Epilepsy & Behavior 28 (2013) S18–S24

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Epilepsy & Behavior

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Review

Lifetime prognosis of juvenile myoclonic epilepsy

Betul Baykan a,⁎, Iris E. Martínez-Juárez b, Ebru A. Altindag c, Carol S. Camfield d, Peter R. Camfield d
a
Istanbul University Epilepsy Center and Istanbul Faculty of Medicine, Department of Neurology, Istanbul, Turkey
b
Epilepsy Clinic and Professor of Clinical Epileptology Fellowship, Faculty of Medicine, National Autonomous University of Mexico and Mexico's National Institute of Neurology,
GENESS Consortium, Mexico
c
Istanbul Bilim University, Epilepsy Center and Faculty of Medicine, Department of Neurology, Istanbul, Turkey
d
Dalhousie University and IWK Health Care Centre, Halifax, Nova Scotia, Canada

a r t i c l e i n f o a b s t r a c t

Article history: Juvenile myoclonic epilepsy (JME) is among the most common types of genetic epilepsies, displaying a good
Accepted 25 June 2012 prognosis when treated with appropriate drugs, but with a well-known tendency to relapse after withdrawal.
The majority of patients with JME have continuing seizures after a follow-up of two decades. However, 17%
Keywords: are able to discontinue medication and remain seizure-free thereafter. Clinicians should remember that there
Juvenile myoclonic epilepsy
is a small but still considerable subgroup of JME patients whose seizures are difficult to treat before informing
Prognosis
Remission
patients with newly-diagnosed JME about their “benign” prognosis. This resistant course is not fully
Therapy resistance explained, though there are many suggested factors. The dominating myoclonic seizures disappear or dimin-
SUDEP ish in severity in the fourth decade of life. Despite the favorable seizure outcome in most of the cases, 3/4 of
Subsyndromes patients with JME have at least one major unfavorable social outcome. The possible subsyndromes of JME, its
Long-term follow-up genetic background, and its pathophysiological and neuroimaging correlates should be further investigated.
Population-based studies
Epidemiology This article is part of a supplemental special issue entitled Juvenile Myoclonic Epilepsy: What is it Really?
Status epilepticus
© 2012 Elsevier Inc. All rights reserved.

1. Introduction
Juvenile myoclonic epilepsy (JME) has been widely recognized and
investigated by clinicians and neuroscientists as a frequent form of idi-
opathic (genetic) generalized epilepsy in the last two decades. It has
been presumed to be a lifelong genetic trait with a high recurrence
rate after withdrawal of antiepileptic drugs (AEDs), but prospective
long-term studies documenting these suggestions are scant. In one of
the first important case-based follow-up studies, 88% of 66 patients
remained seizure-free for ≥3 years of follow-up with valproate (VPA),
but 9 of 11 patients relapsed after VPA discontinuation [1], supporting
the view for high recurrence risk, which is now well-known by
clinicians.
Only one of the relevant very long-term prognostic studies of JME
was population-based [2], showing that all seizure types in JME were
resolved in 17%, and for 13%, only myoclonus persisted 25 years after
seizure onset. Thus, one-third of patients with JME had no more trou-
blesome seizures and AED treatment was no longer used by this seg-
ment of the JME population [2].
⁎ Corresponding author at: Istanbul University, Istanbul Faculty of Medicine, Department
of Neurology, Millet Cad, 34390, Capa, Istanbul, Turkey. Fax: +90 212 533 4393.
E-mail addresses: baykanb@istanbul.edu.tr, betulbaykan@yahoo.com (B. Baykan),
imartinez@innn.edu.mx (I.E. Martínez-Juárez), draykutlu@hotmail.com (E.A. Altindag),
camfield@dal.ca (C.S. Camfield), camfield@dal.ca (P.R. Camfield).
Another long-term study of JME from a tertiary epilepsy center was
started in 1997 [3,4]. There were 48 JME patients (29 females and 19
males aged 39.9± 9.5 years) having a mean follow-up of 19.6±
5.7 years (median: 18.8, range: 10–37 years) with a mean visit count
of 21.6 ±14.3, excluding telephone calls. There was a clear tendency
to delay or forget the yearly visits in 73% of the patients after their
seizures were controlled, partly explaining the rarity of “older” JME
patients in clinical centers throughout the world. The remission for
5 years (either treated or untreated) and relapses were evaluated for
all seizure types in all cases at the end of this study. Fig. 1 shows a com-
parison of the types of seizures at the beginning and at the end of the
study.
During follow-up, 31 out of 48 patients (64.6%) showed a 5-year
remission of myoclonic seizures or GTCS (13 for both seizure types;
27.1%). Sixteen of the thirty-one patients showed relapses of GTCS
or myoclonia with or without triggering factors. The triggering factors
for seizures such as sleep deprivation or dose reduction of AEDs did
not have specific influence on the relapsing seizure types. The num-
ber of relapses with GTCS was clearly higher than the number of re-
lapses with myoclonus.
Although seizures in JME can be controlled with accurate diagno-
sis and appropriate therapy and VPA has been to be effective in 90%
of the patients [5], Baykan et al. found a benign course in only 66.6%
of the patients, whereas 16.7% had pseudo-resistance due to prob-
lems in treatment or lifestyle, even though they were closely

1525-5050/$ – see front matter © 2012 Elsevier Inc. All rights reserved.
http://dx.doi.org/10.1016/j.yebeh.2012.06.036
 B. Baykan et al. / Epilepsy & Behavior 28 (2013) S18–S24
50
45
40
35
30 myoclonus
25 absences
20
GTCS
15
10
5 status epilepticus occurred in 8 (36%) during the course of their epi-
0 lepsy. At the end of the follow-up, 12 (52%) remained on AED treat-
at the onset at the end
Fig. 1. The seizure types of 48 juvenile myoclonic epilepsy patients at the beginning
and at the end of the long-term follow-up of two decades. GTCS: generalized tonic-
clonic (or clonic-tonic-clonic) seizure.
Figure from the study of Ref. [3], not printed in the original paper.
monitored. The true-resistant course observed in another 16.7% was
significantly associated with psychiatric disorders and the presence
of thyroid diseases. In 54.2% of the patients, myoclonia were in remis-
sion for a mean duration of 8.4± 7.7 years, at an average age of 32.9 ±
9.6 years. Of these patients, 6 were on a lower dose of AED in compari-
son to the dosage needed to control the seizures in the beginning, and 5
patients had stopped AED treatment. None of the latter 11 patients ex-
cept one relapsed during the follow-up. Furthermore, 21 other patients
(43.8%) described substantial alleviation after age 31.3± 8.4 in the se-
verity of myoclonia. Thus, although a majority of the JME patients had
continuing seizures after a follow-up of 20 years, remarkably almost
all had either 5-year remission or a substantial alleviation of the myo-
clonic seizures.
Another multicentered follow-up study based on the families of JME
patients with a mean follow-up of 11± 6 years indicated that JME was
perhaps lifelong [6]. This interesting large-scale study exploring the
possibility of subsyndromes of JME will be discussed later in this review.
2. Evidence from population-based studies
Many important basic questions remain about the life course of per-
sons with epilepsy. Population-based studies provide a better under-
standing of the natural history of epilepsy compared to hospital-based
or large case series because they are less likely to be biased. Prospective,
longitudinal population-based cohorts of newly diagnosed individuals
with epilepsy can offer much needed information. Unfortunately,
there are few such studies. In fact, we were able to find only one such
study of adults with JME. This population-based study of idiopathic gen-
eralized epilepsy found 13 patients with JME from a catchment area of
615,000 [7]. Over the next 7–31 years, about one-third had >2-year
terminal remission but none discontinued AED treatment.
There appear to be only two pediatric population-based cohorts
that contain JME patients. The first is a population-based inception co-
hort of 494 children with newly-diagnosed unprovoked epilepsy, aged
1 month through 15 years, followed up prospectively for 15 years
[8]. There were only 7 children with JME. Fifty-seven percent had
remission for more than 3 years and none died during the follow-up.
Medical and social outcomes were not available, because the subjects
were grouped into “idiopathic”, “cryptogenic”, and “symptomatic”
categories and not by syndrome.
The second study is from the Nova Scotia Childhood Epilepsy Study
which is a population-based cohort of 692 children resident in Nova
Scotia with newly-diagnosed epilepsy, whose onset was at the age of
1 month through 15 years [2]. They were followed up prospectively
and each contacted most recently at least 25 years after diagnosis
by patient visit or phone contact. There were 24 patients with JME
(3.5% of all childhood-onset epilepsy) whose mean age of onset was
S19
10 years and 23 were contacted at a mean age of 36 years. All received
an AED initially, but interestingly, only 14 (60%) ever had received VPA.
2.1. Medical outcome [2]
At the end of the 25 years of follow-up, one 36-year-old woman had
drowned during a witnessed seizure in a pool and the remaining 22
were alive. Throughout their entire clinical course, 4 patients had 1–6
GTCS while 5 had 6–20, 9 had 21–99, and 5 had >100 GTCS. Convulsive
ment. There were 3 with intractable epilepsy, each of whom had
received VPA.
Eighteen (78%) of the 23 patients became seizure-free for ≥3 years
and attempted to discontinue AED treatment. We do not know for most
if they chose to stop AED treatment on their own or asked the advice of
a physician. Six continued in remission without AEDs for 5–23 years. In
addition, five others had also discontinued and remained off treatment:
3 had myoclonic seizures only (without AEDs for >18 years), and 2
continued with rare seizures that were felt to be controlled by avoiding
seizure precipitants. Therefore, 11/23 (48%) were entirely off of AED
treatment after 25 years of follow-up.
There were 12 who continued AED treatment at the end of the
follow-up, which includes 7 who had tried to stop AEDs (their seizures
recurred and they then restarted treatment), plus 5 who never
attempted to stop AED treatment. The 5 who had never attempted to
stop AEDs included 1 patient with a terminal remission of 10 years
but the others had >2 years of being seizure-free at some time during
the follow-up [2].
It has been generally accepted that JME is lifelong and that it is un-
wise to discontinue treatment once seizure control has been established
[1,9]. However, in this population-based cohort, 6/23 (26%) patients
with JME were seizure-free and off AED treatment for a mean of
17 years. In addition, 13% experienced only mild myoclonic seizures
which had persisted for up to 22 years after stopping AED treatment.
This seizure type was not felt to be problematic by these adults. In sum-
mary, 39% of the cohort eventually stopped AEDs and became seizure-
free or experienced only mild myoclonus [2].
2.2. Social outcome [2]
Using Likert scales, 70% reported good satisfaction with their health,
work, friendships, and social life. High school graduation was accom-
plished by 87%. Nonetheless, despite educational success, 31% were un-
employed with insufficient income to be self-sufficient 25 years later
(unemployment rate in Nova Scotia at this time was 6.7%). Sixteen
(70%) had been married or lived common-law, but 7 had divorced
with 4 remarrying. However, social isolation was a problem for 7
(30%) who lived alone with few hobbies or outside activities. Fourteen
(61%) had taken a mood or behavior altering medication during the
follow-up (5 took stimulant medications and 9 had taken antidepres-
sants of whom 5 were taking these medications at the end of the
follow-up). Ten women had >1 pregnancy and 4 men fathered a
child. Surprisingly, eleven pregnancies (80%) were unplanned, outside
of a stable relationship. Unfortunately, for this cohort, 74% had at least
1 major unfavorable social outcome noted after at least a 25-year
follow-up [2].
This important study had some limitations. Firstly, the inclusion
criteria limited the age of onset to less than 16 years. Large adult
case-based series suggest that the mean age of onset is approximately
14 years. Secondly, the sample size with JME is modest, although it is
the largest childhood cohort in the literature and has a long follow-up
into mid-adulthood. Lastly, those children who had the syndrome of
childhood absence epilepsy and later evolved into JME during adoles-
cence were not included in the study and may represent a different
S20 B. Baykan et al. / Epilepsy & Behavior 28 (2013) S18–S24
subgroup of JME with a varying prognosis. However, this study is
unique because of its long follow-up and population-based design [2].
While there is little doubt that the majority of people with JME
have a lifelong disorder, this study indicates that there are some
with a relatively short active phase (4–5 years) followed by a long-
term remission without AED treatment. It seems reasonable to con-
sider a trial of stopping AED treatment in late adolescence or young
adulthood for people with JME. It is possible that the relatively opti-
mistic rate of remission is related to the age of the patients at the
end of the follow-up, inasmuch as seizure-provoking factors such as
binge drinking and sleep deprivation likely decrease with age.
3. Factors influencing the lifetime prognosis of JME
Even though JME is thought of as a benign form of idiopathic gener-
alized epilepsy, there are cases with true drug resistance. Little is known
about the prevalence of drug-resistance in patients with JME. True AED
resistance is reported in about 1/6 of patients in two relevant series
[3,10]. On the other hand, the criteria for evaluating the course of JME
differ and are quite complex; thus, the findings are difficult to compare.
Baykan et al. labeled the patients as having a “benign course” if they
had no generalized tonic-clonic seizures (GTCS) and less than 2 myo-
clonic seizures monthly. Resistance was defined as having one or
more GTCS or clonic-tonic-clonic seizures per year, or more than
one episode of myoclonus monthly despite treatment with valproate
(VPA) or other appropriate AED for this syndrome with therapeutic
drug levels for the last 5 years. Lamotrigine (LTG), in some cases,
levetiracetam (LEV), topiramate, clonazepam and phenobarbital
(the last 2 drugs were mostly used in the past), and their combina-
tions with VPA were cited as appropriate choices in JME [3].
Those patients having seizures due to problems in lifestyle, inap-
propriate use of seizure-aggravating drugs, or being noncompliant
could be classified as having a “pseudo-resistant course”. Gelisse
et al. reported in their pioneering study that among 155 consecutive
JME patients followed-up for at least 1 year (13.5 ± 9.9 years), there
were 15 (9.7%) patients with pseudo-resistant seizures (due to lack
of compliance (eight), insufficient treatment (three), and abnormal
lifestyle (four)) [10]. Frequent counseling of these patients about
seizure-aggravating factors is helpful, and appropriate psychiatric
management is important to improve their seizure control.
3.1. The reported clinical risk factors for poor outcome of JME
3.1.1. The presence of all three seizure types of JME
The coexistence of myoclonic, absence, and GTCS is consistently
reported to be a risk factor for AED resistance in JME [3,6,10,11], where-
as myoclonus alone [3,10,12] or a combination of absence seizures and
myoclonus is not [3,10,13]. In other words, none of the patients with
treatment-resistant epilepsies had myoclonia as the only seizure type
or a combination of absence and myoclonic seizures [10]. A combination
of GTCS on awakening related to rare myoclonic seizures was also
reported to have a benign course suggesting that the prognosis was
already determined by the pathophysiology at the beginning of the
treatment [13].
3.1.2. The presence of psychiatric problems
In addition to being an important factor for pseudo-resistance, psy-
chiatric comorbidity is a risk factor for true drug resistance [3,10,11]. Per-
sonality disorders and depression are also frequent psychiatric findings,
emphasizing the need for a psychiatric evaluation when appropriate of
patients with JME, as discussed elsewhere in this supplement [14,15].
3.1.3. The role of systemic disorders
Systemic disorders such as thyroid diseases [16,17] are also im-
portant for AED resistance in JME as well as for fluctuations in seizure
control in a patient with otherwise well-controlled seizures [3].
3.1.4. Delay in the diagnosis
The first report about patients with drug-resistant JME [18] included
12 patients with long duration of epilepsy during which the diagnosis
and appropriate treatment were delayed together with a high percentage
of asymmetries or focal discharges on EEG. However, it is controversial
whether delayed diagnosis is a real risk factor of resistance [3,7,19,20].
3.1.5. The coexistence of JME with focal epilepsy
There are intriguing reports showing overlaps of JME with idio-
pathic photosensitive occipital lobe epilepsy or temporal lobe epilep-
sy [3,21–23]. This may be responsible for unexpected fluctuations in
seizure control and create confusion in treatment decisions. There
are no controlled series on this issue, only case reports and family
studies. For example, a single patient from our series initially had
well-controlled temporal lobe epilepsy with typical clinical features
and temporal lobe spikes. Her seizures were no longer controlled
with VPA and better seizure control was achieved with carbamaze-
pine (CBZ) [3]. In addition, there are reports showing that temporal
lobe epilepsy developed first, followed by JME after epilepsy surgery
[22,23]. Interestingly, these peculiar cases suggest that the phenotype
may not be a merged syndrome, but the two conditions can retain
their inherent electroclinical profile, responsiveness to treatment, and
prognosis [23]. Taylor et al. described four families with phenotypic
overlap between JME and idiopathic photosensitive occipital lobe epi-
lepsy; half of the affected individuals in families with visual aura had
myoclonic seizures [21]. They reported that visual aura and conscious
head version are under-recognized features of JME, particularly among
photosensitive patients [21]. These findings could be explained by
shared genetic determinants of these disorders and must account for
the striking female predominance, variable phenotypes associated with
photosensitivity, and the overlap of clinical features classically regarded
as distinguishing between focal and generalized syndromes [21].
3.1.6. Treatment failure
The main reason for treating patients with JME with drugs other
than VPA is the occurrence of relatively frequent side effects such as
weight gain, tremor, and loss of hair, as well as its risk for teratogen-
esis. It is well appreciated that patients inappropriately treated with
phenytoin (PHT), CBZ, or other drugs suitable for focal epilepsy
should not be considered as having drug-resistant JME. Using ade-
quate drugs can dramatically improve seizure control in those pa-
tients with JME. The mechanism of striking deterioration observed
in some JME patients taking PHT or CBZ has not been explained yet
[24]. Gelisse et al. reported that 40 out of 155 JME patients received
CBZ or PHT, leading to an aggravation of seizures, consisting mostly
of an increase of myoclonic jerks or absences in 68% and in 38%, an
improvement in 14% and 12%, and no change in 18% and 50%, after re-
ferral to a tertiary center [10]. Following a change of treatment, all pa-
tients who had shown seizure aggravation clearly improved [10].
Also, in Baykan et al.'s study, 60.4% of patients with JME had received
PHT or CBZ with an aggravation of myoclonic seizures in 44.8%, an im-
provement in 37.9%, and no change in 17.3% [3]. Patients were often
unwilling to transition from CBZ or PHT to VPA if they were doing
well clinically. In fact, the usefulness of these AEDs in selected JME
as add-on treatment has been reported, especially for GTCS [25].
The possible aggravation of myoclonic jerks by LTG is still controver-
sial [26,27]. Thus, despite recent advances, much remains to be dis-
covered about the treatment of this common epilepsy syndrome
and careful clinical follow-up is still needed.
3.1.7. Miscellaneous
Atypical seizure characteristics including auras and post-ictal con-
fusion as well as cognitive dysfunction were also cited as characteris-
tics of drug-resistant JME in a small group of 33 patients [28]. Poor
prognosis was reported to be associated with longer epilepsy dura-
tion and younger age at epilepsy onset in a series of 65 JME patients
 B. Baykan et al. / Epilepsy & Behavior 28 (2013) S18–S24 S21

[11], but not confirmed by others in long-term follow-up studies Table 1

[3,10]. Family history of epilepsy and sex did not seem to influence Juvenile myoclonic epilepsy (JME) subsyndromes and phenotype in long-term follow-up.a

the prognosis [3,10]. Phenotype Classic JME CAE/JME JME/apA JME/astatic

n = 222 n (%) n (%) n (%) n (%)
3.2. The proposed relationships of EEG findings with the prognosis of JME MS only 10 (6%) 0 0 0
MS + Abs 1 (1%) 1 (3%) 3 (17%) 0
There have been many proposed associations found or excluded MS + GTC 96 (59%) 0 0 0
MS + Abs + GTC 54 (34%) 34 (97%) 15 (83%) 0
between EEG findings and later prognosis of JME. These include the
MS + As+ GTC 0 0 0 7 (87%)
following: MS + Abs + As + GTC 0 0 0 1 (13%)

1) In a retrospective evaluation with a seven-year follow-up, the early
normalization of the first diagnostic EEG pattern after successful
treatment indicated a good prognosis of JME; however, this could
not be confirmed in later studies [3,4].
2) Focal clinical and/or EEG features in addition to typical generalized
spike–waves have been consistently reported in patients with JME,
creating diagnostic confusion. [3,29–36]. Some reports suggest that
focal findings are frequent in JME patients with a treatment-
resistant course [13,28]; however, other studies could not confirm
this prognostic association [3,11,37].
3) Photoparoxysmal response has not been found to have an associa-
tion with poor prognosis [3,10]. A cases series from India reported
that seizures in JME patients with photoparoxysmal response on
EEG responded very well to VPA alone [37].
4) Aggravation of generalized spike–wave discharges during hyper-
ventilation on EEG did not seem to affect the prognosis for seizure
control [3,4].
5) Eye closure-related epileptiform EEG discharges (eye closure sensi-
tivity) in children and adults with absence epilepsy favor the diag-
nosis of eyelid myoclonia with absences, which has a relatively
poor prognosis among the genetic generalized epilepsies [38–40].
It has been shown that eye closure sensitivity on the EEG may also
be present in a subset of JME patients [41–43]. In these first reports,
the effect of eye closure sensitivity on prognosis was not negative
[41–43], but a recent report suggested that JME patients with eye
closure-induced seizures did not become seizure-free [11].
6) The presence of reflex traits could represent an aggravating feature
in JME and might have a negative impact on prognosis. Compared
to seizure-free patients, those with persistent seizures presented a
higher incidence of EEG sensitivity to praxis and language tasks
[11]. Matsuoka reported that JME patients sensitive to neuropsycho-
logical EEG activations at the beginning of treatment had an unfavor-
able outcome [13]. In addition, there was a correlation between
disappearance of reflex EEG activation and improvement in seizure
control [11].
7) The presence of epileptic discharges and recorded clinical seizures
on the EEG at the time of initial diagnosis may indicate a higher
epilepsy severity. This finding was noted in a recent study with
3 years of follow-up [11].
3.3. Other factors and prognosis of JME
Routine neuroimaging procedures are not useful in patients with
typical JME; in one study, abnormal findings, which were present in
12% of the JME cases, did not influence therapeutic decisions or prog-
nosis [44]. In addition, a recent small study supported the view that
the prognosis of patients with MRI abnormalities was poor [45], but
the presence of minor abnormalities on conventional neuroimaging
was not found to be associated with drug resistance [3,28]. Advanced
neuroimaging studies need to address phenotypic variations such as
response to therapy in JME [46], which would likely uncover prognos-
tic factors.
Genetic studies suggest a polygenetic mode of inheritance, and JME
is closely related to the syndrome of juvenile absences and the syn-
drome of pure grand mal on awakening [47]. The complete genetic
pathophysiology and underlying mutations of JME are currently far
CAE/JME = childhood absence epilepsy evolving to JME, JME/apA = JME with adolescent
onset pyknoleptic absence, JME/astatic = JME with astatic seizures, MS = myoclonic sei-
zures, Abs = absence seizures depending on the syndrome, could be pyknoleptic, GTC =
generalized tonic-clonic or clonic-tonic-clonic seizures, As = astatic seizures.
a
Reference [6,49].
from explained. Future studies will hopefully elucidate the potential
genetic mechanisms underlying drug resistance in JME.
4. Are there subsyndromes of JME which influence prognosis?
The GENESS (Genetic Epilepsy Studies International Consortium)
described a sample of 257 families with four subsyndromes of JME [6].
Evidence of the subsyndromes in JME was based on electroclinical find-
ings including the following: age at onset of first seizure, seizure types
(phenotype), family history of epilepsy considering phenotype in affect-
ed relatives, and interictal EEG findings (Table 1). Long-term follow-up
(1 to 52 years) and response to AED therapy were also determined in
222 (86%) of patients [6,48,49] (Table 2).
The most common subsyndrome of JME was classic JME, which cor-
responded to 73% (n= 186) of the GENESS cases and which resembles
the original descriptions of JME [50–52]. The age of seizure onset in clas-
sic JME was 7 to 28 years, with a peak at 15 years old, and no clear gen-
der preponderance was seen (1.2 females/1male). Myoclonia affected
upper extremities symmetrically or asymmetrically and were mainly
on awakening. Generalized tonic-clonic seizures or clonic-tonic-clonic
seizures were present in most patients, whereas absence seizures
were seen in only 33%. The interictal EEG showed 4- to 6-Hz spike–
wave or polyspike–wave complexes. Epilepsy was seen in 49% of family
members, and JME was the most frequent epilepsy syndrome found
among relatives of patients with classic JME. Myoclonic, GTCS, and ab-
sence seizures stopped in 58% of patients with AEDs and 82% were
free of GTCS with AEDs. Eight patients were seizure-free from one up
to eleven years without taking AEDs. Those who had more seizure
types were less responsive to AED treatment.
A second common form of JME, comprising 17% (n= 45) of the
GENESS series, was childhood absence epilepsy (CAE) evolving to JME
(CAE/JME), as described by Moore et al. and Wirrell et al. [53,54]. Ab-
sence seizures were the first to appear, starting at a mean age of
6.9 years (range 1–10 years), followed by GTCS and myoclonic seizures,
developing during adolescence. Childhood absence epilepsy/juvenile
myoclonic epilepsy had a female preponderance (1.8 females:1 male)
Table 2
Juvenile myoclonic epilepsy (JME) subsyndromes and response to treatment in long-term
follow-up.a
Response to treatment Classic JME CAE/JME JME/apA JME/astatic
n = 222 n (%) n (%) n (%) n (%)
All seizure types controlled 97 (60%) 3 (7%) 10 (56%) 6 (63%)
GTC controlled but persisting 132 (84%) 26 (74%) 17 (94%) 7 (75%)
MS and/or Abs and/or As
Persisting seizures 64 (40%) 32 (93%) 8 (44%) 2 (37%)
CAE/JME = childhood absence epilepsy evolving to JME, JME/apA = JME with adoles-
cent onset pyknoleptic absence, JME/astatic = JME with astatic seizures.
a
Modified from [49].
S22 B. Baykan et al. / Epilepsy & Behavior 28 (2013) S18–S24
with more maternal transmission [55]. All patients had 3-Hz spike and
wave and CAE/JME had the higher photoparoxysmal response of all JME
subsyndromes (22%). Family history of epilepsy was positive in 71%
presenting with absence epilepsy. Only three patients were free from
all seizures on AEDs and 23 patients were free of GTCS.
The third subsyndrome was JME with adolescent onset pyknoleptic
absence (JME/apA) and accounted for 7% (n= 18) of all cases. The pres-
ence of pyknoleptic absence was defined as having absence seizures “at
least once a day and up to hundreds per day”, accompanied by GTCS
and/or myoclonus. The JME/apA typically started at 16 years of age
(range 11–32 years) with a high female preponderance (2.6 females:1
male). Interictal EEG showed 4- to 6-Hz spike–wave and polyspike–
wave complexes mixed with 3-Hz spike and wave complexes.
Sixty-one percent had a positive family history of epilepsy, with JME
as the most frequent phenotype of affected family members. Ten pa-
tients were completely seizure-free on AEDs and seven were free of
GTCS with AEDs.
The fourth variant was JME with astatic seizures or JME/astatic,
accounting for 3% (n = 8) of all cases. The presence of astatic sei-
zures was defined as “patient fell limp to the ground like a rag
doll” [6] and included myoclonic seizures that may also have been
complicated by either GTCS or rarely absence seizures. The age of
onset was 8 to 19 years (mean 14 years). Interictal EEG consisted
of 4- to 6-Hz polyspike and slow wave complexes. Three patients
had a history of epilepsy in the family with JME in the affected rel-
atives. Six patients became free of all seizure types with AEDs. One
patient persisted with astatic seizures and one patient persisted
with GTCS.
Only 8 of 161 (5%) GENESS patients with classic JME became
seizure free off medication, indicating that classic JME is chronic, if
not, lifelong in duration. Among the GENESS patients with CAE/JME,
only 3 of 35 (93%) achieved complete seizure control with medica-
tion. It seems that CAE/JME also is a chronic, if not a lifelong
subsyndrome, even with AED treatment. Because of the small sample
size, it is not possible to state that JME/apA and JME/astatic are life-
long in duration; however, no patient has shown remission of sei-
zures without AED treatment [6,48].
5. The prognosis of individual seizure types of JME
Juvenile myoclonic epilepsy has several seizure patterns, with the
major characteristic being myoclonic seizures [47,56,57]. Generalized
tonic-clonic seizures often occur, and some individuals also have ab-
sence seizures (Fig. 1) [58], but myoclonia may remain the only sei-
zure type.
The prognosis of absence seizures remained obscure even after
close follow-up in some cases, due to their short duration and mini-
mal effects on quality of life [3]. In 2 JME patients with a follow-up
duration of 20 years, myoclonic seizures decreased under the same
drug regimen after 30 years of age [59]. Baykan et al. showed a sub-
stantial alleviation or remission of the myoclonic seizures in the
fourth decade. This tendency is probably unrelated to the AED treat-
ment or to the lifestyle changes but it is not possible to exclude the
long-term effects of the AED treatment and changes in lifestyle
when getting older. The unknown pathophysiology of the cortical
myoclonus of JME seemed to improve with time in at least some of
the patients though persisting for a long time. There is a marked over-
lap in the mean ages at remission or alleviation of myoclonia (32.9 ±
9.6 versus 31.3 ± 8.4 years) [3]. Myoclonic seizures are important
symptoms, causing impairments and restrictions of social life for
JME patients at least in the beginning. Thus, patients should be ad-
vised that severe myoclonic seizures during adolescence will be
much better in time and they will have a chance of complete seizure
control.
Likewise, myoclonia progressed only during the first 5 years, and
no worsening of myoclonia was seen beyond the initial 10 to 15
years of progressive myoclonic epilepsy of the Unverricht–Lundborg
type [60]. Even though JME and this form of progressive myoclonic
epilepsy have no similarities regarding pathogenesis and course, it
is interesting to note that both forms of myoclonic epilepsies have
similarities in the alleviation of myoclonus with advancing age.
6. JME in “old” age
Observations in the epilepsy outpatient clinics suggested that JME
patients older than 40 years of age are rare. There are a few anecdotal
case reports of JME in old age [61,62]. Although rare, idiopathic myo-
clonic epilepsy could remain asymptomatic for decades and can pres-
ent in the elderly. Proper classification of this epileptic syndrome,
even in the elderly, is essential in view of the response to appropriate
antiepileptic therapy [63]. There were 22 patients older than 40 years
at the final evaluation in Baykan et al.'s study; 11 were seizure-free
for 5 years. Only 2 patients (9.1%) had complete remission without
AED treatment for a mean of 5 years. The remaining patients experi-
enced milder myoclonic seizures (6 pts), GTCS and myoclonic seizures
(3 pts), GTCS and absence seizures (1 pt), and all three types of seizures
(1 pt).
7. JME and status epilepticus over the lifetime
Different types of status epilepticus (SE) including predominantly
nonconvulsive SE and myoclonic SE can be seen in patients with JME.
Convulsive SE was detected in 8 out of 23 (35%) patients in the
population-based follow-up. One occurred at onset as the first recog-
nized seizure and the remainder between 1 and 25 years after the ini-
tial diagnosis [2].
Little is known about the subtype and frequency of nonconvulsive
SE (NCSE) in JME, except for occasional case reports. A retrospective
study of 69 JME patients identified 3 patients with typical absence
status epilepticus (ASE). All were women and one later experienced
recurrent SE [64]. The estimated prevalence of NCSE in JME is 5.8%
with an annual incidence of 1.2%. This corresponds to the results of
Agathonikou et al. [65], who diagnosed ASE in 2 of 30 adult JME pa-
tients (6.7%). Tomson et al. used a different approach and analyzed
the frequency of different epileptic syndromes in a large series of
NCSE in adults using a retrospective study design. Five of 32 patients
had a history of absence epilepsy in this series, but only one patient
had JME, thus suggesting that NCSE in patients with JME is rare
[66]. Baykan et al. reported 2 JME cases with more than 2 events of
ASE within a period of 15 years [67]. The EEGs during ASE of these
2 JME patients with recurrent ASE had marked similarities. Rapid gen-
eralized spike (6 Hz) bursts associated with generalized myoclonus,
followed by less prominent delta waves and frequent interruptions
lasting 1–4 s during the ASE were observed. Although these 2 patients
showed a highly similar EEG pattern of ASE, their clinical courses
were different regarding their response to AED therapy [67].
Myoclonic seizures frequently present as repeated jerks lasting for
minutes with or without transition to a GTCS. Very rarely, they last
more than 30 min, fulfilling the formal criteria for myoclonic status
epilepticus (MSE) [68], also termed “impulsive-petit mal status” in
the original descriptions. Available literature about the presence of
MSE in JME is limited to single case reports or small series [69–73].
In a retrospective hospital-based study, seven (3%) of 247 patients
(5 women and 2 men) had a history of MSE with an incidence of
3.2/1000 patient years [74]. Previous studies on MSE in patients
with JME reported a wide variance, from 1.4% to 42% [1,64].
Larch et al. distinguished three types of MSE [74]. In the first type,
patients with bilateral myoclonic jerks synchronous with generalized
polyspike–wave discharges on EEG and without impairment of con-
sciousness are designated as having impulsive-petit mal SE’ or ‘typical
MSE’ [64,75]. In the second type, MSE may be preceded, followed
or interrupted by a GTCS. The consciousness of these patients is
 B. Baykan et al. / Epilepsy & Behavior 28 (2013) S18–S24
generally impaired after GTCS. A routine EEG can detect the ictal na-
ture and govern parenteral AED treatment of SE. Absence status
epilepticus with superimposed myoclonic jerks is the third type of
MSE. Eyelid jerks are prominent with variable involvement of the
upper extremities. Consciousness is moderately impaired in these
patients, and myoclonic jerks are rarely time-locked with polyspike–
wave discharges on EEG [75]. A combination of type 2 and type 3 of
MSE in JME can also be observed [73,75]. The boundaries between
MSE and myoclonic absence status as well as their impact on prognosis
of JME are not well delineated and the combination may simply repre-
sent a biological continuum [74].
The occurrence of both MSE and ASE in patients with JME is clearly
associated with triggering factors such as AED withdrawal, inappropri-
ate treatment with CBZ and PHT, or sleep deprivation. Larch et al. found
that 2 of 7 patients with MSE were treated with either CBZ or PHT [74].
In other retrospective series, 19 out of 28 (68%) patients with JME who
were treated with CBZ as well as 6 out of 16 (38%) who were treated
with PHT experienced seizure aggravation, resulting in MSE in 2 pa-
tients [24]. Another retrospective case series reported 14 patients
with ASE or MSE, often with atypical features, among them 4 JME
patients who were treated with CBZ and LTG [75]. In another small
series, 3 patients developed ASE with mild myoclonic components
after replacement of VPA with LTG and following reinstitution of VPA;
the clinical course was favorable [76].
Both ASE and MSE seem to respond well to the administration of
benzodiazepines such as diazepam and, preferentially, clonazepam
[66,67]. In one study, MSE was easily controlled in all patients with
low doses of intravenous benzodiazepines and without any psychiat-
ric or cognitive sequalae [74]. Intravenous VPA or LEV are other treat-
ment options in MSE [77].
8. Conclusions
Identifying JME patients prospectively who will have remission of
their epilepsy and be able to discontinue AED treatment is a goal for fur-
ther study. Juvenile myoclonic epilepsy is generally an easily controlled
epilepsy syndrome, but clinicians should remember the fact that sei-
zures in a small proportion of patients with JME pose therapeutic prob-
lems before informing patients with newly-diagnosed JME that their
prognosis is “benign”. Most patients benefit from a long duration of
AED treatment accompanied by counseling about triggering factors.
Status epilepticus in JME, including ASE and MSE, is a rare event related
most often to inappropriate drug treatment and easily controlled by
benzodiazepines. Although it is often unwise to discontinue treatment
once seizure control has been achieved, severity of myoclonic seizures
diminishes in the 4th decade. Despite the favorable seizure outcome
in most cases, 75% of JME patients have at least one major unfavorable
social outcome such as failure to complete high school, unplanned preg-
nancy, depression, unemployment, or living alone. Thus, attention to
these social factors is indicated. Further work is needed to elucidate
the genetic background of JME and associated prognostic markers as
well as pathophysiological and neuroimaging correlates.
Conflict of interest
The authors declare that there are no conflicts of interest.
Acknowledgments
B. Baykan thanks her colleagues and EEG technicians in the rele-
vant studies on the prognosis of JME for their contributions and par-
ticularly thanks her mentors Prof. Dr. Ahmet Caliskan and Prof. Dr.
Aysen Gokyigit. I.E. Martínez-Juárez acknowledges the participation
of the GENESS consortium, particularly A.V. Delgado-Escueta, M.E.
Alonso, M.T. Medina, A. Ochoa, A. Jara-Prado, and R.M. Durón.
S23
References
[1] Panayiotopoulos CP, Obeid T, Tahan AR. Juvenile myoclonic epilepsy: a 5-year
prospective study. Epilepsia 1994;35(2):285-96.
[2] Camfield CS, Camfield PR. Juvenile myoclonic epilepsy 25 years after seizure
onset: a population-based study. Neurology 2009;73(13):1041-5.
[3] Baykan B, Altindag EA, Bebek N, et al. Myoclonic seizures subside in the fourth de-
cade in juvenile myoclonic epilepsy. Neurology 2008;70(22):2123-9.
[4] Öztürk A, Baykan B, Gürses C, Gökyigit A. Long-term follow-up of juvenile myo-
clonic epilepsy. Archives of Neuropsychiatry (Noropsikiyatri Arşivi) 1999;36(3):
123-7.
[5] Baykan-Kurt B, Gökyigit A, Çaliskan A. Juvenile myoclonic epilepsy. Archives of
Neuropsychiatry (Noropsikiyatri Arşivi) 1991;28(1):41-8.
[6] Martinez-Juarez IE, Alonso ME, Medina MT, et al. Juvenile myoclonic epilepsy
subsyndromes: family studies and long-term follow-up. Brain 2006;129(5):1269-80.
[7] Siren A, Eriksson K, Jalava H, et al. Idiopathic generalised epilepsies with 3 Hz and
faster spike wave discharge: a population-based study with evaluation and
long-term follow-up in 71 patients. Epileptic Disord 2002;4:209-16.
[8] Geerts A, Arts WF, Stroink H, et al. Course and outcome of childhood epilepsy: a
15-year follow-up of the Dutch Study of Epilepsy in Childhood. Epilepsia 2010;51:
1189-97.
[9] Loddenkemper T, Benbadis SR, Serrasota JM, Berkovic SF. Idiopathic generalized
epilepsy syndromes of childhood and adolescence. In: Wyllie E, editor. The treat-
ment of epilepsy. Principles and practice. Philadelphia: Lippincott Williams &
Wilkins; 2006. p. 296.
[10] Gelisse P, Genton P, Thomas P, Rey M, Samuelian JC, Dravet C. Clinical factors of drug
resistance in juvenile myoclonic epilepsy. J Neurol Neurosurg Psychiatry 2001;70(2):
240-3.
[11] Guaranha MS, Filho GM, Lin K, Guilhoto LM, Caboclo LO, Yacubian EM. Prognosis of
juvenile myoclonic epilepsy is related to endophenotypes. Seizure 2011;20(1):42-8.
[12] Jain S, Padma MV, Maheshwari MC. Occurrence of only myoclonic jerks in juvenile
myoclonic epilepsy. Acta Neurol Scand 1997;95(5):263-7.
[13] Matsuoka H. The seizure prognosis of juvenile myoclonic epilepsy. Jpn J Psychiatry
Neurol 1992;46(2):293-6.
[14] Moschetta S, Fiore LA, Fuentes D, Gois J, Valente KD. Personality traits in patients
with juvenile myoclonic epilepsy. Epilepsy Behav 2011;21(4):473-7.
[15] de Araújo Filho GM, Pascalicchio TF, Sousa Pda S, Lin K, Ferreira Guilhoto LM,
Yacubian EM. Psychiatric disorders in juvenile myoclonic epilepsy: a controlled
study of 100 patients. Epilepsy Behav 2007;10(3):437-41.
[16] Su YH, Izumi T, Kitsu M, Fukuyama Y. Seizure threshold in juvenile myoclonic ep-
ilepsy with Graves disease. Epilepsia 1993;34(3):488-92.
[17] Obeid T, Awada A, al Rajeh S, Chaballout A. Thyroxine exacerbates absence seizures
in juvenile myoclonic epilepsy. Neurology 1996;47(2):605-6.
[18] Dasheiff RM, Ritaccio AL. Characterization of intractable juvenile myoclonic epilepsy:
new perspectives on primarily generalized seizures. Seizure 1993;2(1):11-9.
[19] Atakli D, Sözüer D, Atay T, Baybas S, Arpaci B. Misdiagnosis and treatment in juve-
nile myoclonic epilepsy. Seizure 1998;7(1):63-6.
[20] Grünewald RA, Chroni E, Panayiotopoulos CP. Delayed diagnosis of juvenile myo-
clonic epilepsy. J Neurol Neurosurg Psychiatry 1992;55(6):497-9.
[21] Taylor I, Marini C, Johnson MR, Tuner S, Berkovic SF, Scheffer IE. Juvenile myoclonic
epilepsy and idiopathic photosensitive occipital lobe epilepsy: is there overlap?
Brain 2004;127(8):1878-86.
[22] Diehl B, Wyllie E, Rothner D, Bingaman W. Worsening seizures after surgery for focal
epilepsy due to emergence of primary generalized epilepsy. Neurology 1998;51(4):
1178-80.
[23] Koutroumanidis M, Hennessy MJ, Elwes RD, Binnie CD, Polkey CE. Coexistence of
temporal lobe and idiopathic generalized epilepsies. Neurology 1999;53(3):490-5.
[24] Genton P, Gelisse P, Thomas P, Dravet C. Do carbamazepine and phenytoin aggra-
vate juvenile myoclonic epilepsy? Neurology 2000;55(8):1106-9.
[25] Knott C, Panayiotopoulos CP. Carbamazepine in the treatment of generalised tonic
clonic seizures in juvenile myoclonic epilepsy. J Neurol Neurosurg Psychiatry
1994;57(4):503.
[26] Carrazana EJ, Wheeler SD. Exacerbation of juvenile myoclonic epilepsy with
lamotrigine. Neurology 2001;56(10):1424-5.
[27] Bodenstein-Sachar H, Gandelman-Marton R, Ben-Zeev B, Chapman J, Blatt I. Out-
come of lamotrigine treatment in juvenile myoclonic epilepsy. Acta Neurol Scand
2011;124(1):22-7.
[28] Fernando-Dongas MC, Radtke RA, VanLandingham KE, Husain AM. Characteristics of
valproic acid resistant juvenile myoclonic epilepsy. Seizure 2000;9(6):385-8.
[29] Usui N, Kotagal P, Matsumoto R, Kellinghaus C, Lüders HO. Focal semiologic and
electroencephalographic features in patients with juvenile myoclonic epilepsy.
Epilepsia 2005;46(10):1668-76.
[30] Aliberti V, Grünewald RA, Panayiotopoulos CP, Chroni E. Focal electroencephalo-
graphic abnormalities in juvenile myoclonic epilepsy. Epilepsia 1994;35(2):297-301.
[31] Lancman ME, Asconape JJ, Penry JK. Clinical and EEG asymmetries in juvenile
myoclonic epilepsy. Epilepsia 1994;35(2):302-6.
[32] Lancman ME, Asconape JJ, Golimstok A. Circling seizures in a case of juvenile myo-
clonic epilepsy. Epilepsia 1994;35(2):317-8.
[33] Panayiotopoulos CP, Tahan R, Obeid T. Juvenile myoclonic epilepsy: factors of
error involved in the diagnosis and treatment. Epilepsia 1991;32(5):672-6.
[34] Montalenti E, Imperiale D, Rovera A, Bergamasco B, Benna P. Clinical features, EEG
findings and diagnostic pitfalls in juvenile myoclonic epilepsy: a series of 63 patients.
J Neurol Sci 2001;184(1):65-70.
[35] Topcuoglu MA, Saygi S, Ciger A. Rotatory seizures in juvenile myoclonic epilepsy.
Clin Neurol Neurosurg 1997;99(4):248-51.
S24 B. Baykan et al. / Epilepsy & Behavior 28 (2013) S18–S24
[36] So GM, Thiele EA, Sanger T, Schmid R, Riviello Jr JJ. Electroencephalogram and clinical
focalities in juvenile myoclonic epilepsy. J Child Neurol 1998;13(11):541-5.
[37] Jain S, Tripathi M, Srivastava AK, Narula A. Phenotypic analysis of juvenile myo-
clonic epilepsy in Indian families. Acta Neurol Scand 2003;107:356-62.
[38] Appleton RE, Panayiotopoulos CP, Acomb BA, Beirne M. Eyelid myoclonia with typi-
cal absences: an epilepsy syndrome. J Neurol Neurosurg Psychiatry 1993;56(12):
1312-6.
[39] Giannakodimos S, Panayiotopoulos CP. Eyelid myoclonia with absences in adults:
a clinical and video-EEG study. Epilepsia 1996;37(1):36-44.
[40] Striano S, Capovilla G, Sofia V, et al. Eyelid myoclonia with absences (Jeavons
syndrome): a well-defined idiopathic generalized epilepsy syndrome or a spectrum
of photosensitive conditions? Epilepsia 2009;50(Suppl. 5):15-9.
[41] Baykan-Kurt B, Gökyiğit A, Parman Y, Kinay D, Gürses C. Eye closure related spike
and wave discharges: clinical and syndromic associations. Clin Electroencephalogr
1999;30(3):106-10.
[42] Sevgi EB, Saygi S, Ciger A. Eye closure sensitivity and epileptic syndromes: a ret-
rospective study of 26 adult cases. Seizure 2007;16(1):17-21.
[43] Destina Yalçin A, Forta H, Kiliç E. Overlap cases of eyelid myoclonia with absences
and juvenile myoclonic epilepsy. Seizure 2006;15(6):359-65.
[44] Gelisse P, Genton P, Raybaud C, Thomas P, Dravet C. Structural brain lesions do not in-
fluence the prognosis of juvenile myoclonic epilepsy. Acta Neurol Scand 2000;102(3):
188-91.
[45] Aslan K, Bozdemir H, Yapar Z, Burgut R. The effect of electrophysiological and neu-
roimaging findings on the prognosis of juvenile myoclonic epilepsy proband.
Neurol Res 2010;32(6):620-4.
[46] Anderson J, Hamandi K. Understanding juvenile myoclonic epilepsy: contributions
from neuroimaging. Epilepsy Res 2011;94:127-37.
[47] Janz D. Epilepsy with impulsive petit mal (juvenile myoclonic epilepsy). Acta
Neurol Scand 1985;72(5):449-59.
[48] Alonso ME, Medina MT, Martínez-Juárez IE, et al. Familial juvenile myoclonic ep-
ilepsy. Adv Neurol 2005;95:227-43.
[49] Medina MT, Martínez-Juárez IE, Durón RM, et al. Treatment of myoclonic epilep-
sies of childhood, adolescence and adulthood. Adv Neurol 2005;95:307-23.
[50] Herpin T. Des acces incomplets d'epilepsie. Paris: Bailliere; 1867.
[51] Rabot L. De la myoclonie e'pileptique. Medical thesis. Paris; 1899.
[52] Janz D, Christian W. Impulsive-petit mal. Dtsch Z Nervenheilkd 1957;176:344-86.
[53] Wirrell EC, Camfield PR, Camfield PR, Gordon KE, Dooley JM. Long-term prognosis
of typical childhood absence epilepsy: remission or progression to juvenile myo-
clonic epilepsy. Neurology 1996;47:912-8.
[54] Moore T, Hecquet S, McLellann A, et al. Polymorphism analysis of JRK/JH8, the
human homologue of mouse jerky, and description of a rare mutation in a case
of CAE evolving to JME. Epilepsy Res 2001;46:157-67.
[55] Durón RM, Medina MT, Martínez-Juárez IE, et al. Seizures of idiopathic general-
ized epilepsies. Epilepsia 2005;46(Suppl. 9):34-47.
[56] Asconape J, Penry JK. Some clinical and EEG aspects of benign juvenile myoclonic
epilepsy. Epilepsia 1984;25(1):108-14.
[57] Delgado-Escueta AV, Enrile-Bacsal F. Juvenile myoclonic epilepsy of Janz. Neurology
1984;34(3):285-94.
[58] Panayiotopoulos CP, Obeid T, Waheed G. Absences in juvenile myoclonic epilepsy:
a clinical and video-electroencephalographic study. Ann Neurol 1989;25(4):
391-7.
[59] Matsuoka H, Takahashi T, Sasaki M, Yoshida S, Numachi Y, Sato M. The long-term
course of seizure susceptibility in two patients with juvenile myoclonic epilepsy.
Seizure 2002;11(2):126-30.
[60] Magaudda A, Ferlazzo E, Nguyen VH, Genton P. Unverricht–Lundborg disease, a con-
dition with self-limited progression: long-term follow-up of 20 patients. Epilepsia
2006;47(5):860-6.
[61] Gram L, Alving J, Sagild JC, Dam M. Juvenile myoclonic epilepsy in unexpected age
groups. Epilepsy Res 1988;2(2):137-40.
[62] Grünewald RA, Panayiotopoulos CP. Diagnosing juvenile myoclonic epilepsy in an
elderly patient. Seizure 1994;3(3):239-41.
[63] Jacob S, Martin D, Rajabally YA. Juvenile myoclonic epilepsy in an elderly patient.
Age Ageing 2006;35(2):194-6.
[64] Dziewas R, Kellinghaus C, Lüdemann P. Nonconvulsive status epilepticus in patients
with juvenile myoclonic epilepsy: types and frequencies. Seizure 2002;11:335-9.
[65] Agathonikou A, Panayiotopoulos CP, Giannakodimos S, Koutroumanidis M. Typi-
cal absence status in adults: diagnostic and syndromic considerations. Epilepsia
1998;39:1265-76.
[66] Tomson T, Lindborn U, Nilsson BY. Nonconvulsive status epilepticus in adults:
thirty-two consecutive patients from a general hospital population. Epilepsia
1992;33:829-35.
[67] Baykan B, Gökyigit A, Gurses C, Eraksoy M. Recurrent absence status epilepticus:
clinical and EEG characteristics. Seizure 2002;11:310-9.
[68] Walker M, Cross H, Smith S, et al. Nonconvulsive status epilepticus: Epilepsy
Research Foundation Workshop Reports. Epileptic Disord 2005;7:253-96.
[69] Badhwar A, Siren A, Andermann E, Andermann F. Myoclonic status epilepticus:
video presentation. Mov Disord 2002;17:409-11.
[70] Terzano MG, Gemignani F, Mancia D. Petit mal status with myoclonus: case re-
port. Epilepsia 1978;19:385-92.
[71] Grüneberg F, Helmchen E. Impulsive petit mal status and paranoid psychosis.
Nervenarzt 1969;40:381-5.
[72] van Leeuwen SW, Jennekens F, Sterk CE. A case of petit mal status with myoclonus.
Epilepsia 1969;10:407-14.
[73] Schneeman N, Brune F, Busch H. Impulsive petit mal and twilight state, an unusu-
al combination of epileptic phenomena. Schweiz Arch Neurol Neurochir Psychiatr
1969;105:281-92.
[74] Larch J, Unterberger I, Bauer G, Reichsoellner J, Kuchukhidze G, Trinka E. Myoclon-
ic status epilepticus in juvenile myoclonic epilepsy. Epileptic Disord 2009;11:
309-14.
[75] Thomas P, Valton L, Genton P. Absence and myoclonic status epilepticus precipi-
tated by antiepileptic drugs in idiopathic generalized epilepsy. Brain 2006;129:
1281-92.
[76] Trinka E, Dilitz E, Unterberger I, et al. Nonconvulsive status epilepticus after re-
placement of valproate with lamotrigine. J Neurol 2002;249:1417-22.
[77] Trinka E. Valproate and newer antiepileptic drugs for status epilepticus. Epilepsia
2007;48(Suppl. 8):49-51.