Progressive myoclonus epilepsy

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Progressive Myoclonus Epilepsy (Pronunciation: pro-gres-iv my-o-klo-nus ep-i-lep-sy) is a rare epilepsy syndrome which is characterized by myoclonic seizures, progressive neurological decline and other types of recurrent seizures. The term is derived from the Greek words "pro" meaning "forward", "gressus" meaning "step", "myo" meaning "muscle", "clonus" meaning "violent, irregular motion", and "epilepsia" meaning "seizure".

Overview

Progressive Myoclonus Epilepsy (PME) is a group of diseases characterized by myoclonus, epilepsy, and progressive neurological dysfunction. These disorders tend to worsen over time and are often resistant to treatment. PME is different from other types of epilepsy because of the progressive neurological decline and the presence of other types of seizures in addition to myoclonus.

Symptoms

The symptoms of PME may include:

  • Myoclonus: Quick, involuntary muscle jerk.
  • Seizures: Episodes of disturbed brain activity that cause changes in attention or behavior.
  • Ataxia: Decreased coordination or loss of balance.
  • Dementia: A decline in mental ability severe enough to interfere with daily life.

Causes

PME is usually caused by genetic mutations and can be inherited. The most common forms of PME are Lafora disease, Unverricht-Lundborg disease, myoclonus epilepsy with ragged red fibers (MERRF), and neuronal ceroid lipofuscinoses.

Treatment

Treatment for PME is symptomatic, focusing on managing myoclonus and other seizures. Medications like anticonvulsants and sedatives are often used.

See Also

External links

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