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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.
Deep brain stimulation (DBS) has been found to be an effective and safe treatment for myoclonus dystonia patients, whose severe and debilitating symptoms are resistant to drug treatments. Electrical stimulation within the brain is a common treatment for many movement disorders because of the ability to excite or inhibit neurons within the brain.
Progressive myoclonus epilepsy is a disease associated with myoclonus, epileptic seizures, and other problems with walking or speaking. These symptoms often worsen over time and can be fatal. [6] MERRF syndrome is also known as myoclonic epilepsy with ragged-red fibers. This rare inherited disorder affects muscles cells. [7]
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.
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 ).
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.
These findings may explain subtle structural and functional brain abnormalities seen in patients with JME. [25] JME is distinct from other forms of genetic generalized epilepsy due to the prominence of myoclonus. There is evidence that patients with JME have hyperexcitable motor cortexes, most pronounced in the morning and after sleep deprivation.
A hypnic jerk, hypnagogic jerk, sleep start, sleep twitch, myoclonic jerk, or night start is a brief and sudden involuntary contraction of the muscles of the body which occurs when a person is beginning to fall asleep, often causing the person to jump and awaken suddenly for a moment.