What is a myoclonic encephalopathy?

What is a myoclonic encephalopathy?

Collapse Section. CHD2 myoclonic encephalopathy is a condition characterized by recurrent seizures (epilepsy), abnormal brain function (encephalopathy), and intellectual disability. Epilepsy begins in childhood, typically between ages 6 months and 4 years. Each individual may experience a variety of seizure types.

What causes myoclonic encephalopathy?

Metabolic causes are common. A few of these include non-ketotic hyperglycinemia, amino and organic acid disorders, urea cycle disorders, mitochondrial disorders, pyridoxine and pyridoxal-5-phosphate disorders, sulfite oxidase deficiency, Menke syndrome, Zellweger syndrome, amongst many others.

What metabolic disorders cause myoclonus?

Regarding the nature of onset, the acute onset of myoclonus is seen in toxic–metabolic disorders such as hepatic and renal failure, thyrotoxicosis, electrolyte disturbances (e.g. hyponatraemia, hypoglycaemia, nonketotic hyperglycaemia), some neuroinfectious diseases (herpes simplex encephalitis, neuroboreliosis).

What is the treatment for myoclonus?

Treatment of myoclonus focuses on medications that may help reduce symptoms. The drug of first choice is clonazepam, a type of tranquilizer. Many of the drugs used for myoclonus, such as barbiturates, phenytoin, and primidone, are also used to treat epilepsy.

Can EEG detect myoclonus?

Electroencephalography (EEG) This procedure records the electrical activity of your brain and may help determine where in your brain the myoclonus originates.

Can myoclonus be benign?

Benign myoclonus of early infancy is a rare condition characterized by nonepileptic spasms that may resemble the epileptic spasms seen in West’s syndrome. The spells in benign myoclonus of early infancy begin before age 1 year and are self-limited.

Is myoclonus a form of epilepsy?

There are many different types of epilepsy. Myoclonic epilepsy causes the muscles in the body to contract. This type of seizure causes quick jerking movements. Myoclonic seizures often happen in everyday life.

Does myoclonus affect the brain?

It can be the most disabling form of myoclonus affecting the arms, legs, and face. One of the causes may be brain damage that results from a lack of oxygen and blood flow to the brain, or it can be secondary to other medical or neurological conditions.