Amyotrophic lateral sclerosis 1

Inheritance

How is Amyotrophic Lateral Sclerosis inherited?

Changes in several genes have been reported to cause familial amyotrophic lateral sclerosis (ALS), which are inherited. ALS can be inherited in three different ways: autosomal dominant, autosomal recessive and X-linked dominant. In autosomal dominant forms of ALS only one of the two copies of the gene need to have a disease-causing change. Individuals with a parent with dominant ALS have a 50% chance to develop the disease. In the autosomal recessive forms of ALS, both copies of the gene must have a disease-causing change. Autosomal recessive cases only occur when both parents have a genetic variation and both pass it on to a child. In X-linked dominant ALS, both men and women are affected but the gene can not be passed from male to male.

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What percentage of Amyotrophic Lateral Sclerosis is inherited?

What percentage of Amyotrophic Lateral Sclerosis is sporadic?

Are my kids are risk for Amyotrophic Lateral Sclerosis if I have it?

What does it mean to have inherited a “variant” in the gene for Amyotrophic Lateral Sclerosis?

What percentage of Amyotrophic Lateral Sclerosis is inherited?

An estimated 10% of all amyotrophic lateral sclerosis (ALS) cases are familial or inherited.

What percentage of Amyotrophic Lateral Sclerosis is sporadic?

An estimated 90% of amyotrophic lateral sclerosis (ALS) cases occur sporadically. People with sporadic ALS have no family history of the disorder. Environmental exposures may be possible contributors to sporadic ALS.

Are my kids are risk for Amyotrophic Lateral Sclerosis if I have it?

If an individual has a familial form of amyotrophic lateral sclerosis (ALS), their children may be at risk. The specific risk depends on what gene contains the variant causing the disease. For individuals who have an autosomal dominantly-inherited type of ALS, their children have a 50% chance to inherit the same variant and develop the disease. For individuals who have an autosomal recessively-inherited type of ALS, their children will all inherit one copy of a disease-causing variant but are only at risk to develop the disease if they also inherit a variant from their other parent. For individuals who have an X-linked type of ALS, the chance for a child to inherit the variant depends on their gender and the gender of the affected parent, but no male to male transmission can occur.

What does it mean to have inherited a “variant” in the gene for Amyotrophic Lateral Sclerosis?

A variant is a change in the DNA when compared to a standard reference. A variant may or may not cause a higher risk for disease depending on the type of variant, the location and how it might change a protein.

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