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Types of Myoclonus and Myoclonus Epilepsies

 * There are many types of myoclonus, yet it is important to note that correctly diagnosing these types is difficult due to their various biological orgins, behavioral effects, and their overall reaction to therapies

Listed below are some of the most common forms of myoclonus, for far more indepth detail of those listed refer to the Myoclonus wikipedia page:

Progressive myoclonus epilepsy
 * a disease assoicated with myoclonus, epileptic seizures, and other problems with walking or speaking, these symptoms often worsen over the life time and can be fatal

Juvenile Myoclonic Epilepsy
 * one of the most common forms of myolonus and is responsible for 7% of all cases of epilepsy

Lennox–Gastaut syndrome
 * one of the most troubling forms of myoclonus, often assoicated with intellectual deficts as well as having a reputation for lack of responses towards medicational therapy

MERRF syndrome
 * also known as Myoclonic Epilepsy with Ragged-Red Fibers, this rare inherited disorder effects the muscles cells of the affected indivduals

Recticular reflex myoclonus
 * a generalized form of epilepsy orginating from the brain stem, jerks assoicated can effect all muscles on the body or be selective toward certain areas

Action myoclonus
 * the most disabiling form of myoclonuus usually caused by brain damage resulting from oxygen and blood deprivation when breathing or heartbeat is temporarily halted

Cortical reflex myoclonus
 * orginates from the cerebral cortex, the jerks may involved few or many muscles

Essential myoclonus
 * this form can result without epilepsy or brain abnormalities, often no known cause to be assoicated with these symptoms

Partial myoclonus
 * effecting mainly the face, throat, and diaphram is form creates rapic contractions that persist even in sleep

Stimulus-senstive myoclonus
 * symptoms of myoclonus are often triggered by external events often assoicated with surprise

Sleep myoclonus
 * jerks often occur in the inital phrases of sleep, although many do not seek treatment for these symptoms this form of myoclonus can be a symptom of an assoicated sleep disorder yet to be diagnosed

'Lennox-Gastaut Syndrome'

Lennox-Gastaut syndrome is one of the most unfavorable and severe form of epilepsey due to its lack of response to medication.(#5) Defined by three characteristics: multiple seizure types, a distinctive brain-wave pattern and mental deficts(ranging from slight to profound) Myolclonic seizures occur in 30% of patients with LGS, the term for those with such seizures is myoclonic variant of LGS


 * 1.)Symptoms


 * The usage of the Electroencephalography has been very important in understanding the brain activity of Lennox-Gastaut. EEGs show that it is characterized with slow spike-wave complexities(Niedermeyer). Meaning that, those with LGS show generally increased amount of slow activity brain waves
 * Multiple forms of seziures are seen in those suffering from Lennox-Gastaut, making Lennox-Gastaunt such a severe syndrome. These include: tonic-clonic seizure, atonic seizure, myoclonic, absence seizure, chronic (rapidly repetice clonic movements), hemiclonic, atypical seizures (with slow spike-waves) (Niermeyer & #13)
 * Along with the various seizures the disorder is assoicated with intellecual deficits are associated with it as well (except if the seizure onset is past age ten) (Niedermeyer). These deficits can range from slight to profound in impariment. (#13) This cognitive impairment often does not become apparemt itil later in the disorder.


 * Often times there are epileptic syndromes and diseases that mimic that of LGS including: myoclonic epilepsies of early childhood, atypical partial benign epilepsy of childhood, epilepsy with electrical status epilepticus during slow-wave sleep, neuronal ceroid lipofuscinosis, and Angelman syndrome (#3).

The most common include those with myoclonic symptoms:

a.) Myoclonic Astatic Epilepsy:  A syndrome characterized by myoclonic, astatic, typical absence and tonic-clonic seizures that develops between one and five years of age. (#3) The distinction with LGS and this syndrome is not always clearm however thos with myoclonc-astatic have better seziure control and mental development than those with LGS (#3)

b.) Atypical Partial Benign Epilepsy: A syndrome characterized by myoclonic and atonic seizure clusters lasting between 2-4 weeks between the ages of two and six years of age (#3). Unlike LGS those with this syndrome usually have seizures that remit before the age of ten, and mental development regression is not signficant (#3).

c.) Angelman Syndrome: A syndrome characterized by microcephaly, flat occiput, severe metal retardation (like that of LGS), ataxia, jerky limb movements (myoclonus), and a cheerful dispostion (#3). This syndrome developes much earlier than LGS with first seizures occuring in infancy (#3).


 * 2.) Causes


 * There is no known direct primary cause for the syndrome, however there are many underlying casuse for sympotmatic Lennox:

Encephalitis and / or meningitis

Encephalopathy

Traumatic brain injury

Disease and / or developmental disorder

Malformation of the brain

Tuberous sclerosis

Trauma or injury at birth

Hypoxia ischemia injury

Lesions in the frontal lobe

Hereditary metabolic diseases (#14)

However, in many cases there is no know particular cause found for the development of the disease.


 * 3.) Onset/Prevance/Prognosis


 * Onset is in children, the first seizure happens between one and eight years of life(#3),most commonly developing at a peak between the ages of three and five, with a higher prevances amoung males than females (#3,#13). A rare disorder, Lennox-Gastaut occurs in 0.3 of 1,000 births. However, the symptoms may not always develop in childhood (#6).


 * In about 25% of childhood cases of LGS there is normal neurologically prior to the oneset, whereas in 30-41% of cases LGS is preceded by West Syndrome (#3).


 * Unlike other childhood epilpetic syndromes that tend to go into remission or stop all together by adolscence, Lennox-Gastaut are usually present in the rest of one's life once they occur (#6). Many with the disorder never are able to have independent adult lives with seizures that occur daily (#3). The prognosis of somone with normal mental health development is poor with LGS, however it should be noted, that the age of seizure onset is usally the best correlation to mental outcome, those with onset prior to age three show severe mental degression (#3). The type of seizures experienced also have an impact on prognosis with seizure types of myoclonic or atypical absence forms being more hopeful in prognosis (#3).


 * 4.) Treatment


 * Lennox-Gastuant is a particualar tricky syndrome to treat, usually multimodial forms are required. Even with multiple forms of treatment, total control of the seizures is a rarity (#13).

a.) Medication

Generally, LGS has a bad reputation for having little to no response towards antiepileptic medications (Niedermeyer). However, usage of medications is usually administered, these medications include: Anticonvulsants, anesthetics, steroids such as prednosolone, valproates, benzodiazepines,and felbamate (#14). With regard to medications considerations must be made to treat the seizures best as possible while avioding negative side effects also it is important to note that treatment from a medical standpoint may be very difficult due to periods of active verus' inactive siezure periods(#13). Co-morbidity with intellecual deficts should also be respected.

b.) Surgery


 * The usage of corpus callosotomy may be used in severe cases of LGS (#13, #14) This surgery consists of severing the interconnecting network of nerves that allow the two hemispheres of the brain to be connected (#5).


 * Those who have poor resoponse to medication or are unlikey to recieve a psychosurgery can undergo vagus nerve stimulation (#5 and #6). This treatment involves inplanting a "battery", small device under the skin of the left side of the chest (#5). The battery when stimulated ommitts short repetated pluses of electrical stimulation thoughout the twenty-four hours of the day (#5 and #6). The theory is that the pulse interfers with the abnormal elctrical pluses in the brain which result in the siezures in the first place, thus preventing the seizure before it can begin (#5 and #6). 15% of those with LGS use VNS (#5). Results of seizure control vary from patient to patient; 1/3 of VNS use experience massive improvement (more than 50%), 1/3 report moderate control, and 1/3 report no change (#5).

c.) Ketogenic Diet

Known as the "long-chained triglyceride diet", the Ketogenic diet  involves a strict regiment of high-fat, low-carbohydrate intake, this increases the amount of ketones in the body (#5 and #6). Ketones are formed with the body uses fat for energy instead of carbohydrates (#6). Doctors are unsure how this build up of ketones directly corresponds to reducing seizures, although some have theories (#5, #6, #14). Strict adherance to the diet is nesssary, sudden haults in the diet result in severe relapse of seizures, therefore patients who do well on the diet must be gradually taken off the diet over several months ( #5 and #6). Over half of children on the diet show a 50$ reduction in seizures and some chidren 10-15% become seizure-free ( #6). Side effects of long-term use of the diet include, high cholesterol, slowed growth, wieght gain, and more.


 * 4.) Future research

A majority of research into LGS focuses on finding a primary cause for the disease as well as a productive treatment plan.