juvenile myoclonic epilepsy

Summary

Summary: 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)

Top Publications

  1. Welty T. Juvenile myoclonic epilepsy: epidemiology, pathophysiology, and management. Paediatr Drugs. 2006;8:303-10 pubmed
    b>Juvenile myoclonic epilepsy (JME) is a common epilepsy syndrome that begins most frequently in the early teenage years...
  2. Camfield C, Camfield P. Management guidelines for children with idiopathic generalized epilepsy. Epilepsia. 2005;46 Suppl 9:112-6 pubmed
    ..Many large case series suggest the value of VPA for juvenile myoclonic epilepsy (JME) but the relative value of other, newer AEDs has not been established...
  3. Delgado Escueta A, Medina M, Serratosa J, Castroviejo I, Gee M, Weissbecker K, et al. Mapping and positional cloning of common idiopathic generalized epilepsies: juvenile myoclonus epilepsy and childhood absence epilepsy. Adv Neurol. 1999;79:351-74 pubmed
  4. Mattson R. Overview: idiopathic generalized epilepsies. Epilepsia. 2003;44 Suppl 2:2-6 pubmed
    ..This article reviews the more common IGE syndromes and associated seizure types as the first step in identifying the recent advances in our knowledge of these syndromes. ..
  5. Kothare S, Valencia I, Khurana D, Hardison H, Melvin J, Legido A. Efficacy and tolerability of zonisamide in juvenile myoclonic epilepsy. Epileptic Disord. 2004;6:267-70 pubmed
    The recommended treatment for juvenile myoclonic epilepsy (JME) is valproate (VPA). Recently, topiramate and lamotrigine have also been shown to be effective...
  6. Landvogt C, Buchholz H, Bernedo V, Schreckenberger M, Werhahn K. Alteration of dopamine D2/D3 receptor binding in patients with juvenile myoclonic epilepsy. Epilepsia. 2010;51:1699-706 pubmed publisher
    To quantify extrastriatal and striatal D2/D3 receptor binding in patients with juvenile myoclonic epilepsy (JME) using the high-affinity dopamine D2/D3 receptor positron emission tomography (PET) ligand (18) F-Fallypride ([(18) F]FP)...
  7. Hempelmann A, Heils A, Sander T. Confirmatory evidence for an association of the connexin-36 gene with juvenile myoclonic epilepsy. Epilepsy Res. 2006;71:223-8 pubmed
    b>Juvenile myoclonic epilepsy (JME) is a genetically determined common subtype of idiopathic generalized epilepsy...
  8. de Nijs L, Lakaye B, Coumans B, Leon C, Ikeda T, Delgado Escueta A, et al. EFHC1, a protein mutated in juvenile myoclonic epilepsy, associates with the mitotic spindle through its N-terminus. Exp Cell Res. 2006;312:2872-9 pubmed
    A novel gene, EFHC1, mutated in juvenile myoclonic epilepsy (JME) encodes a protein with three DM10 domains of unknown function and one putative EF-hand motif...
  9. Suzuki T, Delgado Escueta A, Aguan K, Alonso M, Shi J, Hara Y, et al. Mutations in EFHC1 cause juvenile myoclonic epilepsy. Nat Genet. 2004;36:842-9 pubmed
    b>Juvenile myoclonic epilepsy (JME) is the most frequent cause of hereditary grand mal seizures. We previously mapped and narrowed a region associated with JME on chromosome 6p12-p11 (EJM1)...

More Information

Publications62

  1. Pal D, Evgrafov O, Tabares P, Zhang F, Durner M, Greenberg D. BRD2 (RING3) is a probable major susceptibility gene for common juvenile myoclonic epilepsy. Am J Hum Genet. 2003;73:261-70 pubmed
    b>Juvenile myoclonic epilepsy (JME) is a common form of generalized epilepsy that starts in adolescence...
  2. Savic I, Lekvall A, Greitz D, Helms G. MR spectroscopy shows reduced frontal lobe concentrations of N-acetyl aspartate in patients with juvenile myoclonic epilepsy. Epilepsia. 2000;41:290-6 pubmed
    Neuropsychological studies suggest frontal lobe dysfunctions in patients with juvenile myoclonic epilepsy (JME)...
  3. King S. Axonemal protofilament ribbons, DM10 domains, and the link to juvenile myoclonic epilepsy. Cell Motil Cytoskeleton. 2006;63:245-53 pubmed
    b>Juvenile myoclonic epilepsy (JME) is a common neurological disorder that results in short uncontrolled muscle contractions and sometimes more severe seizures. Genetic studies have suggested that JME may be caused by mutations in EFHC1...
  4. Meschaks A, Lindstrom P, Halldin C, Farde L, Savic I. Regional reductions in serotonin 1A receptor binding in juvenile myoclonic epilepsy. Arch Neurol. 2005;62:946-50 pubmed
    b>Juvenile myoclonic epilepsy (JME) is classified as primarily generalized epilepsy and as such is assumed to lack an anatomic substrate...
  5. Ikeda T, Ikeda K, Enomoto M, Park M, Hirono M, Kamiya R. The mouse ortholog of EFHC1 implicated in juvenile myoclonic epilepsy is an axonemal protein widely conserved among organisms with motile cilia and flagella. FEBS Lett. 2005;579:819-22 pubmed
    The gene product of EFHC1 recently implicated in juvenile myoclonic epilepsy (JME) was found to be a homolog of Chlamydomonas axonemal protein Rib72, whose homologs are present in a wide variety of organisms that have motile cilia and ..
  6. Layouni S, Buresi C, Thomas P, Malafosse A, Dogui M. BRD2 and TAP-1 genes and juvenile myoclonic epilepsy. Neurol Sci. 2010;31:53-6 pubmed publisher
    b>Juvenile myoclonic epilepsy (JME) is a genetically determined common subtype of idiopathic generalized epilepsy. Linkage of JME to the chromosomal region 6p21.3 has been reported...
  7. Lin K, Jackowski A, Carrete H, de Araújo Filho G, Silva H, Guaranha M, et al. Voxel-based morphometry evaluation of patients with photosensitive juvenile myoclonic epilepsy. Epilepsy Res. 2009;86:138-45 pubmed publisher
    We aim to investigate structural brain abnormalities in juvenile myoclonic epilepsy (JME) patients with photosensitivity (PS). Sixty JME patients, 19 (32%) of whom were photosensitive, were submitted to 1...
  8. Ding L, Feng H, Macdonald R, Botzolakis E, Hu N, Gallagher M. GABA(A) receptor alpha1 subunit mutation A322D associated with autosomal dominant juvenile myoclonic epilepsy reduces the expression and alters the composition of wild type GABA(A) receptors. J Biol Chem. 2010;285:26390-405 pubmed publisher
    A GABA(A) receptor (GABA(A)R) alpha1 subunit mutation, A322D (AD), causes an autosomal dominant form of juvenile myoclonic epilepsy (ADJME)...
  9. Gelisse P, Genton P, Samuelian J, Thomas P, Bureau M. [Psychiatric disorders in juvenile myoclonic epilepsy]. Rev Neurol (Paris). 2001;157:297-302 pubmed
    Mild personality problems have been described in patients with juvenile myoclonic epilepsy (JME), but clinical practice shows that JME can be diagnosed in patients with more or less severe psychiatric disorders (PD)...
  10. Santiago Rodríguez E, Harmony T, Fernández Bouzas A, Hernández Balderas A, Martinez Lopez M, Graef A, et al. Source analysis of polyspike and wave complexes in juvenile myoclonic epilepsy. Seizure. 2002;11:320-4 pubmed
    ..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...
  11. Medina M, Suzuki T, Alonso M, Duron R, Martinez Juarez I, Bailey J, et al. Novel mutations in Myoclonin1/EFHC1 in sporadic and familial juvenile myoclonic epilepsy. Neurology. 2008;70:2137-44 pubmed publisher
    b>Juvenile myoclonic epilepsy (JME) accounts for 3 to 12% of all epilepsies. In 2004, the GENESS Consortium demonstrated four missense mutations in Myoclonin1/EFHC1 of chromosome 6p12.1 segregating in 20% of Hispanic families with JME...
  12. Gu W, Sander T, Heils A, Lenzen K, Steinlein O. A new EF-hand containing gene EFHC2 on Xp11.4: tentative evidence for association with juvenile myoclonic epilepsy. Epilepsy Res. 2005;66:91-8 pubmed
    ..The EFHC1 gene on 6p12-p11 was previously described as the first susceptibility gene for juvenile myoclonic epilepsy (JME)...
  13. Suzuki T, Delgado Escueta A, Alonso M, Morita R, Okamura N, Sugimoto Y, et al. Mutation analyses of genes on 6p12-p11 in patients with juvenile myoclonic epilepsy. Neurosci Lett. 2006;405:126-31 pubmed
    b>Juvenile myoclonic epilepsy (JME) is a distinct form of idiopathic generalized epilepsy (IGE). One of the candidate regions for human JME has been mapped on chromosome band 6p11-p12 by linkage analyses and is termed EJM1 (MIM 254770)...
  14. Krampfl K, Maljevic S, Cossette P, Ziegler E, Rouleau G, Lerche H, et al. Molecular analysis of the A322D mutation in the GABA receptor alpha-subunit causing juvenile myoclonic epilepsy. Eur J Neurosci. 2005;22:10-20 pubmed
    b>Juvenile myoclonic epilepsy (JME) belongs to the most common forms of hereditary epilepsy, the idiopathic generalized epilepsies...
  15. Cossette P, Liu L, Brisebois K, Dong H, Lortie A, Vanasse M, et al. Mutation of GABRA1 in an autosomal dominant form of juvenile myoclonic epilepsy. Nat Genet. 2002;31:184-9 pubmed
    ..receptor subtype A (GABA(A)), is found in affected individuals of a large French Canadian family with juvenile myoclonic epilepsy. Compared with wildtype receptors, GABA(A) receptors that contain the mutant subunit show a lesser ..
  16. Bai D, Bailey J, Duron R, Alonso M, Medina M, Martinez Juarez I, et al. DNA variants in coding region of EFHC1: SNPs do not associate with juvenile myoclonic epilepsy. Epilepsia. 2009;50:1184-90 pubmed publisher
    b>Juvenile myoclonic epilepsy (JME) accounts for 3 to 12% of all epilepsies. In 2004, we identified a mutation-harboring Mendelian gene that encodes a protein with one EF-hand motif (EFHC1) in chromosome 6p12...
  17. Mas C, Taske N, Deutsch S, Guipponi M, Thomas P, Covanis A, et al. Association of the connexin36 gene with juvenile myoclonic epilepsy. J Med Genet. 2004;41:e93 pubmed
  18. Taske N, Williamson M, Makoff A, Bate L, Curtis D, Kerr M, et al. Evaluation of the positional candidate gene CHRNA7 at the juvenile myoclonic epilepsy locus (EJM2) on chromosome 15q13-14. Epilepsy Res. 2002;49:157-72 pubmed
    A previous study of 34 nuclear pedigrees segregating juvenile myoclonic epilepsy (JME) gave significant evidence of linkage with heterogeneity to marker loci on chromosome 15q13-14 close to the candidate gene CHRNA7 (Hum. Mol. Genet...
  19. Ratnapriya R, Vijai J, Kadandale J, Iyer R, Radhakrishnan K, Anand A. A locus for juvenile myoclonic epilepsy maps to 2q33-q36. Hum Genet. 2010;128:123-30 pubmed publisher
    ..genome linkage analysis in a three-generation south Indian family with multiple members affected with juvenile myoclonic epilepsy (JME). The maximum two-point LOD score obtained was 3...
  20. Manni R, De Icco R, Cremascoli R, Ferrera G, Furia F, Zambrelli E, et al. Circadian phase typing in idiopathic generalized epilepsy: Dim light melatonin onset and patterns of melatonin secretion-Semicurve findings in adult patients. Epilepsy Behav. 2016;61:132-137 pubmed publisher
    ..Twenty adults diagnosed with IGE (grand mal on awakening [GM] in 7 cases and juvenile myoclonic epilepsy in 13 cases) were investigated by means of a face-to-face semistructured sleep interview, Morningness-..
  21. Jaladyan V, Darbinyan V. Insulinoma misdiagnosed as juvenile myoclonic epilepsy. Eur J Pediatr. 2007;166:485-7 pubmed
  22. Mayer T, Schroeder F, May T, Wolf P. Perioral reflex myoclonias: a controlled study in patients with JME and focal epilepsies. Epilepsia. 2006;47:1059-67 pubmed
    ..We studied the frequency of PORM in patients with juvenile myoclonic epilepsy (JME) compared with patients with focal epilepsies...
  23. Kasteleijn Nolst Trenite D, Piccioli M, Parisi P, Hirsch E. Commentary on the usefulness of a morning routine EEG recording in patients with juvenile myoclonic epilepsy. Epilepsy Res. 2008;78:244-5 pubmed publisher
  24. Fittipaldi F, Curra A, Fusco L, Ruggieri S, Manfredi M. EEG discharges on awakening: a marker of idiopathic generalized epilepsy. Neurology. 2001;56:123-6 pubmed
    ..All recordings came from patients with idiopathic generalized epilepsy, predominantly with juvenile myoclonic epilepsy and generalized tonic-clonic seizures on awakening...
  25. Siniatchkin M, Koepp M. Neuroimaging and neurogenetics of epilepsy in humans. Neuroscience. 2009;164:164-73 pubmed publisher
    ..Such studies will help to better understand functional consequences of genetic alterations and may contribute to a better phenotype characterization. ..
  26. Gelisse P, Genton P, Thomas P, Rey M, Samuelian J, Dravet C. Clinical factors of drug resistance in juvenile myoclonic epilepsy. J Neurol Neurosurg Psychiatry. 2001;70:240-3 pubmed
    b>Juvenile myoclonic epilepsy is a comparatively benign form of idiopathic generalised epilepsy. Little is known about the prevalence of difficult to treat or drug resistant patients...
  27. Oğuz Akarsu E, Aydin Özemir Z, Bebek N, Gürses C, Gökyiğit A, Baykan B. Status epilepticus in patients with juvenile myoclonic epilepsy: Frequency, precipitating factors and outcome. Epilepsy Behav. 2016;64:127-132 pubmed publisher
    Status epilepticus (SE) is rarely described in patients with juvenile myoclonic epilepsy (JME), and little is known about its frequency, subtypes, and predictors and the prognosis of these patients...
  28. Renganathan R, Delanty N. Juvenile myoclonic epilepsy: under-appreciated and under-diagnosed. Postgrad Med J. 2003;79:78-80 pubmed
    b>Juvenile myoclonic epilepsy (JME) is a hereditary, idiopathic, generalised epilepsy and is found in 5%-11% of patients with epilepsy...
  29. Larch J, Unterberger I, Bauer G, Reichsoellner J, Kuchukhidze G, Trinka E. Myoclonic status epilepticus in juvenile myoclonic epilepsy. Epileptic Disord. 2009;11:309-14 pubmed publisher
    Myoclonic status epilepticus (MSE) is rarely found in juvenile myoclonic epilepsy (JME) and its clinical features are not well described...
  30. Arntsen V, Sand T, Syvertsen M, Brodtkorb E. Prolonged epileptiform EEG runs are associated with persistent seizures in juvenile myoclonic epilepsy. Epilepsy Res. 2017;134:26-32 pubmed publisher
    In juvenile myoclonic epilepsy (JME), various EEG characteristics have been suggested as poor prognostic signs, but their significance is unclear...
  31. Vidal Dourado M, Nunes K, Guaranha M, Giuliano L, Yacubian E, Manzano G. Expression of praxis induction on cortical excitability in juvenile myoclonic epilepsy. Clin Neurophysiol. 2016;127:2551-60 pubmed publisher
    ..to evaluate the effects of praxis induction on sensorimotor cortical and transcallosal excitability in juvenile myoclonic epilepsy (JME). A total of 36 subjects (18-62years) were included...
  32. Uchida C, de Carvalho K, Guaranha M, Guilhoto L, de Araújo Filho G, Wolf P, et al. Phenotyping juvenile myoclonic epilepsy. Praxis induction as a biomarker of unfavorable prognosis. Seizure. 2015;32:62-8 pubmed publisher
    b>Juvenile myoclonic epilepsy (JME) is a heterogeneous syndrome with seizures presenting typical fluctuation in diurnal cycle and relation with awakening...
  33. Salgueiro A, Velazquez Fragua R, Martinez Bermejo A. [Benign myoclonic epilepsy -a curious case report]. Rev Neurol. 2007;45:152-4 pubmed
    ..Both situations are very uncommon and there is no apparent relationship between them. ..
  34. Kandemir M, Gündüz A, Uzun N, Yeni S, Kızıltan M. Enhanced blink reflex recovery in juvenile myoclonic epilepsy. Neurosci Lett. 2015;609:7-10 pubmed publisher
    b>Juvenile myoclonic epilepsy (JME), which has been attributed to the dysfunction of cortico-thalamic pathway, has been considered to be one of the system epilepsies...
  35. Badawy R, Macdonell R, Jackson G, Berkovic S. Can changes in cortical excitability distinguish progressive from juvenile myoclonic epilepsy?. Epilepsia. 2010;51:2084-8 pubmed publisher
    ..any characteristic cortical excitability changes in progressive myoclonic epilepsy (PME) compared to juvenile myoclonic epilepsy (JME). Six patients with PME were studied...
  36. Wallace R, Hodgson B, Grinton B, Gardiner R, Robinson R, Rodriguez Casero V, et al. Sodium channel alpha1-subunit mutations in severe myoclonic epilepsy of infancy and infantile spasms. Neurology. 2003;61:765-9 pubmed
    ..Less severe mutations associated with GEFS+ could interact with other loci to cause SMEI in cases with a family history of GEFS+. This study extends the phenotypic heterogeneity of mutations in SCN1A to include IS. ..
  37. Cevik N, Koksal A, Dogan V, Dirican A, Bayramoglu S, Ozturk M, et al. Evaluation of cognitive functions of juvenile myoclonic epileptic patients by magnetic resonance spectroscopy and neuropsychiatric cognitive tests concurrently. Neurol Sci. 2016;37:623-7 pubmed publisher
    ..investigating the hypothesis of biochemical changes in frontal cortex and thalamocortical pathways in juvenile myoclonic epilepsy (JME) and the interaction between the biochemical changes and cortical functions...
  38. Wu S, Ye H, Yang X, Lu Z, Qu Q, Qu J. Case-control pharmacogenetic study of HCN1/HCN2 variants and genetic generalized epilepsies. Clin Exp Pharmacol Physiol. 2017;: pubmed publisher
    ..047). Rs7255568 was associated with the risk of CAE (p=0.028) and juvenile myoclonic epilepsy (JME) (p=0.02)...
  39. Marzec M, Edwards J, Sagher O, Fromes G, Malow B. Effects of vagus nerve stimulation on sleep-related breathing in epilepsy patients. Epilepsia. 2003;44:930-5 pubmed
    ..The AHI after VNS treatment remained <5 in the majority of patients and was only mildly elevated (<12) in five patients. In one patient, CPAP resolved VNS-related respiratory events. ..
  40. Fregni F, Boggio P, Valle A, Otachi P, Thut G, Rigonatti S, et al. Homeostatic effects of plasma valproate levels on corticospinal excitability changes induced by 1Hz rTMS in patients with juvenile myoclonic epilepsy. Clin Neurophysiol. 2006;117:1217-27 pubmed
    ..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...
  41. de Araujo Filho G, Pascalicchio T, Lin K, Sousa P, Yacubian E. Neuropsychiatric profiles of patients with juvenile myoclonic epilepsy treated with valproate or topiramate. Epilepsy Behav. 2006;8:606-9 pubmed
    In this cross-sectional study, the neuropsychiatric profiles of 42 patients with juvenile myoclonic epilepsy (JME) who were treated with valproate (VPA) or topiramate (TPM) in monotherapy were compared with the aim of verifying the ..
  42. Erdem H, Yigit A, Culcuoglu A, Mutluer N. Effect of sodium valproate on somatosensory evoked potentials in juvenile myoclonic epilepsy. Ups J Med Sci. 2001;106:197-203 pubmed
    We analysed somatosensory evoked potentials (SEPs) in the patients with juvenile myoclonic epilepsy (JME) in order to find out if sodium valproate (VPA) affects SEP latencies and amplitudes...
  43. Bolszak M, Anttonen A, Komulainen T, Hinttala R, Pakanen S, Sormunen R, et al. Digenic mutations in severe myoclonic epilepsy of infancy. Epilepsy Res. 2009;85:300-4 pubmed publisher
    ..Due to increased risk of valproate-induced liver failure in patients with POLG1 mutations, we recommend POLG1 gene analysis for SMEI patients before valproate administration. ..
  44. Martinez Juarez I, Alonso M, Medina M, Duron R, Bailey J, López Ruiz M, et al. Juvenile myoclonic epilepsy subsyndromes: family studies and long-term follow-up. Brain. 2006;129:1269-80 pubmed
    ..The implication is that juvenile myoclonic epilepsy (JME) does not exist as the sole phenotype of family members and that it should no longer be classified ..
  45. Badawy R, Macdonell R, Jackson G, Berkovic S. Why do seizures in generalized epilepsy often occur in the morning?. Neurology. 2009;73:218-22 pubmed publisher
    ..Thirty drug-naive patients with epilepsy (20 idiopathic generalized epilepsy [IGE], including 10 juvenile myoclonic epilepsy [JME], and 10 focal epilepsy) and 10 control subjects without epilepsy were studied both early in the ..
  46. Oles K, Bell W. Zonisamide concentrations during pregnancy. Ann Pharmacother. 2008;42:1139-41 pubmed publisher
    ..There are no other published data regarding zonisamide concentrations during pregnancy. The change in zonisamide serum concentrations in a pregnant woman suggests an increase in clearance at the end of the second trimester. ..
  47. Alfradique I, Vasconcelos M. Juvenile myoclonic epilepsy. Arq Neuropsiquiatr. 2007;65:1266-71 pubmed
    ..Whenever VPA is judged to be inappropriate, other antiepileptic drugs such as lamotrigine may be considered. Treatment should not be withdrawn, otherwise recurrences are frequent. ..
  48. Grisar T, de Nijs L, Chanas G, Leon C, Coumans B, Foidart A, et al. Some genetic and biochemical aspects of myoclonus. Neurophysiol Clin. 2006;36:271-9 pubmed
    ..of view on how some structural genes could, under certain conditions, when altered, produced idiopathic generalized epilepsy with myoclonic jerks, taking juvenile myoclonic epilepsy (JME) and the myoclonin (EFHC-1) gene as examples.
  49. Azevedo A, Alonso N, Vidal Dourado M, Noffs M, Pascalicchio T, Caboclo L, et al. Validity and reliability of the Portuguese-Brazilian version of the Quality of Life in Epilepsy Inventory-89. Epilepsy Behav. 2009;14:465-71 pubmed publisher
    ..patients with refractory temporal lobe epilepsy (TLE) with mesial temporal sclerosis (MTS) and 51 with juvenile myoclonic epilepsy (JME). Reliability and test-retest reliability were assessed...
  50. Toth V, Rasonyi G, Fogarasi A, Kovacs N, Auer T, Janszky J. Juvenile myoclonic epilepsy starting in the eighth decade. Epileptic Disord. 2007;9:341-5 pubmed
    b>Juvenile myoclonic epilepsy (JME) typically begins at age 10-17 years. We present two patients, with no previous history of epileptic seizures, in whom JME began after the age of 70...
  51. Specchio L, Gambardella A, Giallonardo A, 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;71:32-9 pubmed
    Patients with juvenile myoclonic epilepsy (JME) may be resistant or show adverse effects to valproate. We present a multicenter, prospective, long-term, open-label study evaluating the efficacy and safety of levetiracetam in JME...
  52. Lorenz S, Taylor K, Gehrmann A, Becker T, Muhle H, Gresch M, et al. Association of BRD2 polymorphisms with photoparoxysmal response. Neurosci Lett. 2006;400:135-9 pubmed
    ..response (PPR) has been mapped to the chromosomal region 6p21 near a susceptibility locus for juvenile myoclonic epilepsy (JME)...
  53. Kim A, Moffatt E, Ursell P, Devinsky O, Olgin J, Tseng Z. Sudden neurologic death masquerading as out-of-hospital sudden cardiac death. Neurology. 2016;87:1669-1673 pubmed
    ..causes included intracranial hemorrhage (8), sudden unexpected death in epilepsy (6, including 2 with juvenile myoclonic epilepsy), aneurysmal subarachnoid hemorrhage (2), acute ischemic stroke (1), and aspiration from Huntington ..