Jansky-Bielschowsky disease /yahn′skē byelschov′ske/ [Jan Janský, Czech physician, 1873–1921, and Max Bielschowsky, German neuropathologist, 1869–1940] , the late infantile form of neuronal ceroid lipofuscinosis, occurring between 2 and 4 years of age and characterized by abnormal accumulation of lipofuscin. It begins as myoclonic seizures and progresses to neurological and retinal degeneration and death, usually by the age of 8 to 12 years.
