Batten disease

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Related to Batten's Disease: Spielmeyer-Vogt-Sjögren-Batten disease

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

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
References in periodicals archive ?
In August 2016 they received the devastating news that Kaycee had rare Batten's disease.
Eventually Saoirse was diagnosed with Batten's Disease - a moment the Heffernans will never forget.
People with Batten's Disease are affected in different ways but common symptoms t include visual impairment resulting in blindness, epilepsy, muscle spasms, decline of speech, language and swallowing skills and the loss of mobility.
"At the time, doctors in Yorkhill had only seen one other case of infantile Batten's disease.
The two children had a rare and incurable neuro-degenerative disorder called late-infantile Batten's disease. They died in 1998 and 1999.
It was set up by Barry and Carmen Daniels who experienced the need for 'home from home' care when their children Charly and Rhys, who later died, were in hospital with Batten's disease.
Jackie and her husband, welder Kelvin, were devastated when doctors told them their then five-year-old daughter Llinos had been diagnosed with the rare and incurable Batten's disease.
The film was mentioned in the House of Commons and parliamentary questions were raised about the funding given to Late Infantile Batten's disease. A second part of the movie is a comedy, Hard day at Office Love?, which contains a host of celebrity cameos.
"The trust is a children's charity set up after the death last Christmas from Batten's Disease of Rhys Daniels, whose parents I know," he said.
Lovejoy star Ian McShane, left, was among the mourners comforting the father of brave little Charly Daniels, the ten-year-old Batten's disease victim who was buried yesterday after losing her battle against the same crippling brain condition that claimed her brother.