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Ezri Cox

Med. Term: Unit 4


Dr. Tami Cottrell
1/13 1/18, 2016
Juvenile Myoclonic Epilepsy,
Seizures, and Muscular Episodes
Juvenile Myoclonic Epilepsy often referred to as Janz syndrome or JME -- is an
idiopathic, neurological disorder that affects myokinesis (muscle movement) and distresses the
voluntary control of a patient for mild or severe intervals at a time. Like other forms of epilepsy
disorders, a patient with JME will experience frequent episodes which include disorientation,
involuntary muscle spasms and interruption of daily tasks, or full-scale seizures. Seizures in
patients with JME are most likely to occur upon waking up in the morning or after taking a nap.
Patients can also experience seizures that are triggered, commonly by drug use, alcohol, lack of
sleep, or interrupted sleep or early awakening. Risk of episodes or seizures provoked by JME are
highest in patients aged twelve to eighteen years old, however patients from eight to their midtwenties are fairly vulnerable as well; Disregarding age of the patient, seizures and their
symptoms can be rated from moderate to severe.
Symptoms that result from or determine seizures can be indefinite: some patients may
endure all symptoms, while some patients present with a portion of symptoms, and each patient
will have varying levels of discomfort and a distinctive degree of seizure as a majority of
seizures induce spasms while other forms of seizures are indicated by stillness and mental pause.
Basic symptoms occur prior to, during, or following a seizure, which will mainly affect muscular
function and physical condition. One experiencing a seizure will experience ataxia (loss of
voluntary control over one's movement), which can be accompanied by disorientation and
confusion, hallucinations of any of the five basic senses, lack of focus, or abnormal sensations or
discomfort -- commonly cephalgia or gastralgia, stiffness of body, or feelings of "pins and
needles" in any area of the body. JME can be a cause of three types of seizures, which include
absence seizures, of which are characterized by loss of responsiveness and bradykinesia (also
known as hypokinesis), myoclonic seizures, determined by myoclonus and hyperkinesis)
throughout any portion of the body, and tonic-clonic seizures (otherwise, convulsive seizures)
that occur when a myoclonic seizure affects the entire brain and display more defined, severe
symptoms of a myoclonic seizure. Patients who suffer episodes will remain conscious while
enduring symptoms, yet irresponsive during seizure.
The symptoms are not the only impact a patient will face; seizures or any form of
myoclonus due to neurological distress have the potential to place a patient, especially a small
child, at risk for injury or damage as the striated, skeletal muscles are no longer in control and
are influenced into involuntary action unlike their intended function. Seizures and myoclonus
of any form are result of neurological illnesses such as JME and render the patient ataxic and
possibly weak with no coordination, and, with lack of supervision and assistance, a patient can
be disrupted from daily activities and can present precarious behavior out of their control such as

throwing or dropping objects in their hands or falling onto the floor during intense convulsions
(usually occurring throughout the entire body). Some patients are claimed to have auditory
hallucinations, visual hallucinations, olfactory hallucinations, gustatory hallucinations, or tactile
hallucinations, and most are reported unpleasant or unsettling, which could expose any patient
for risk of injury due to distraction during tasks or unclear sense of environment.
The seizures and myoclonic episodes that are brought upon by JME and their
complications will be undergone as informed above most commonly by JME patients that are
aged twelve to eighteen years old. The use of substances, over-engaging in complex or
challenging tasks, menstruation, a series of fasting, and overtiredness can greatly increase a
patients risk of seizures; flickering, stunning, or flashing lights can be a trigger in 30-40%, or
nearly one-third, of patients. The chances of a child being identified with JME are higher if the
child has been diagnosed with brain abnormalities and differences in gray matter distribution, if
epilepsy can be traced back to their family history, or if they have previously experienced febrile
seizures or childhood absence epilepsy. Through reported cases, specialists have determined that
JME is currently detected in about 10% of patients with epileptic disorders, making it appear
uncommon.
Epilepsy and complications, including JME develop when there is an imbalance in one's
brain signaling, particularly when neurons cannot properly communicate and excess electrical
impulses have the potential to overstimulate certain areas of the brain. The effects occur in the
brain's temporal lobe, occipital lobe, and frontal lobe, and the overstimulation sets roots for
neurological dysfunction and the episodes that involve ataxia of voluntary muscles and seizures.
Most cases happen out of genetic odds (family history) or brain deformities. Affected patients
and their families can consult neurologists and myologists to become better informed on their
condition and get assistance on managing the effects that take place within the muscular system
due to these neurological malfunctions. Patients experiencing epilepsy can be referred to an
epileptologist to diagnose JME through testing, which mainly involves these brain signals and
are practically pain free. An electromyography, or EMG, is a diagnostic procedure to assess the
condition of a patient's muscles and motor neurons, or the nerve cells that control the muscles.
The motor neurons transmit signals that cause muscle activity, specifically contractions, which
an EMG will allow a specialist to detect and interpret. A nerve conduction study uses devices
called "surface electrodes," which are taped onto a patient's skin, to measure the speed and
strength of signals traveling between multiple points of the muscular system. By adhering
surface electrodes to a patient's scalp, a technician can perform an electroencephalogram, or
EEG, to record brain waves in order for a neurologist to interpret the results. In a patient with, a
pattern of "spikes" is often present, especially as a response to a patient's exposure to flashing
lights. To confirm the diagnosis of JME, an EEG can be performed while a patient is asleep and
just after awakening. Magnetic resonance imaging, commonly recognized as an MRI, is a
process that grants a high-resolution scan of the brain and root of neurological activity, as well as
a lower quality however faster and more widely available alternative that is a CT, or computed
tomography, scan. With the inclusion of a phlebotomist, count of erythrocytes and leukocytes in
the blood and measure of hemoglobin via blood testing will help indicate epileptic disorders such

as JME. Along with the aforementioned causes, determination or prediction of JME being
present in a patient using family history or past medical records can be effective.
While the condition is not curable, patients have opportunities to suppress symptoms and
seizures in the form of medication. Upon diagnosis and testing, an epileptologist will conclude
which treatment(s) would be most effective for the patient. Depakote (valproate or valproic acid)
is often used to treat JME. Depakote is an anti-seizure drug, and can treat absence, myoclonic, or
tonic-clonic seizures that occur in people with JME. It is reported to be quite effective in
controlling seizures, however, with use, a patient is vulnerable to adverse side effects such as
gastralgia, weight gain, and/or hair loss. A choice more often explored by patients would be
benzodiazepine, a neurological depressant, used to treat ongoing seizure activity. Lorazepam,
diazepam, midazolam or clonazepam are common examples of benzodiazepine medications.
When benzodiazepine treatment fails to suppress seizure activity, phenytoin or fosphenytoin or
penobarbital can be administered. Carbamazepine, oxcarbazepine and topiramate are
anticonvulsant medications that epileptologists may choose to provide along with a patient's
other medicinal treatments at any point during the process of halting an urgent case. Diazepam
gel can be prescribed for home use, through rectal administration. Lamotrigine should be
avoided, as it may aggravate a patient's condition and worsen myoclonus. Controlling alcohol or
recreational drug intake and maintaining a proper diet and sleep habits can affect and
significantly lower the frequency of a patient's seizures; avoidance of non-treatment drugs and
alcohol is crucial for the effectiveness and safety of any anti-seizure medications.
Juvenile Myoclonic Epilepsy, and its seizures and myoclonus, is a neurological disorder
that frequently affects the myokinesis in the voluntary, striated muscles of patients from as young
as age eight to mid-twenties. A firm majority of JME cases, which consist of around 10% of
cases out of all epileptic disorders, have stemmed from abnormalities or damage to the brain or
through a childs genetics. Many tests are available with help of a technician, and, upon
diagnosis, a broad variety of medications are available to treat or suppress symptoms.

Sources Cited

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"Juvenile Myoclonic Epilepsy Symptoms, Causes, and Treatments." WebMD. WebMD, n.d.
Web. 27 Jan. 2016.
"Different Parts of the Brain That Epilepsy Effects." Livestrong. LS Health, 08 Sept. 2015. Web.
27 Jan. 2016.
"Juvenile Myoclonic Epilepsy Treatment & Management." Juvenile Myoclonic Epilepsy
Treatment & Management: Approach Considerations, Anticonvulsant Therapy, Surgical Care.
N.p., n.d. Web. 27 Jan. 2016.
"What Are Hallucinations?" Types, Causes, and Treatments of Hallucinations. MD, n.d. Web. 27
Jan. 2016.
"Electromyography (EMG)." Mayo Clinic. Specialists at Mayo, n.d. Web. 27 Jan. 2016.
"Medicines to Stop a Seizure." Livestrong CO. Livestrong Health, 07 July 2015. Web. 27 Jan.
2016.

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