What are Lafora bodies?

Researchers have discovered that people with Lafora progressive myoclonus epilepsy have distinctive clumps called Lafora bodies within their cells. Lafora bodies are made up of an abnormal form of glycogen that cannot be broken down and used for fuel. Instead, it builds up to form clumps that can damage cells.

How do you treat Lafora?

Currently, AEDs are the only available treatments that control the severity and frequency of seizures and myoclonus to some degree in patients with Lafora disease. Among these drugs, valproic acid is the mainstay.

Is Lafora disease curable?

Treatment. Unfortunately there is no cure for Lafora Disease with treatment being limited to controlling seizures through anti-epileptic and anti-convulsant medications. The treatment is usually based on the individual’s specific symptoms and the severity of those symptoms.

What causes laforin?

Lafora disease is an inherited, severe form of progressive myoclonus epilepsy. The condition most commonly begins with epileptic seizures in late childhood or adolescence. Other signs and symptoms include difficulty walking, muscle spasms (myoclonus) and dementia.

Who gets Lafora disease?

Abstract. Lafora disease is a severe, autosomal recessive, progressive myoclonus epilepsy. The disease usually manifests in previously healthy adolescents, and death commonly occurs within 10 years of symptom onset.

What type of mutation is Lafora disease?

Lafora progressive myoclonus epilepsy (Lafora disease) is a rare, usually childhood-onset, fatal neurodegenerative disease caused by biallelic mutations in EPM2A (Laforin) or EPM2B (NHLRC1; Malin).

How many people are affected by Lafora disease?

The disease has been found in more than 250 families throughout the world, resulting from EPM2A (responsible for Laforin) and EPM2B (responsible for E3 ubiquitin-protein ligase NHLRC1) mutations, and the prevalence seems to be close to four cases per one million persons.

How common is Lafora disease?

What disease is associated with laforin?

Lafora disease (LD) is an autosomal recessive, progressive myoclonus epilepsy, which is characterized by the accumulation of polyglucosan inclusion bodies, called Lafora bodies, in the cytoplasm of cells in the central nervous system and in many other organs.

Can epilepsy ever go away?

While many forms of epilepsy require lifelong treatment to control the seizures, for some people the seizures eventually go away. The odds of becoming seizure-free are not as good for adults or for children with severe epilepsy syndromes, but it is possible that seizures may decrease or even stop over time.

What does laforin do in the body?

Additionally, laforin may act as a tumor suppressor protein, which means that it keeps cells from growing and dividing in an uncontrolled way. Laforin and malin likely play a critical role in regulating the production of a complex sugar called glycogen. Glycogen is a major source of stored energy in the body.

What are the best foods for epilepsy?

A low glycemic index diet focuses on foods with a low glycemic index, meaning they affect blood glucose levels slowly, if at all. Although it’s not understood why, low blood glucose levels control seizures in some people. Foods on this diet include meat, cheese, and most high-fiber vegetables.