History of Batten Disease

Batten disease, a common name for a rare class of diseases called neuronal ceroid lipofuscinoses (NCLs), was first described in 1903 by British neurologist and pediatrician Frederick Batten.

Today, there are 13 known subtypes of Batten disease. Symptoms and disease management for each subtype of Batten disease vary, although several subtypes share similar features and symptoms. Batten disease originally referred specifically to the juvenile-onset type of NCL, but now has been used more generally to describe all types of NCLs. Batten disease affects an estimated 2-4 out of every 100,000 children in the United States.

History of CLN7 (Neuronal Ceroid Lipofuscinosis 7)
CLN7 was first reported in 2004; to date, more than 70 known cases of CLN7 exist in the scientific literature. CLN7 affects children globally, across ethnicities and races, and was first diagnosed in the Turkish population.

CLN4 and CLN9 genes have still not been identified.

Sources:

  1. Batten Disease Fact Sheet. (2019, June 24). Retrieved July 31, 2019, from link.
  2. CLN5 disease. (2019, July 16). Retrieved July 31, 2019, from link.
  3. Frederick Eustace Batten. (n.d.). Retrieved July 31, 2019, from link.
  4. Batten Disease Support and Research Association. (2000). Batten Disease Neuronal Ceroid Lipofuscinosis (Publication). Retrieved August 14, 2019, from link.
  5. CLN1 Disease, infantile onset and others. (n.d.). Retrieved August 14, 2019, from link.
  6. CLN10 disease, congenital, neonatal and late infantile. (n.d.). Retrieved August 14, 2019, from link.
  7. CLN2 Disease, Late Infantile. (n.d.). Retrieved August 14, 2019, from link.
  8. CLN8 Disease, EPMR and late infantile variant. (n.d.). Retrieved August 14, 2019, from link.
  9. McKusick, V. A. (1997, April 28). CEROID LIPOFUSCINOSIS, NEURONAL, 6; CLN6. Retrieved August 14, 2019, from link.
  10. Types of Batten Disease. (n.d.). Retrieved August 14, 2019, from link.
  11. CLN7 disease. (2019, July 16). Retrieved July 31, 2019, from link.
  12. CLN7. (2010, February). Retrieved July 31, 2019, from link.
  13. CEROID LIPOFUSCINOSIS, NEURONAL, 7; CLN7. (2016, December 8). Retrieved July 31, 2019, from link.