The following information may help to address your question:
Is amyotrophic lateral sclerosis (ALS) inherited?
About 90-95% percent of cases of ALS are not
inherited and occur in individuals with no history of the condition in their family. The remaining 5-10% of cases are familial, and are thought to be caused by mutations
in any one of several genes
. The inheritance pattern associated with familial ALS varies depending on the disease-causing gene involved.
Most familial cases are inherited in an autosomal dominant
manner. This means that only one altered (mutated) copy of the disease-causing gene in each cell is sufficient to cause the condition. In most of these cases, an affected individual has one parent with the condition. When an individual with an autosomal dominant form of ALS has children, each child has a 50% (1 in 2) risk to inherited the mutated copy of the gene and be affected.
Less frequently, ALS is inherited in an autosomal recessive
manner. In autosomal recessive inheritance, both copies of the disease-causing gene (typically one copy inherited from each parent) must have a mutation for the individual to be affected. The parents of an individual with an autosomal recessive condition, who presumably each carry one mutated copy of the gene, are referred to as carriers. Carriers typically do not have any signs or symptoms of the condition. When two carriers for the same condition are having children, each child has a 25% (1 in 4) risk to have the condition, a 50% (1 in 2) risk to be a carrier like each parent, and a 25% risk to not have the condition and
not be a carrier. Autosomal recessive forms of ALS may be mistaken for non-inherited (sporadic) forms due to having a negative family history of the condition.
In rare cases, ALS is inherited in an X-linked dominant
manner. This occurs when the disease-causing gene is located on the X chromosome (a sex chromosome). Although females have 2 X chromosomes, having a mutation in one X chromosome is still sufficient to cause the condition. Males who have a mutation (and only one X chromosome) will have the condition. Usually, males with an X-linked dominant form of ALS experience more severe symptoms than females with the same form.
Some individuals who do inherit a mutation known to cause ALS never develop signs and symptoms of ALS, although the reason for this is unclear. This phenomenon is referred to as reduced penetrance
Last updated: 1/11/2013
How is age of onset of amyotrophic lateral sclerosis (ALS) associated with the likelihood of it being an inherited form?
Age of onset alone is not a reliable predictor of the form of ALS that an individual has. Furthermore, if an individual is found to have an inherited form of ALS, the specific inheritance pattern would depend on the disease-causing gene involved. Establishing the specific subtype of ALS in a given individual usually involves obtaining family history and performing molecular genetic testing.
Onset of ALS may occur any time from the teenage years up to the late 80s.
The average age of onset for sporadic (non-inherited) ALS has been estimated to be around 56 to 65 years (estimates vary among sources).
The average age of onset for familial ALS is approximately 46 years.
However, onset of either form may occur years before or after these average ages.
Last updated: 1/11/2013
Is genetic testing available for amyotrophic lateral sclerosis?
Yes. Clinical genetic testing is currently available for several genes in which mutations are known to cause ALS. Genetic testing on a research basis is also available for select susceptibility genes associated with ALS.
You can find laboratories offering clinical and research genetic testing for ALS on a Web site called GeneTests. To see GeneTests' list of the types of ALS for which genetic testing is available, click here. Click on "Testing" next to each type of ALS of interest to see a list of the laboratories that offer clinical testing. Click on "Research" next to each type of ALS of interest to see a list of the laboratories that offer research testing.
Please note that most of the laboratories listed through GeneTests do not accept direct contact from patients and their families. Therefore, if you are interested in learning more, you will need to work with a health care provider or a genetics professional.
Last updated: 1/11/2013
How can I find a genetics professional in my area?
Last updated: 7/15/2016
We hope this information is helpful. We strongly recommend you discuss this information with your doctor. If you still have questions, please
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