Batten disease is a fatal disease of the nervous system that typically begins in childhood.[1] Onset of symptoms is usually between 5 and 10 years of age.[1] Often, it is autosomal recessive. It is the common name for a group of disorders called the neuronal ceroid lipofuscinoses (NCLs).[1]
Although Batten disease is usually regarded as the juvenile form of NCL (or "type 3"), some physicians use the term Batten disease to describe all forms of NCL. Historically, the NCLs were classified by age of disease onset as infantile NCL (INCL), late infantile NCL (LINCL), juvenile NCL (JNCL) or adult NCL (ANCL).[2] At least 20 genes have been identified in association with Batten disease, but juvenile NCL, the most prevalent form of Batten disease, has been linked to mutations in Battenin, the protein encoded by the CLN3 gene.[3][4] It was first described in 1903.[1]
^ abcdefg"Batten Disease Fact Sheet | National Institute of Neurological Disorders and Stroke". www.ninds.nih.gov. Retrieved 30 November 2020.
^Hobert JA, Dawson G (October 2006). "Neuronal ceroid lipofuscinoses therapeutic strategies: past, present and future". Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease. 1762 (10): 945–53. doi:10.1016/j.bbadis.2006.08.004. PMID 17049436.
^Rakheja D, Narayan SB, Bennett MJ (September 2007). "Juvenile neuronal ceroid-lipofuscinosis (Batten disease): a brief review and update". Current Molecular Medicine. 7 (6): 603–8. doi:10.2174/156652407781695729. PMID 17896996.
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death for late infantile variant is 10–12 years. Juvenile NCL (JNCL, Battendisease, or Spielmeyer-Vogt), with a prevalence of one in 100,000, usually arises...
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neurodegenerative diseases commonly known as Battendisease, also known as Juvenile Neuronal Ceroid Lipofuscinosis (JNCL) or Juvenile Battendisease. GRCh38: Ensembl...
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between the ages of 2 and 8 years old. Neuro-degenerative diseases (disorders like Battendisease) can result in a child losing previously acquired motor...
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1908. Battendisease was named after him after he first described it in 1903. Batten died from infection after a routine prostatectomy. Battendisease E....
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