Batten disease(redirected from juvenile neuronal ceroid lipofuscinosis)
more generally, any or all of the group of disorders constituting neuronal ceroid lipofuscinosis.
cerebral sphingolipidosis, late juvenile type.
Synonym(s): ceroid lipofuscinosis
Any of the various types of neuronal ceroid lipofuscinosis, especially the juvenile type with onset typically between ages four and eight (also known as Spielmeyer-Vogt disease).
1 Also called Vogt-Spielmeyer disease.
2 more generally, any or all of the group of disorders constituting neuronal ceroid lipofuscinosis.
Batten diseaseA rare autosomal recessive neurodegenerative condition which is the most common form of the eight known neuronal ceroid lipofuscinoses. Batten disease is caused by a mutation in CLN3, a gene on chromosome 16 that encodes battenin, a protein involved in lysosomal function.
Blindness, seizures, dementia, cerebral atrophy, death before age 20.
Bat·ten dis·ease(bat'ĕn di-zēz')
Fatal autosomal recessive trait with onset between 5-9 years of age, marked by early symptoms, which progress to blindness, paralysis, and dementia.
Batten,Frederick Eustace, English ophthalmologist, 1865-1918.
Batten disease - cerebral sphingolipidosis, late infantile and juvenile types. Synonym(s): Batten-Mayou disease; Spielmeyer-Stock disease; Spielmeyer-Vogt disease; Vogt-Spielmeyer disease
Batten-Mayou disease - Synonym(s): Batten disease
Curschmann-Batten-Steinert syndrome - see under Curschmann
Spielmeyer,Walter, German neurologist, 1879-1935.
Spielmeyer acute swelling - a form of degeneration of nerve cells in which the cell body and its processes swell and stain palely and diffusely.
Spielmeyer-Stock disease - Synonym(s): Batten disease
Spielmeyer-Vogt disease - Synonym(s): Batten disease
Vogt-Spielmeyer disease - Synonym(s): Batten disease
see ceroid lipofuscinosis.