
2. Seizures (Absence Seizure)
3. Epileptic Syndromes
4. Idiopathic Generalized Epilepsy
5. Absence Epilepsy (Childhood Absence Epilepsy)

Experts

1. Delgado-Escueta, Antonio V: 10 articles (01/2019 - 09/2005)
2. Lerche, Holger: 9 articles (09/2019 - 04/2003)
3. Sander, Thomas: 8 articles (05/2010 - 04/2002)
4. Berkovic, Samuel F: 7 articles (01/2019 - 04/2003)
5. Heils, Armin: 7 articles (04/2007 - 04/2002)
6. Sander, T: 6 articles (02/2005 - 04/2000)
7. Striano, Pasquale: 5 articles (01/2022 - 01/2009)
8. Gambardella, Antonio: 5 articles (11/2021 - 09/2006)
9. Abou-Khalil, Bassel: 5 articles (10/2018 - 01/2008)
10. Genton, P: 5 articles (08/2004 - 03/2000)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Juvenile Myoclonic Epilepsy:
1. Valproic Acid (Depakote)FDA LinkGeneric
2. Anticonvulsants (Antiepileptic Drugs)IBA
3. Levetiracetam (Keppra)FDA LinkGeneric
4. Lamotrigine (Lamictal)FDA LinkGeneric
5. LacosamideFDA Link
6. Clonazepam (Rivotril)FDA LinkGeneric
7. Immunoglobulin E (IgE)IBA
8. Zonisamide (Zonegran)FDA LinkGeneric
9. Ethosuximide (Zarontin)FDA LinkGeneric
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. "
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."
02/01/2021 - "In girls and WOCP, levetiracetam and lamotrigine should be considered the first-choice drugs in Generalized Tonic-Clonic Seizures Alone and in Juvenile Myoclonic Epilepsy, lamotrigine in Juvenile Absence Epilepsy, and ethosuximide in Childhood Absence 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

Therapies and Procedures

1. Therapeutics
2. Transcranial Magnetic Stimulation
3. Drug Therapy (Chemotherapy)
4. Anesthesia
5. Aftercare (After-Treatment)