eMedicine Specialties > Neurology > Seizures and Epilepsy
Epilepsy, Juvenile Myoclonic: Treatment & Medication
Updated: Nov 18, 2009
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Treatment
Medical Care
Medical therapy with anticonvulsants typically is needed (see Medication). Avoidance of precipitating events such as alcohol use and sleep deprivation may be useful but is not sufficient to control the seizures of juvenile myoclonic epilepsy (JME).
The selection of AEDs for the treatment of JME depends several factors, including patient's comorbidities, preferences, prior history of adverse events, and gender. Traditionally, divalproex sodium has been used as first-line therapy for JME despite not having an approved FDA indication for this condition. Several studies using lamotrigine, topiramate, levetiracetam, and zonisamide have shown similar efficacy, and in some cases better tolerability, than divalproex sodium. In 2006, levetiracetam became the first drug that received an FDA indication for use specifically in JME.
Surgical Care
Surgical treatment is not indicated, as JME is a primary generalized epilepsy. Some uncontrolled studies have suggested that vagal nerve stimulation might be helpful for patients with intractable seizures of primary generalized onset, such as JME.
Consultations
JME is rarely diagnosed in the primary care setting. Most often, an epileptologist diagnoses the condition after several years of inadequate treatment with medications such as carbamazepine or phenytoin.
Activity
Seizure precautions, including restrictions on driving, must be observed until seizures that impair consciousness are controlled (ie, seizure free) for the recommended period, typically 3-12 months, though the length varies from state to state in the United States. Other precautions include the avoidance of heights, swimming alone, and taking unsupervised baths. Patients with seizures cannot have a commercial driving license until they complete a seizure-free period of 5 years. In addition, patients with seizures are not permitted to fly aircraft.
Studies have shown that patients with JME experience similar decreases in quality of life as compared with other epileptic syndromes.45
Medication
The goal of pharmacotherapy is to reduce morbidity and prevent complications.
The US Food and Drug Administration (FDA) has not approved any anticonvulsant solely for the treatment of JME. In 2006, the FDA approved the adjunctive use of levetiracetam for the treatment of JME. Divalproex sodium has been approved as adjunctive therapy for patients with multiple seizure types that include absence seizures. However, many patients with JME do not have absence seizures. In most patients with JME, seizures are well controlled with monotherapy. Valproic acid has been considered the treatment of choice for JME for many years, but epileptologists are increasingly using other choices as first-line therapies. Approximately 80% of patients with JME become seizure free with valproate monotherapy.
Levetiracetam is useful for the treatment of myoclonic seizures.46,47 It received FDA approval for adjunctive therapy for the treatment of JME in 2006. Noachtar et al demonstrated in a randomized, double-blinded, placebo-controlled trial that levetiracetam adjunctive therapy reduced all seizure types and myoclonic seizures in patients with juvenile myoclonic epilepsy.48 Meta-analysis of 2 randomized controlled trials affirm that JME is highly responsive to treatment with levetiracetam.49 Small, uncontrolled studies of levetiracetam monotherapy in JME suggest efficacy and tolerability.50,51
Lamotrigine may also be useful in the treatment of JME. This agent is ideal for patients who cannot tolerate the adverse effects of valproate, such as weight gain, tremor, stomach upset, and hair loss. In some patients, lamotrigine monotherapy has completely controlled their seizures. However, recent evidence indicates that lamotrigine may exacerbate myoclonic jerks. Data from a recent open-label study suggested that lamotrigine was better tolerated than valproate, with similar efficacy.52 A European expert opinion study showed that lamotrigine was first-line choice for JME in adolescent females while valproate was the first-line choice in adolescent males.53
Topiramate has been useful in the treatment of primary generalized seizures; it may effectively prevent the seizures of JME.54
Findings from an open-label study also suggested that zonisamide might be effective and well tolerated in patients with JME.55
In general, low doses of appropriate anticonvulsants are needed to successfully treat JME. Although treatment with phenytoin, carbamazepine, or phenobarbital may control some seizure components of JME (typically at high doses), these drugs may increase seizure frequency (eg, myoclonic exacerbation with carbamazepine) and occasionally precipitate new seizure types, such as absence seizures. However, they may be used in combination if the patient's condition does not respond to other drugs.56
Clonazepam is often used during seizure exacerbations in patients with JME; however, it is inadequate as long-term treatment.
A patient's medication should rarely changed because he or she is not having seizures. In medical school, physicians are taught to treat patients and not serum concentrations. A low-dose requirement is not unusual; in fact, the great majority of patients with JME need relatively low levels of anticonvulsants to achieve adequate seizure control (as long as it is an appropriate medication for the syndrome). A valproic-acid serum concentration of <20 mcg/mL is certainly a concern, and the patient's spouse or other observer should be interviewed for confirmatory evidence that the patient is not having seizures.
Anticonvulsants
These agents are given to prevent myoclonic, generalized tonic-clonic, and absence seizures.
Divalproex sodium (Depakote)
Indicated for monotherapy or adjunctive therapy in simple and complex absence seizures and adjunctively in many seizure types, including absence. In clinical practice, often first-line anticonvulsant in JME. Metabolized to valproic acid.
Valproic acid (Depakene), rapid-release formulation, available as cap and syrup; Depacon is an IV formulation. Available as 125-, 250-, or 500-mg delayed-release tab.
Dosing recommendations from studies of combination therapy. In practice, patients starting divalproex monotherapy need low starting dose and target doses close to about 10 mg/kg/d. In elderly, clearance of unbound drug decreased; lowered doses needed. Children often require higher doses per weight than adult doses; some children given combination therapy (with enzyme-inducing antiepileptic drugs [EIAEDs]) may need doses as high as 60 mg/kg/d.
Adult
Initial: 15 mg/kg/d PO bid; increase 5-10 mg/kg/d qwk until seizures controlled or adverse effects prevent further increase; present authors usually start monotherapy 250 mg PO bid for 2 wk, then increase by 250 mg until efficacy or adverse effects observed
Pediatric
Initial: Administer as in adults; required dose higher in combination therapy than in monotherapy
Bile acid-binding resins may decrease absorption; inhibits metabolism of lamotrigine, increasing level and possibility of severe rash; similar inhibition of metabolism observed with concomitant ethosuximide, zidovudine, or warfarin; cimetidine, clarithromycin, felbamate may inhibit metabolism and increase serum concentration; rifampin, phenobarbital, carbamazepine, or phenytoin may decrease serum concentration; concomitant use of agents that may impair platelet function or anticoagulation (eg, aspirin, NSAIDs, warfarin) may cause thrombocytopenia
Documented hypersensitivity, hepatic disease or dysfunction, bleeding conditions, some congenital metabolic disorders, thrombocytopenia, hyperammonemia, known teratogenic potential (neural-tube defects probably worsened during first 10 wk of pregnancy). Intracranial surgery might require brief discontinuation of valproate/valproic acid because of the platelet dysfunction. This might also be necessary in other major surgical procedures.
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Hepatic failure resulting in fatalities reported in children; acute fulminant pancreatitis; teratogenicity, eg, neural-tube defects (ie, spina bifida) in children born of mothers taking valproic acid during pregnancy; risk of neural-tube defects increased in certain families; folic acid at least 1 mg/d before and during pregnancy may decrease risk of neural-tube defects. The risk of hepatotoxicity is greatest in those younger than 2 years, as compared with those aged 2-8.
Lamotrigine (Lamictal)
FDA approved as add-on therapy in patients >16 y with partial seizures; recommended as adjunctive therapy in generalized seizures of Lennox-Gastaut syndrome in adults and children; also indicated for conversion to monotherapy after failure of at least 1 EIAED (eg, carbamazepine, phenytoin, phenobarbital). Several reports of efficacy in JME and some of its seizure types; present authors found benefit in some patients.
Well-tolerated anticonvulsant; requires slow uptitration because of risk of rash. Probably has fewer cognitive (ie, sedative) effects than most anticonvulsants; some patients with JME have worsening of myoclonic jerks at low doses. In most patients, increasing dose results in clinically significant improvement.
Serum concentrations useful in monitoring compliance and adjusting dose; as few as months into treatment, serum concentrations may decrease slightly because of enzymatic inducement in liver. Conversion from EIAEDs can be faster than recommended. Conversion from (or add-on therapy with) valproic acid requires slow titration because valproic acid inhibits metabolism of lamotrigine. Starting at high doses may increase incidence of rash.
No IV formulation.
Adult
Monotherapy maintenance dose: 500 mg/d PO divided bid
Polytherapy (added to EIAED): 50 mg/d for 2 wk; increase to 100 mg/d for next 2 wk
Polytherapy (with valproic acid): 25 mg PO qod for 2 wk, followed by 25 mg/d in wk 3 and 4
Authors' regimen: 25 mg PO qhs during first wk (for monotherapy or patients taking EIAEDs); increase by 25 mg/d PO qwk or every other wk until 200 mg/d, then reevaluate efficacy; increase by 50 mg/d every other wk prn
Pediatric
<2 years: Not established
2-12 years: Used as add-on therapy
With divalproex sodium: 0.15 mg/kg/d PO qd or divided bid initial dose; round down to nearest 5 mg; double (0.3 mg/kg/d) 2 wk later (wk 3, 4); usual maintenance dose is 1-5 mg/kg/d; not to exceed 200 mg/d; bid typically best tolerated
With EIAEDs: 0.6 mg/kg/d divided bid initial dose; increase to 1.2 mg/kg/d 2 wk later
>12 years: Administer as in adults
Acetaminophen increases renal clearance, decreasing effects; phenobarbital and phenytoin increase metabolism, decreasing levels; valproic acid increases half-life
Documented hypersensitivity, known drug-induced rash
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Serious rash that requires hospitalization (exceeding dosing recommendations during titration may increase risk of this problem); risk of serious rash similar to that of carbamazepine, phenytoin, phenobarbital, and other anticonvulsants
Topiramate (Topamax)
Indicated and FDA approved as adjunctive therapy for adults and children with partial-onset seizures and primary GTCSs. Approved for monotherapy in primary GTCSs. Some patients with JME have primary GTCSs but may also have myoclonic and absence seizures. Available as 25-, 100-, and 200-mg tab and as 15- and 25-mg sprinkle cap.
Adult
Initial: 25-50 mg PO qhs; increased by 25-50 mg/d qwk bid until maximum tolerated dose or 400 mg/d
Authors' regimen: 25 mg/d initially; increased 25 mg/d bid qwk; this regimen may be best tolerated; target dose in JME is 200 mg/d; titration to higher doses might be needed.
Pediatric
<2 years: Not established
2-16 years
Initial dose: 1-3 mg/kg/d PO; increase by 1-3 mg/kg/d q2wk until maintenance dose achieved
Maintenance dose: 5-9 mg/kg/d PO divided bid
Phenytoin, carbamazepine, and valproic acid substantially reduce levels; reduces digoxin and norethindrone levels; may increase risk of renal-stone formation if given with carbonic anhydrase inhibitors (avoid combination); extreme caution with CNS depressants (may have additive effect in CNS depression as well as other cognitive or neuropsychiatric adverse events)
Documented hypersensitivity, concomitant carbonic anhydrase inhibitors
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Caution in renal or hepatic impairment; eliminated mostly through kidneys; metabolism not well understood; 1.5% risk of renal stones (drug is carbonic anhydrase inhibitor)
Zonisamide (Zonegran)
Indicated for adjunctive treatment of partial seizures with or without secondary generalization. Evidence suggests effectiveness in myoclonic and other generalized seizure types as well.
Adult
100 mg/d PO qd or bid for 2 wk, then increase by 100 mg/d PO q2wk; not to exceed 400 mg/d
Pediatric
Not established
May increase serum carbamazepine levels; carbamazepine may increase concentrations; phenobarbital may decrease levels
Documented hypersensitivity
Pregnancy
C - Safety for use during pregnancy has not been established.
Precautions
May cause drowsiness, weight loss, ataxia, nausea, and slowing of mental activity; increased risk of oligohidrosis and hyperthermia in children
Levetiracetam (Keppra)
Indicated as adjunctive therapy for myoclonic seizures in adults and adolescents and in primary generalized tonic clonic seizures. Mechanism of action is unknown, but it is presumed to involve binding to the SV2A site in synaptic terminals.
Adult
1000-3000 mg/d PO divided in bid administration
Pediatric
10-30 mg/kg/d PO bid
None reported
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Caution in renal impairment; major adverse effects include somnolence, asthenia, incoordination, mild leukopenia (3%) and behavioral changes (eg, anxiety, hostility, emotional lability, depression and psychosis [1-2%], depersonalization); may cause drowsiness, weight loss, ataxia, nausea, and slowing of mental activity; increased risk of oligohidrosis and hyperthermia in children
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References
Herpin TH. Des asces incomplets de l'epilepsie. J Balliere et Fils. 1867.
Rabot T. De la myoclonia epileptique. Paris, France: Medical thesis; 1899.
Lundborg H. Die Progresive Myoklonusepilepsie (Unverricht's Myoklonie). Stockholm, Sweden: Almqvist & Wiksell; 1903.
Janz D, Mathes A. Die Propulsiv Petit Mal Epilepsie. New York, NY: Garger; 1955.
Janz D, Christian W. Impulsive petit mal. Deutsche Leitschrift f Nervenheilkunde. 1957;176:346-386.
Lund M, Reintoft H, Simonsen N. Ein kontrolleret social og psychologisk Undersgelse af Patienter med Juvenil Myoklon Epilepsi. Ugeskr Laeg. 1975;137:2415-18.
Bradley CA, Taghibiglou C, Collingridge GL, Wang YT. Mechanisms involved in the reduction of GABAA receptor alpha1-subunit expression caused by the epilepsy mutation A322D in the trafficking-competent receptor. J Biol Chem. Aug 8 2008;283(32):22043-50. [Medline].
Macdonald RL, Kang JQ. Molecular Pathology of Genetic Epilepsies Associated with GABA(A) Receptor Subunit Mutations. Epilepsy Curr. Jan-Feb 2009;9(1):18-23. [Medline].
Ciumas C, Wahlin TB, Jucaite A, Lindstrom P, Halldin C, Savic I. Reduced dopamine transporter binding in patients with juvenile myoclonic epilepsy. Neurology. Sep 9 2008;71(11):788-94. [Medline].
Delgado-Escueta AV. Advances in genetics of juvenile myoclonic epilepsies. Epilepsy Curr. May-Jun 2007;7(3):61-7. [Medline].
Kinirons P, Rabinowitz D, Gravel M, Long J, Winawer M, Sénéchal G, et al. Phenotypic concordance in 70 families with IGE-implications for genetic studies of epilepsy. Epilepsy Res. Nov 2008;82(1):21-28. [Medline].
Hirano Y, Oguni H, Funatsuka M, Imai K, Osawa M. Differentiation of myoclonic seizures in epileptic syndromes: a video-polygraphic study of 26 patients. Epilepsia. Jun 2009;50(6):1525-35. [Medline].
Baykan B, Altindag EA, Bebek N, Ozturk AY, Aslantas B, Gurses C, et al. Myoclonic seizures subside in the fourth decade in juvenile myoclonic epilepsy. Neurology. May 27 2008;70(22 Pt 2):2123-9. [Medline].
Sadleir LG, Scheffer IE, Smith S, Carstensen B, Carlin J, Connolly MB, et al. Factors influencing clinical features of absence seizures. Epilepsia. Dec 2008;49(12):2100-7. [Medline].
Badawy RA, Macdonell RA, Jackson GD, Berkovic SF. Why do seizures in generalized epilepsy often occur in the morning?. Neurology. Jul 21 2009;73(3):218-22. [Medline].
Filho GM, Rosa VP, Lin K, Caboclo LO, Sakamoto AC, Yacubian EM. Psychiatric comorbidity in epilepsy: a study comparing patients with mesial temporal sclerosis and juvenile myoclonic epilepsy. Epilepsy Behav. Jul 2008;13(1):196-201. [Medline].
Piazzini A, Turner K, Vignoli A, Canger R, Canevini MP. Frontal cognitive dysfunction in juvenile myoclonic epilepsy. Epilepsia. Apr 2008;49(4):657-62. [Medline].
Iqbal N, Caswell HL, Hare DJ, Pilkington O, Mercer S, Duncan S. Neuropsychological profiles of patients with juvenile myoclonic epilepsy and their siblings: a preliminary controlled experimental video-EEG case series. Epilepsy Behav. Mar 2009;14(3):516-21. [Medline].
Wallace R. Identification of a new JME gene implicates reduced apoptotic neuronal death as a mechanism of epileptogenesis. Epilepsy Curr. Jan-Feb 2005;5(1):11-3. [Medline].
Suzuki T, Delgado-Escueta AV, Aguan K, et al. Mutations in EFHC1 cause juvenile myoclonic epilepsy. Nat Genet. Aug 2004;36(8):842-9. [Medline].
Conte FF, Ribeiro PA, Marchesini RB, Pascoal VD, Silva JM, Oliveira AR, et al. Expression Profile and Distribution of Efhc1 Gene Transcript During Rodent Brain Development. J Mol Neurosci. Feb 4 2009;[Medline].
Suzuki T, Miyamoto H, Nakahari T, Inoue I, Suemoto T, Jiang B, et al. Efhc1 deficiency causes spontaneous myoclonus and increased seizure susceptibility. Hum Mol Genet. Mar 15 2009;18(6):1099-109. [Medline].
Bai D, Bailey JN, Durón RM, Alonso ME, Medina MT, Martínez-Juárez IE, et al. DNA variants in coding region of EFHC1: SNPs do not associate with juvenile myoclonic epilepsy. Epilepsia. May 2009;50(5):1184-90. [Medline].
Medina MT, Suzuki T, Alonso ME, Durón RM, Martínez-Juárez IE, Bailey JN, et al. Novel mutations in Myoclonin1/EFHC1 in sporadic and familial juvenile myoclonic epilepsy. Neurology. May 27 2008;70(22 Pt 2):2137-44. [Medline].
Pal DK, Evgrafov OV, Tabares P, et al. BRD2 (RING3) is a probable major susceptibility gene for common juvenile myoclonic epilepsy. Am J Hum Genet. Aug 2003;73(2):261-70. [Medline].
de Kovel CG, Pinto D, de Haan GJ, Kasteleijn-Nolst Trenité DG, Lindhout D, Koeleman BP. Association analysis of BRD2 (RING3) and epilepsy in a Dutch population. Epilepsia. Nov 2007;48(11):2191-2. [Medline].
Lin K, Jackowski AP, Carrete H Jr, de Araújo Filho GM, Silva HH, Guaranha MS, et al. Voxel-based morphometry evaluation of patients with photosensitive juvenile myoclonic epilepsy. Epilepsy Res. Oct 2009;86(2-3):138-45. [Medline].
Tae WS, Kim SH, Joo EY, Han SJ, Kim IY, Kim SI, et al. Cortical thickness abnormality in juvenile myoclonic epilepsy. J Neurol. Apr 2008;255(4):561-6. [Medline].
Roebling R, Scheerer N, Uttner I, Gruber O, Kraft E, Lerche H. Evaluation of cognition, structural, and functional MRI in juvenile myoclonic epilepsy. Epilepsia. Jun 1 2009;[Medline].
Kim JH, Lee JK, Koh SB, Lee SA, Lee JM, Kim SI, et al. Regional grey matter abnormalities in juvenile myoclonic epilepsy: a voxel-based morphometry study. Neuroimage. Oct 1 2007;37(4):1132-7. [Medline].
Pulsipher DT, Seidenberg M, Guidotti L, Tuchscherer VN, Morton J, Sheth RD, et al. Thalamofrontal circuitry and executive dysfunction in recent-onset juvenile myoclonic epilepsy. Epilepsia. May 2009;50(5):1210-9. [Medline].
de Araújo Filho GM, Lin K, Lin J, Peruchi MM, Caboclo LO, Guaranha MS, et al. Are personality traits of juvenile myoclonic epilepsy related to frontal lobe dysfunctions? A proton MRS study. Epilepsia. May 2009;50(5):1201-9. [Medline].
Deppe M, Kellinghaus C, Duning T, Möddel G, Mohammadi S, Deppe K, et al. Nerve fiber impairment of anterior thalamocortical circuitry in juvenile myoclonic epilepsy. Neurology. Dec 9 2008;71(24):1981-5. [Medline].
[Guideline] Gaillard WD, Chiron C, Cross JH, Harvey AS, Kuzniecky R, Hertz-Pannier L, et al. Guidelines for imaging infants and children with recent-onset epilepsy. Epilepsia. Sep 2009;50(9):2147-53. [Medline].
Lin K, Carrete H Jr, Lin J, Peruchi MM, de Araújo Filho GM, Guaranha MS, et al. Magnetic resonance spectroscopy reveals an epileptic network in juvenile myoclonic epilepsy. Epilepsia. May 2009;50(5):1191-200. [Medline].
de Araújo Filho GM, Jackowski AP, Lin K, Guaranha MS, Guilhoto LM, da Silva HH, et al. Personality traits related to juvenile myoclonic epilepsy: MRI reveals prefrontal abnormalities through a voxel-based morphometry study. Epilepsy Behav. Jun 2009;15(2):202-7. [Medline].
Akgun Y, Soysal A, Atakli D, Yuksel B, Dayan C, Arpaci B. Cortical excitability in juvenile myoclonic epileptic patients and their asymptomatic siblings: a transcranial magnetic stimulation study. Seizure. Jul 2009;18(6):387-91. [Medline].
Sadleir LG, Scheffer IE, Smith S, Carstensen B, Farrell K, Connolly MB. EEG features of absence seizures in idiopathic generalized epilepsy: impact of syndrome, age, and state. Epilepsia. Jun 2009;50(6):1572-8. [Medline].
Lu Y, Waltz S, Stenzel K, Muhle H, Stephani U. Photosensitivity in epileptic syndromes of childhood and adolescence. Epileptic Disord. Jun 2008;10(2):136-43. [Medline].
Santiago-Rodríguez E, Harmony T, Cárdenas-Morales L, Hernández A, Fernández-Bouzas A. Analysis of background EEG activity in patients with juvenile myoclonic epilepsy. Seizure. Jul 2008;17(5):437-45. [Medline].
Specchio N, Boero G, Michelucci R, Gambardella A, Giallonardo AT, Fattouch J, et al. Effects of levetiracetam on EEG abnormalities in juvenile myoclonic epilepsy. Epilepsia. Apr 2008;49(4):663-9. [Medline].
Labate A, Ambrosio R, Gambardella A, Sturniolo M, Pucci F, Quattrone A. Usefulness of a morning routine EEG recording in patients with juvenile myoclonic epilepsy. Epilepsy Res. Oct 2007;77(1):17-21. [Medline].
Park KI, Lee SK, Chu K, Lee JJ, Kim DW, Nam H. The value of video-EEG monitoring to diagnose juvenile myoclonic epilepsy. Seizure. Mar 2009;18(2):94-9. [Medline].
Stefan H, Paulini-Ruf A, Hopfengärtner R, Rampp S. Network characteristics of idiopathic generalized epilepsies in combined MEG/EEG. Epilepsy Res. Aug 2009;85(2-3):187-98. [Medline].
Westphal-Guitti AC, Alonso NB, Migliorini RC, da Silva TI, Azevedo AM, Caboclo LO, et al. Quality of life and burden in caregivers of patients with epilepsy. J Neurosci Nurs. Dec 2007;39(6):354-60. [Medline].
Sullivan JE, Dlugos DJ. Idiopathic Generalized Epilepsy. Curr Treat Options Neurol. May 2004;6(3):231-242. [Medline].
Specchio LM, Gambardella A, Giallonardo AT, Michelucci R, Specchio N, Boero G, et al. Open label, long-term, pragmatic study on levetiracetam in the treatment of juvenile myoclonic epilepsy. Epilepsy Res. 2006;71(1):32-39. [Medline].
Noachtar S, Andermann E, Meyvisch P, Andermann F, Gough WB, Schiemann-Delgado J. Levetiracetam for the treatment of idiopathic generalized epilepsy with myoclonic seizures. Neurology. Feb 19 2008;70(8):607-16. [Medline].
Rosenfeld WE, Benbadis S, Edrich P, Tassinari CA, Hirsch E. Levetiracetam as add-on therapy for idiopathic generalized epilepsy syndromes with onset during adolescence: analysis of two randomized, double-blind, placebo-controlled studies. Epilepsy Res. Jul 2009;85(1):72-80. [Medline].
Sharpe DV, Patel AD, Abou-Khalil B, Fenichel GM. Levetiracetam monotherapy in juvenile myoclonic epilepsy. Seizure. Jan 2008;17(1):64-8. [Medline].
Verrotti A, Cerminara C, Coppola G, Franzoni E, Parisi P, Iannetti P, et al. Levetiracetam in juvenile myoclonic epilepsy: long-term efficacy in newly diagnosed adolescents. Dev Med Child Neurol. Jan 2008;50(1):29-32. [Medline].
Morris GL, Hammer AE, Kustra RP, Messenheimer JA. Lamotrigine for patients with juvenile myoclonic epilepsy following prior treatment with valproate: results of an open-label study. Epilepsy Behav. Aug 2004;5(4):509-12. [Medline].
Wheless JW, Clarke DF, Arzimanoglou A, Carpenter D. Treatment of pediatric epilepsy: European expert opinion, 2007. Epileptic Disord. Dec 2007;9(4):353-412. [Medline].
Prasad A, Kuzniecky RI, Knowlton RC, et al. Evolving antiepileptic drug treatment in juvenile myoclonic epilepsy. Arch Neurol. Aug 2003;60(8):1100-5. [Medline].
Kothare SV, Valencia I, Khurana DS, et al. Efficacy and tolerability of zonisamide in juvenile myoclonic epilepsy. Epileptic Disord. Dec 2004;6(4):267-70. [Medline].
Nicolson A, Appleton RE, Chadwick DW, Smith DF. The relationship between treatment with valproate, lamotrigine, and topiramate and the prognosis of the idiopathic generalised epilepsies. J Neurol Neurosurg Psychiatry. Jan 2004;75(1):75-9. [Medline].
Tóth V, Rásonyi G, Fogarasi A, Kovács N, Auer T, Janszky J. Juvenile myoclonic epilepsy starting in the eighth decade. Epileptic Disord. Sep 2007;9(3):341-5. [Medline].
Aliberti V, Grunewald RA, Panayiotopoulos CP, Chroni E. Focal electroencephalographic abnormalities in juvenile myoclonic epilepsy. Epilepsia. Mar-Apr 1994;35(2):297-301. [Medline].
Atakli D, Sozuer D, Atay T, et al. Misdiagnosis and treatment in juvenile myoclonic epilepsy. Seizure. Feb 1998;7(1):63-6. [Medline].
Biton V, Montouris GD, Ritter F, et al. A randomized, placebo-controlled study of topiramate in primary generalizedtonic-clonic seizures. Topiramate YTC Study Group. Neurology. Apr 22 1999;52(7):1330-7. [Medline].
Buchanan N. The use of lamotrigine in juvenile myoclonic epilepsy. Seizure. Jun 1996;5(2):149-51. [Medline].
Canevini MP, Mai R, Di Marco C, et al. Juvenile myoclonic epilepsy of Janz: clinical observations in 60 patients. Seizure. Dec 1992;1(4):291-8. [Medline].
Delgado-Escueta AV, Enrile-Bacsal F. Juvenile myoclonic epilepsy of Janz. Neurology. Mar 1984;34(3):285-94. [Medline].
Delgado-Escueta AV, Greenberg DA, Treiman L, et al. Mapping the gene for juvenile myoclonic epilepsy. Epilepsia. 1989;30 Suppl 4:S8-18; discussion S24-7. [Medline].
Delgado-Escueta AV, Serratosa JM, Liu A, et al. Progress in mapping human epilepsy genes. Epilepsia. 1994;35 Suppl 1:S29-40. [Medline].
Dreifuss FE. Juvenile myoclonic epilepsy: characteristics of a primary generalized epilepsy. Epilepsia. 1989;30 Suppl 4:S1-7 discussion S24-7. [Medline].
Durner M, Janz D, Zingsem J, Greenberg DA. Possible association of juvenile myoclonic epilepsy with HLA-DRw6. Epilepsia. Sep-Oct 1992;33(5):814-6. [Medline].
Gram L, Alving J, Sagild JC, Dam M. Juvenile myoclonic epilepsy in unexpected age groups. Epilepsy Res. Mar-Apr 1988;2(2):137-40. [Medline].
Grunewald RA, Panayiotopoulos CP. Juvenile myoclonic epilepsy. A review. Arch Neurol. Jun 1993;50(6):594-8. [Medline].
International League Against Epilepsy. Proposal for classification of epilepsies and epileptic syndromes. Commission on Classification and Terminology of the International League Against Epilepsy. Epilepsia. May-Jun 1985;26(3):268-78. [Medline].
Janz D. Epilepsy with impulsive petit mal (juvenile myoclonic epilepsy). Acta Neurol Scand. Nov 1985;72(5):449-59. [Medline].
Janz D. Juvenile myoclonic epilepsy. Epilepsy with impulsive petit mal. Cleve Clin J Med. 1989;56 Suppl Pt 1:S23-33; discussion S40-2. [Medline].
Janz D. The grand mal epilepsies and the sleeping-waking cycle. Epilepsia. Mar 1962;3:69-109. [Medline].
Kleveland G, Engelsen BA. Juvenile myoclonic epilepsy: clinical characteristics, treatment and prognosis in a Norwegian population of patients. Seizure. Feb 1998;7(1):31-8. [Medline].
Knott C, Panayiotopoulos CP. Carbamazepine in the treatment of generalised tonic clonic seizures in juvenile myoclonic epilepsy [letter]. J Neurol Neurosurg Psychiatry. Apr 1994;57(4):503. [Medline].
Lancman ME, Asconape JJ, Penry JK. Clinical and EEG asymmetries in juvenile myoclonic epilepsy. Epilepsia. Mar-Apr 1994;35(2):302-6. [Medline].
Meencke HJ, Janz D. Neuropathological findings in primary generalized epilepsy: a study of eight cases. Epilepsia. Feb 1984;25(1):8-21. [Medline].
Meencke HJ, Janz D. The significance of microdysgenesia in primary generalized epilepsy: an answer to the considerations of Lyon and Gastaut. Epilepsia. Jul-Aug 1985;26(4):368-71. [Medline].
Obeid T, Panayiotopoulos CP. Juvenile myoclonic epilepsy: a study in Saudi Arabia. Epilepsia. May-Jun 1988;29(3):280-2. [Medline].
Oguni H, Mukahira K, Oguni M, et al. Video-polygraphic analysis of myoclonic seizures in juvenile myoclonic epilepsy. Epilepsia. Mar-Apr 1994;35(2):307-16. [Medline].
Panayiotopoulos CP, Obeid T, Tahan AR. Juvenile myoclonic epilepsy: a 5-year prospective study. Epilepsia. Mar-Apr 1994;35(2):285-96. [Medline].
Panayiotopoulos CP, Obeid T, Waheed G. Differentiation of typical absence seizures in epileptic syndromes. A video EEG study of 224 seizures in 20 patients. Brain. Aug 1989;112 ( Pt 4):1039-56. [Medline].
Panayiotopoulos CP, Tahan R, Obeid T. Juvenile myoclonic epilepsy: factors of error involved in the diagnosis and treatment. Epilepsia. Sep-Oct 1991;32(5):672-6. [Medline].
Pedersen SB, Petersen KA. Juvenile myoclonic epilepsy: clinical and EEG features. Acta Neurol Scand. Mar 1998;97(3):160-3. [Medline].
Penry JK, Dean JC, Riela AR. Juvenile myoclonic epilepsy: long-term response to therapy. Epilepsia. 1989;30 Suppl 4:S19-23; discussion S24-7. [Medline].
Shinnar S, Berg AT, Moshe SL, et al. Discontinuing antiepileptic drugs in children with epilepsy: a prospective study. Ann Neurol. May 1994;35(5):534-45. [Medline].
Wirrell EC, Camfield CS, Camfield PR, et al. Long-term prognosis of typical childhood absence epilepsy: remission or progression to juvenile myoclonic epilepsy. Neurology. Oct 1996;47(4):912-8. [Medline].
Further Reading
Keywords
JME, idiopathic generalized epileptic syndrome, myoclonic jerks, generalized tonic-clonic seizures, GTCSs, absence seizures
