Batten disease


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Batten disease

 [bat´en]
more generally, any or all of the group of disorders constituting neuronal ceroid lipofuscinosis.

Bat·ten dis·ease

(bat'ĕn),
cerebral sphingolipidosis, late juvenile type.

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).

Batten disease

[bat′en]
1 Also called Vogt-Spielmeyer disease.
2 more generally, any or all of the group of disorders constituting neuronal ceroid lipofuscinosis.

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.

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

Batten disease

see ceroid lipofuscinosis.
References in periodicals archive ?
We're working as quickly as possible to find a cure for Batten disease and launch the carrier screening test so people planning to have children can know their risks for transmitting hundreds of horrible conditions; then, they can evaluate their options.
Children afflicted by Batten disease are gravely ill, and this family's courage advances the hope that this trial will lead to a treatment for this devastating disease.
By delivering a working version of a gene to produce a key enzyme that is lacking in Batten disease, the scientists delayed symptoms and extended lifespan in dogs with a comparable disease.
SUPPORT Paul, Claire, Rebekah, Aidan and Connor COURAGEOUS Aidan was battling Batten disease
uk For information on Batten Disease, visit the Batten Disease Family Association website at www.
SUPPORT J Liam Heffernan who suffers from Batten disease with RTE's Miriam O'Callaghan VISION n Artists impression of centre in Co Kerry
AUSTIN, Texas, March 19, 2013 /PRNewswire/ -- The Batten Disease Support and Research Association (BDSRA) and Beyond Batten Disease Foundation (BBDF), working together in close partnership with more than 20 other nonprofits around the world, today announced grants totaling almost $1 million for research to develop treatments and a cure for Batten disease.
Craig Benson got interested in carrier screening for recessive genetic conditions after he and his wife found out that their young daughter had Batten disease, a rare neurodegenerative disease affecting about 1 in 100,000 people.
Batten disease is named after the British paediatrician Frederick Batten, who first described it in 19 0 3 .
Washington, December 22 (ANI): Researchers at the University College London say that it is possible to predict the severity of Batten Disease, a fatal nervous system disorder that begins during childhood, by expressing human gene mutations in yeast cells.
Daniel was born with Batten disease, an inherited and fatal neurological disorder that affects just two to four out of every 100,000 births.
Bennett has also devoted 25 years to studying a group of untreatable and devastating inherited neurodegenerative diseases known collectively as Batten disease.