Familial ALS

 

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What Is Familial ALS?

Familial ALS makes up 10% of all ALS cases. In 20% of the 10% of ALS cases, a genetic mutation has been identified.  Back to What Is ALS?

Curtisí maternal great-great-great-great grandfather, Samuel Farr, born in 1804, died of Farrís disease in 1865.  The doctors named it Farr's disease, but it was later known to be familial ALS. Farrís disease has affected approximately 50% of every generation of Curtisí ancestors and family since Samuel Farr, his brother, who died at age 40 of ALS, and his sister, who died at age 54 of the same disease. Out of Samuel Farr's eight children, four of them died of ALS. His daughter was only 27. His three sons, one of which was Curt's great-great-great grandfather, all died in their forties of the disease. In Curtís familyís case, there is what is called an A4V mutation on the SOD-1 gene. Although doctors have identified the mutation, they do not know what triggers it, or why. And, not all persons with mutations in SOD- 1 develop ALS. There are older people with the defective gene and no symptoms, although generally by age eighty-five, eighty-five percent of persons with the mutation will have ALS. This mutation is autosomal dominant, and thus every child of a parent who has the gene has a 50% chance of inheriting the mutated gene, and thus may develop ALS.  Persons can be genetically tested for the genes that have been discovered.