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Progressive myoclonus epilepsy (PME) is a group of diseases characterized by myoclonus, epileptic seizures, tonic–clonic seizures, and other serious symptoms such as trouble walking or speaking. These rare disorders often get worse over time and can be fatal.
Palatal myoclonus. The myoclonic triangle (also known by its eponym Triangle of Guillain-Mollaret or dentato-rubro-olivary pathway) is an important feedback circuit of the brainstem and deep cerebellar nuclei which is responsible for modulating spinal cord motor activity. [1] [2] The circuit is thus composed: [2]
Myoclonic dystonia or Myoclonus dystonia syndrome is a rare movement disorder that induces spontaneous muscle contraction causing abnormal posture. The prevalence of myoclonus dystonia has not been reported, however, this disorder falls under the umbrella of movement disorders which affect thousands worldwide. [1]
Familial adult myoclonus Epilepsy (FAME) This is a condition characterized by the repetition of non-coding sequences and has been identified using various abbreviations. Initially, it was associated with four primary gene locations: FAME1 (8q23.3–q24.1), FAME2 (2p11.1–q12.1), FAME3 (5p15.31–p15.1), and FAME4 (3q26.32–3q28).
Opsoclonus myoclonus syndrome (OMS), also known as opsoclonus-myoclonus-ataxia (OMA), is a rare neurological disorder of unknown cause which appears to be the result of an autoimmune process involving the nervous system. It is an extremely rare condition, affecting as few as 1 in 10,000,000 people per year.
Palatal myoclonus is a rare condition in which there are rhythmic jerky movements or a rapid spasm of the palatal (roof of the mouth) muscles. Chronic clonus is often due to lesions of the central tegmental tract (which connects the red nucleus to the ipsilateral inferior olivary nucleus ).
Myoclonus refers to the jerking movement of muscles, that tend to be sudden and brief, typically involving the arms, legs, or the whole body. [8] Other common clinical findings include generalized epilepsy, ataxia (poor coordination), myopathy difficulty keeping up with physical activity, dementia, ptosis , optic atrophy , peripheral neuropathy ...
MEAK is a form of progressive myoclonus epilepsy that typically begins between the ages of 3 and 15 years (the average of onset is 10 years). The first symptoms may include ataxia and myoclonus (unsteadiness and difficulty coordinating movements), along with generalized tonic-clonic ("grand mal") seizures.