Main Entry: Bat·ten disease

Pronunciation: \ˈbat-^ən-\

Function: noun

: a fatal lipofuscinosis that is inherited as an autosomal recessive trait, has an onset between five and eight or nine years of age, and is marked by early symptoms which progress to blindness, paralysis, and dementia

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 poliomyelitis in 1916.