Medical Dictionary

Batten disease

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noun Bat·ten disease \ˈbat-ən-\
variants: also

Batten's disease

Medical Definition of Batten disease

  1. :  a fatal genetic neurodegenerative disease that is characterized by seizures, progressive loss of vision, and mental impairment progressing to blindness, paralysis, and dementia and that has an onset during childhood with death typically occurring during the late teens and twenties; also :  any of a group of related diseases Batten disease is a lipofuscinosis marked by the abnormal accumulation of lipofuscin in the brain. The most common juvenile form is inherited as an autosomal recessive trait requiring that both parents pass on a copy of the defective gene on a chromosome other than a sex chromosome.

Biographical Note for batten disease

Batten

,

Frederick Eustace

(1865–1918), British neurologist and pediatrician. In London, Batten held posts at the Hospital for Sick Children and at the National Hospital for the Paralysed and Epileptic. His research centered on the histology of disorders of the nervous system. In 1902 he published the first description of a fatal form of lipofuscinosis that is often known as Batten disease. Additional descriptions of the disease were separately published by the German neurologists Heinrich Vogt in 1905 and Walter Spielmeyer in 1908. Batten is also remembered for his 1903 description of a benign form of congenital muscular dystrophy. The author of more than 100 medical papers, he published a book on acute polio in 1916.


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