Janský-Bielschowsky disease

(yahn´ske byels-chof´ske) the late infantile form of neuronal ceroid lipofuscinosis , occurring between two to four years of age and characterized by abnormal accumulation of lipofuscin; it begins as myoclonic seizures and progresses to neurologic and retinal degeneration and death, usually by the age of 8 to 12 years.

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