Our goal was to investigate the long-term clinical course of juvenile myoclonic epilepsy (JME) in a cohort of patients and to identify prognostic factors for refractoriness and seizure relapse after anti-seizure medications (ASMs) withdrawal. A literature review is also presented to consolidate and compare our findings with the previously reported cases.
We retrospectively studied a series of patients diagnosed with JME with 15 years or more of evolution. We collected clinical, neurophysiological and neuroimaging data from patients who met defined inclusion and exclusion criteria.
Study involved 61 patients (65.5% female) with mean age at study of 37.6 years, and mean age at its outset of 14.8 years. Median follow-up was 31.0 years (mean 28.9, range 15–53). They presented more frequently with a combination of myoclonic and generalized tonic-clonic seizures (GTCS) (65.6%). Sixty-five percent of patients (n = 40) had a 5-year terminal remission with a mean age at last seizure of 27.4 years. Thirty-two percent of seizure-free patients (n = 13) withdrew ASMs: 6 out of 13 had a recurrence of the seizures while 7 remained seizure-free (mean age at ASMs withdrawal 21.0 versus 35.7 years, p < 0.05). In the multivariate model, a high GTCS frequency at onset (p = 0.026) was a prognostic factor of drug resistance.
JME is often regarded as a benign epileptic syndrome, although a quarter of the individuals have refractory epilepsy. The possibility of withdrawing ASMs in patients who have been free of seizures over an extended time seems feasible.
To read this article in full you will need to make a payment
Purchase one-time access:Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
One-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:Subscribe to Brain and Development
Already a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
- Impulsiv-Petit mal (in German).Deutsche Zeitschrift f Nervenheilkunde. 1957; 176: 346-386
- Epidemiology of idiopathic generalized epilepsies.Epilepsia. 2005; 46: 10-14
- Occurrence of only myoclonic jerks in juvenile myoclonic epilepsy.Acta Neurol Scand. 1997; 95: 263-267
- Some clinical and EEG aspects of benign juvenile myoclonic epilepsy.Epilepsia. 1984; 25: 108-114
- Juvenile myoclonic epilepsy of Janz.Neurology. 1984; 34: 285-294
- Photosensitivity in juvenile myoclonic epilepsy.Seizure. 2000; 9: 108-111
- Focal clinical and electroencephalographic features in patients with juvenile myoclonic epilepsy.Acta Neurol Scand. 2010; 122: 115-123
- Resistance to valproic acid as predictor of treatment resistance in genetic generalized epilepsies.Epilepsia. 2017; 58: e64-e69
- Clinical heterogeneity of juvenile myoclonic epilepsy: Follow-up after an interval of more than 20 years.Seizure. 2014; 23: 344-348
- Juvenile Myoclonic Epilepsy: A 5-Year Prospective Study.Epilepsia. 1994; 35: 285-296
- Long-term prognosis of typical childhood absence epilepsy: Remission or progression to juvenile myoclonic epilepsy.Neurology. 1996; 47: 912-918
- Myoclonic seizures subside in the fourth decade in juvenile myoclonic epilepsy.Neurology. 2008; 70: 2123-2129
- Predictors for long-term seizure outcome in juvenile myoclonic epilepsy: 25–63 years of follow-up.Epilepsia. 2012; 53: 1379-1386
- Prognosis of juvenile myoclonic epilepsy 45 years after onset: Seizure outcome and predictors.Neurology. 2013; 81: 2128-2133
- Observations on juvenile myoclonic epilepsy amongst ethnic Bengalees in West Bengal-An Eastern Indian State.Seizure. 2007; 16: 134-141
- Juvenile myoclonic epilepsy 25 years after seizure onset: A population-based study.Neurology. 2009; 73: 1041-1045
- Juvenile myoclonic epilepsy subsyndromes: Family studies and long-term follow-up.Brain. 2006; 129: 1269-1280
- Seizure outcome in 175 patients with juvenile myoclonic epilepsy - A long-term observational study.Epilepsy Res. 2014; 108: 1817-1824
- Pharmacological outcomes in juvenile myoclonic epilepsy: Support for sodium valproate.Epilepsy Res. 2016; 119: 62-66
- Refractory juvenile myoclonic epilepsy: a meta-analysis of prevalence and risk factors.Eur J Neurol. 2019; 26: 856-864
- Consensus on diagnosis and management of JME: From founder’s observations to current trends.Epilepsy Behav. 2013; 28: S87-S90
- Manual for the Wechsler adult intelligence scale - Revised.Psychological Corporation. 1981;
- Prognosis of seizure recurrence after stopping antiepileptic drugs in seizure-free patients: A long-term population-based study of childhood-onset epilepsy.Epilepsy Behav. 2006; 8: 713-719
- Definition of drug resistant epilepsy: Consensus proposal by the ad hoc Task Force of the ILAE Commission on Therapeutic Strategies.Epilepsia. 2010; 51: 1069-1077
- Photosensitivity in epileptic syndromes of childhood and adolescence.Epileptic Disord. 2008; 10: 136-143
- Lifetime prognosis of juvenile myoclonic epilepsy.Epilepsy Behav. 2013; 28: S18-S24
- Psychiatric comorbidity in juvenile myoclonic epilepsy.Epilepsia. 2006; 47: 2086-2091
- Juvenile myoclonic epilepsy: Psychiatric comorbidity and impact on outcome.Epilepsy Behav. 2013; 28: S74-S80
- Risk-taking behavior in juvenile myoclonic epilepsy.Epilepsia. 2013; 54: 2158-2165
- Seizure control in patients with epilepsy: The physician vs. medication factors.BMC Health Serv Res. 2008; 18: 8-264
- Juvenile myoclonic epilepsy refractory to treatment in a tertiary referral center.Epilepsy Behav. 2018; 82: 81-86
- Prognosis of Juvenile myoclonic epilepsy with eye-closure sensitivity.Seizure. 2018; 62: 17-25
Published online: March 26, 2021
Accepted: February 28, 2021
Received in revised form: February 24, 2021
Received: September 28, 2020
© 2021 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.