How is batten disease inherited?

Neuronal ceroid lipofuscinoses (NCLs), also called Batten disease, are a group of inherited neurodegenerative disorders that are characterized by the accumulation of autofluorescent storage material within the lysosomes of neurons and other cell types. The most common form of NCL is CLN1 disease, which is inherited in an autosomal recessive manner. This means that both copies of the CLN1 gene must be defective in order for the disease to develop.

Other forms of NCL are inherited in an autosomal dominant manner, which means that only one defective copy of the gene is needed for the disease to develop. Some forms of NCL are also inherited in a X-linked manner, which means that the defective gene is located on the X chromosome.