Batten disease

(redirected from type-3 neuronal ceroid lipofuscinosis)

Batten disease

 [bat´en]
more generally, any or all of the group of disorders constituting neuronal ceroid lipofuscinosis.
Miller-Keane Encyclopedia and Dictionary of Medicine, Nursing, and Allied Health, Seventh Edition. © 2003 by Saunders, an imprint of Elsevier, Inc. All rights reserved.

Bat·ten dis·ease

(bat'ĕn),
cerebral sphingolipidosis, late juvenile type.
Farlex Partner Medical Dictionary © Farlex 2012

Batten disease

(băt′n)
n.
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).
The American Heritage® Medical Dictionary Copyright © 2007, 2004 by Houghton Mifflin Company. Published by Houghton Mifflin Company. All rights reserved.

Batten disease

A 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.

Clinical findings
Blindness, seizures, dementia, cerebral atrophy, death before age 20.
Segen's Medical Dictionary. © 2012 Farlex, Inc. All rights reserved.

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.
Medical Dictionary for the Health Professions and Nursing © Farlex 2012

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
Medical Eponyms © Farlex 2012