ATEP 3750
Dr. Rehberg
JUVENILE MYOCLONIC
EPILEPSY
Ameer Shihadeh
Abstract
Introduction: this study will explain the cause of Juvenile myoclonic
epilepsy (JME) in a female freshman high school basketball player and traits
that indicates JME.
Case presentation: a fourteen year old female basketball player
experiences episodes of seizures months after she was diagnosed with a
concussion which prevented her to play the remainder of the basketball
season.
Results: subject did not experience any seizures after hospitalization and
was prescribed medication to reduce the risk of seizure reoccurrence.
Conclusion: sleep deprivation, stress, and incompliance to taking
medication are major factors that could make the subject relapse and
experience seizures once again. It is important to make lifestyle changes to
reduce episodes of seizures.
Introduction
Juvenile Myoclonic Epilepsy is a syndrome of idiopathic generalized
epilepsy. It is usually found in patients from ages 9-14. Polyspikes readings
from an EEG would indicate whether or not a person has JME. JME is when a
patient experiences a serious of different types of seizures, predominantly
myoclonic jerks and in rare cases, generalized tonic clonic seizures. There
are factors that influence a high prevalence of seizures among patients who
are positive in those factors. Also medication is a key contributor to the
remiss of seizures of any kind if taken regularly.
This study will explain possible outcomes that may have caused sprout
of JME in a female high school freshman basketball player. The factors that
would influence seizures, and what personality traits those who have JME
may possess will be discussed, as well as the importance of medication
throughout the process.
Literature Review
Juvenile Myoclonic Epilepsy (JME) is a syndrome of idiopathic
generalized epilepsy (IGE), and can be found more often in the adolescent
and young adult years in ones life.1 Usually electroencephalography (EEG) is
the method that is used to diagnose a patient with any type of generalized
tonic clonic seizures (GTCS). Abnormal spike-waves or polyspikes and a
normal background is what is being measured.1 Abnormalities consists of
polyspike waves at 4-6 Hz in 30% of the cases with photosensitivity, and up
to 90% after photonic stimulation.2 JME has shown to be more prevalent in
females than in males, and should be genetically observed, for members of
the family may carry a trait of JME.3
JME can be characterized as seizures with single, bilateral or
repetitive, arrhythmic, irregular myoclonic jerks (MJ) In some instances, these
jerks may cause the patients to fall suddenly.4 MJs are spontaneous, brief,
and sudden. They are not associated with any changes of consciousness but
patients may report a lapse in attention or may feel absent for a moment.4
Typically, seizures occur upon awakening or may be due to sleep deprivation.
The precipitating factors that are commonly seen in JME patients is lack of
sleep which has been seen in 84% of the patients, stress, which 70% of
patients have said they have, also alcohol consumption can play a role in JME
in 51% of the patients.4
GTCS are present in 80-90% of JME patients, and are in most cases
medically referred.4 These episodes consist of longer than usual and a cluster
of MJs.4 The frequency and intensity are heightened and gradually dissolve
into the tonic phase of the GTCS episode.4
There is also a psychological aspect in diagnosing JME, some patients
who have JME have a peculiar personality profile and a social
maladjustment.5 Studies have shown that that there have been psychiatric
comorbidity, meaning that there are two chronic conditions the patient
currently obtain, and the psychiatric disorders are more prevalent in patients
who are antiepileptic drug-resistant.5 Other behaviors that have been noted
amongst JME patients are social problems, sleep or wake rhythm, impulsivity,
risk-taking and decision making behaviors. JME patients are considered to
have . . .an engaging, but emotionally unstable [and] fairly immature
personality. . ..5
JME management is usually handle through antiepileptic drugs (AED),
and remission will occur as long as the patients are compliant with taking
their medication. Those who refrain from using AED have a relapse rate of
over 90%.2 Lifelong treatment may be necessary despite the toxicity some of
these medication may cause, but treatment might have to be weighted
against the risk of relapsing and having reoccurring seizures. 2 A long-term
observational study has been conducted on 175 subjects who had JME. 62%
were seizure free for one year and 53% were seizure free for two on AED,
and the outcome of patients who experienced no episodes of any seizures
without the use AEDs for two years was 9%.2
Case Presentation
January 12, 2016 a fourteen year old high school freshman girls
basketball player sustained a head injury and was diagnosed with a
concussion by her physician. She was forced to not participate for the rest of
the basketball season due to her concussion. Three months later after the
season had ended, the school was on spring break, during that time period
the subject had experienced two seizures. The subject was hospitalized after
the first seizure and was told that it was stress related. Although the
basketball season had ended, the subject was cleared from her concussion to
play in any sports. A few weeks later, the subject was hospitalized again for
the second seizure. A CAT scan was performed and the physician diagnosed
the subject with juvenile myoclonic epilepsy. The subject was prescribed a
drug called Keppra. Keppra is an anticonvulsant which is used to treat
epilepsy and subarachnoid hemorrhage. Although, the drug is more
commonly used for epilepsy.
Despite the subjects history of the concussion suffered, the doctor
explained to the subject and her mother that the concussion had no
correlation to why she experienced the seizures, for that the date she was
diagnosed with the concussion and the date from when she experienced the
seizures were too far apart.
The subject described her experience with her seizures and explained
that her first episode occurred in her sleep. The second episode occurred
while she was in the bathroom and collapsed on to the floor. The subject
complained of symptoms close to what would be symptoms of a concussion,
including sensitivity to light and headaches.
Results
After the subject told the head athletic trainer of her seizures, she
followed up with the athletic trainer two weeks later. She explained that she
had no seizures after she was prescribed Keppra. The athletic trainer wanted
to stress test the subject since she did not complete her post-concussion
stress test to return to play, but she decided that she did not want to
participate in sports anymore. Once the athlete stated she did not want to
play sports, she did not appear for anymore follow-ups based on her
condition.
Based on the limited information, the subject was classified to have no
relapses of seizures based on the follow-up time after the last hospitalization
of the subject.
Discussion
Because juvenile myoclonic epilepsy has no symptoms of its
prevalence in early childhood, it is hard to detect and treat for this disorder
at a young age. Most patients are unaware that they even experience MJs,
and do not take them as a serious indication of a disorder.4 Patients realize
that something is wrong when they are being hospitalized after their first
episode of GTCS, just like the case for the subject in this study.
Once a patient has been diagnosed with JME, it is much easier to spot
signs, knowing that they are now aware of their condition. Momentary MJs
can happen in a matter of seconds, these jerks are commonly symmetrical,
meaning that they occur simultaneously in both limbs. The subject in this
study had no recollect of whether or not she experienced any MJs or an
absence of time. Her first encounter of a seizure occurred in her sleep as
previously stated, but perhaps the subject experienced the seizure upon
awakening, for that is when it occurs the most.
The subject is a high school student and claims that she experiences a
lot of stress. Psychological aspects play a key role on how JME is managed a
strange personality or behavior pattern is typical for someone with JME.5 The
subject was not a troubled teen, although she was very aggressive, and at
some points found herself getting disciplined through the school for her
behavior. Her behavioral characteristics fit the description of someone who
has JME.
There is no known cure to JME, although medication suppress the
chances of seizing a great amount. Most patients do not seize or experience
any MJs if they adhere to their medication for years at a time.2 Although,
poor drug adherence could increase the amount of seizures a patient has.1
JME has an excellent response to treatment as long as the treatment is taken
regularly.1 Treatment plans and medication should be individualized based on
sex, gender, and comorbidities of other factors that could affect treatment.1
In order for the subject to decrease the amount of MJs or GTCS, she must find
methods to decrease her stress, be sure to have enough rest and sleep, and
to adhere to all the medications her physician gives her.
References
1. Asadi-pooya AA, Hashemzehi Z, Emami M. Predictors of seizure control
in patients with juvenile myoclonic epilepsy (JME). Seizure.
2014;23(10):889-91.
2. Hfler J, Unterberger I, Dobesberger J, Kuchukhidze G, Walser G, Trinka
E. Seizure outcome in 175 patients with juvenile myoclonic epilepsy--a
long-term observational study. Epilepsy Res. 2014;108(10):1817-24.
3. Cvetkovska E, Panov S, Kuzmanovski I. Clinical genetic study in juvenile
myoclonic epilepsy. Seizure. 2014;23(10):903-5.
4. Genton P, Thomas P, Kasteleijn-nolst trenit DG, Medina MT, Salas-puig
J. Clinical aspects of juvenile myoclonic epilepsy. Epilepsy Behav.
2013;28 Suppl 1:S8-14.
5. Syvertsen MR, Thuve S, Stordrange BS, Brodtkorb E. Clinical
heterogeneity of juvenile myoclonic epilepsy: follow-up after an interval
of more than 20 years. Seizure. 2014;23(5):344-8.