Childhood<unk> s are generally autosomal recessive disorders; that is, they occur only when a child inherits two copies of the defective gene, one from each parent. When both parents carry one defective gene, each of their children faces a one in four chance of developing<unk> . At the same time, each child also faces a one in two chance of inheriting just one copy of the defective gene. Individuals who have only one defective gene are known as carriers, meaning they do not develop the disease, but they can pass the gene on to their own children. The most commonly identified mutations are in the CLN3 gene, which is located on the short arm of chromosome 16 (16p12.1). The normal function of the gene is not presently known, but results in a transmembrane protein. Adult<unk>  may be inherited as an autosomal recessive (Kufs), or less often, as an autosomal dominant (Parry's) disorder. In autosomal dominant inheritance, all people who inherit a single copy of the disease gene develop the disease. As a result, no carriers of the gene are unaffected. Many authorities refer to the<unk> s collectively as Batten disease.
Neuronal ceroid lipofuscinosis
Tay–Sachs disease
Pantothenate kinase-associated neurodegeneration
Infantile neuronal ceroid lipofuscinosis
Glycogen storage disease
Granulovacuolar degeneration
Cerebral creatine deficiency
Familial encephalopathy with neuroserpin inclusion bodies