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 ?
Approving the first drug for the treatment of this form of Batten disease is an important advance for patients suffering with this condition.
A review of the Batten Disease products under development by companies and universities/research institutes based on information derived from company and industry-specific sources.
The researchers highlighted the fact that Batten disease, which appears in children between ages 4 and 7, is caused by mutations in a gene called CLN3.
Bothuel recalls how Batten disease snuck into the life she was building for her family, when Daniel began having difficulty stepping off a sidewalk curb.
Tibetan terriers can be stricken with a neurological disease known as neuronal ceroid-lipofuscinosis (NCL), a condition which has a human equivalent called Batten disease.
Natalie is fighting a rare, degenerative brain disorder called Batten disease.
The only way physicians have been able to distinguish Batten disease from other disorders is by noticing the accumulation of certain pigments and proteins within neurons and many other cell types--an accretion that may cause the illness or be simply a by-product of the true problem.
NASDAQ: STEM) today provided an update on the ongoing clinical development program of its proprietary HuCNS-SC([R]) product candidate (purified human neural stem cells) for neuronal ceroid lipofuscinosis (NCL), often referred to as Batten disease.
Gail and Matthew Rich, from Throckley, thought they would never recover when they learned both their precious girls had Batten disease within three months of one another.
org/what-is-batten-disease/) Batten Disease Support And Research Association reported.