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Checking time stamp

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Real time: 11:32 Neuropsychprof (talk) 16:32, 24 October 2012 (UTC)

Misc wikipedia codes

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Use the sandbox to practice posting things on Wikipedia. In addition to the Wikimarkup cheatsheet, here are a couple of helpful tips: To indent, use :

once to indent
twice to indents 2 tabs
three times to indents 3, etc.

To start a new paragraph, put in an empty line.

Remember to always sign your contributions by typing 4 of these ~ squigglys.

Feel free to play in my sandbox! But if I see any offending content, I retain the right to delete your stuff because this is my sandbox. Neuropsychprof (talk) 20:40, 27 August 2012 (UTC)


Myoclonic epilepsy edits

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Myoclonic epilepsy refers to a family of epilepsies that present with myoclonus. When myoclonic jerks are associated with abnormal brain wave activity one or two times, it can be categorized as myoclonic seizure. If the abnormal brain wave activity is persistent and results from ongoing seizures, then a diagnosis of myoclonic epilepsy may be considered.

Classification

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There are two major syndromes and several related disorders.

Juvenile Myoclonic Epilepsy

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Juvenile Myoclonic Epilepsy is responsible for 7% of all cases of epilepsy[unreliable medical source?][1]. Seizures usually begin around puberty and usually has a genetic basis[1]. Seizures can be stimulus-selective, with flashing lights being one of the most common triggers[1].

Progressive myoclonus epilepsies

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Progressive myoclonus epilepsy is a disease associated with myoclonus, epileptic seizures, and other problems with walking or speaking. These symptoms often worsen over the life time and can be fatal[2]. These diseases are due to mutations that are of recessive autosomal inheritance, which causes the inability for an important protein to be produced or function properly[3].

MERRF syndrome

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MERRF syndrome is also known as Myoclonic Epilepsy with Ragged-Red Fibers. This rare inherited disorder affects muscles cells[4]. Features of MERRF, along with myoclonus epilepsy seizures, include ataxia, peripheral neuropathy, and dementia[4].

Lafora disease

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Lafora disease is also known as Lafora progressive myoclonus epelepsy, which is an autosomal recessive inherited disorder involving reoccurrent seizures and degradation of mental capabilities[5]. Lafora disease usually occurs in late childhood and usually leads to death around 10 years after first signs of the disease[5]. It is brought on by mutations of either the EPM2A or the NHLRC1 genes[5].

Unverricht-Lundborg disease

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Unverricht-Lundborg disease is an autosomal recessive inherited disorder seen in individuals as young as 6 years of age[3]. It is associated with possible loss of consciousness, rigidity, ataxia, dysarthria, declination of mental functioning, and involuntary shaking[3]. A mutation on the CSTB gene is the cause of this disease[3].

Neuronal ceroid lipofuscinosis

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Neuronal ceroid lipofuscinosis is a group of diseases that cause blindness, loss of mental abilities, and loss of movement [6]. All diseases in this group are lysosomal-storage disorders that also lead to death roughly 10 years after onset of the disease[6].

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Lennox–Gastaut syndrome

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Recticular reflex myoclonus=

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Recticular reflex myoclonus is a generalized form of epilepsy originating from the brain stem. Jerks associated can effect all muscles on the body or be selective toward certain areas[2]. Jerks can be triggered by voluntary movement or be simulus-selective[2].

Signs and symptoms

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Myoclonus

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Myoclonus can be described as brief jerks of the body[8]. It can involve any part of the body, but it is mostly seen in patient’s limbs or facial muscles. The jerks are usually involuntary and can be responsible for multiple falls[8]. EEG is used to read brain wave activity from the scalp. Spike activity produced from the brain is usually correlaed with brief jerks seen on EMG or excessive muscle artifact. They usually occur without detectable loss of consciousness and may be generalized, regional or focal on the EEG tracing[8]. Myclonus jerks can be epileptic or not epileptic[8]. Epileptic myoclonus is an elementary electroclinical manifestation of epilepsy involving descending neurons, whose spatial (spread) or temporal (self-sustained repetition) amplification can trigger overt epileptic activity[8]. If the jerks are not epileptic, they are not caused by over firing of neurons in the brain. Instead, they may be due to some nervous system disorder, for example Parkinson's Disease.

Types of Myoclonus

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There are many types of myoclonus, yet it is important to note that correctly diagnosing these types is difficult due to their various biological origins, behavioral effects, and their overall reaction to therapies[2] Listed below are some of the most common forms of myoclonus:

Action myoclonus
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This is the most disabiling form of myoclonus and is usually caused by brain damage resulting from oxygen and blood deprivation when breathing or heartbeat is temporarily halted. The associated jerks of this syndrome can be made worse if the individual attempts precise, coordinated movements[2].

Cortical reflex myoclonus
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This originates from the cerebral cortex. The jerks may involved few or many muscles. The associated jerks can be intensified when precise movement is attempted or a particular sensation is perceived[2].

Essential myoclonus
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This can occur without epilepsy or brain abnormalities. Essential myoclonus often has no known cause, and can occur in people with no family history of jerks. However, there is some indication of it being a possible inhereited disorder[2].

Palatal myoclonus
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This affects mainly the face, throat, and diaphram. Palatal myoclonus creates rapid contractions that persist even in sleep. This condition usually appears in adulthood. Some complain of it as only a minor problem while others claim it causes severe discomfort and pain[2].

Stimulus-senstive myoclonus
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Symptoms are often triggered by external events, often associated with surprise. Some of the common triggering events can be flashing lights, movement from televisions or video games, or loud noises[2].

Sleep myoclonus
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These jerks often occur in the initial phrases of sleep. Many do not seek treatment for these symptoms. This form of myoclonus can be a symptom of an associated sleep disorder yet to be diagnosed. Some forms of this myoclonus can be stimulus-sensitive, whereby jerks are caused by external events[2].


References

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  1. ^ a b c Juvenile Myoclonic Epilepsy: Characteristics and Treatment". Epilepsy.com.
  2. ^ a b c d e f g h i j Myoclonus Fact Sheet". National Institute of Neurological Disorders and Stroke.
  3. ^ a b c d Unverricht-Lundborg disease. Genetics Home Reference. Cite error: The named reference "ULD" was defined multiple times with different content (see the help page).
  4. ^ a b "Myoclonic epilepsy with ragged-red fibers". Genetics Home Reference.
  5. ^ a b c "Lafora progressive myoclonus epilepsy". Genetics Home Reference. Cite error: The named reference "lafora" was defined multiple times with different content (see the help page).
  6. ^ a b "Neuronal Ceroid-Lipofuscinoses". GeneReviews. Cite error: The named reference "Neuronal Ceroid-Lipofuscinoses" was defined multiple times with different content (see the help page).
  7. ^ a b Niedermeyer, Ernst (1992). "Epileptic Syndromes: A Remarkable Contribution of EEG to Epileptology". American Jounal of EEG Technology (32): 10–12.
  8. ^ a b c d e Wyllie, Elaine (2006). The Treatment of Epilepsy. Lippincott, Williams and Wilkins. ISBN 0-7817-4995-6., 307-408. Cite error: The named reference "Wyllie" was defined multiple times with different content (see the help page).