Batten disease(redirected from Batten's Disease)
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Related to Batten's Disease: Spielmeyer-Vogt-Sjögren-Batten disease
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).
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