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Myoclonus is a brief, involuntary, irregular (lacking rhythm) twitching of a muscle, a joint, or a group of muscles, different from clonus, which is rhythmic or regular. Myoclonus (myo-"muscle", clonus "spasm") describes a medical sign and, generally, is not a diagnosis of a disease.
Myoclonus is usually classified physiologically to optimize treatment. Myoclonus is a precursor effect to myoclonus dystonia and most commonly begins in childhood or adolescence. [4] [5] Myoclonus is classified as cortical, subcortical, peripheral or spinal. Cortical myoclonus is the most common of these four and affects the upper limbs and face.
Asterixis (more colloquially referred to as flapping tremor) is not actually a tremor, but rather a negative myoclonus.This movement disorder is characterized by an inability to maintain a position, which is demonstrated by jerking movements of the outstretched hands when bent upward at the wrist (which can be similar to a bird flapping its wings, hence the name "flapping tremor").
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).
Progressive Myoclonic Epilepsies (PME) are a rare group of inherited neurodegenerative diseases characterized by myoclonus, resistance to treatment, and neurological deterioration. [2] The cause of PME depends largely on the type of PME. Most PMEs are caused by autosomal dominant or recessive and mitochondrial mutations. [2]
It is a disease that presents Myoclonus as a sequela of hypoxic disorders in the brain due to asphyxiation and cardiopulmonary arrest. [ 2 ] [ 3 ] It is exacerbated by mental and physical anxiety such as intention, intentional movement, and tension.
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Lafora disease is a rare, autosomal recessive [4] genetic disorder which results in myoclonus epilepsy and usually results in death several years after the onset of symptoms. The disease is characterized by the accumulation of inclusion bodies, known as Lafora bodies, within the cytoplasm of the cells in the heart, liver, muscle, and skin.