Abstract
Objective
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.
Methods
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.
Results
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.
Conclusion
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.
Keywords
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Article info
Publication history
Published online: March 26, 2021
Accepted:
February 28,
2021
Received in revised form:
February 24,
2021
Received:
September 28,
2020
Identification
Copyright
© 2021 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.
