Synonyms
Adult-Onset Ceroidosis
Amaurotic Familial Idiocy, Adult
Ceroid-Lipofuscinosis, Adult form
Generalized Lipofuscinosis
Neuronal Ceroid Lipofuscinosis, Adult Type

Disorder Subdivisions
None

General Discussion
Kufs Disease is characterized by neurologic symptoms that may mimic mental illness, movement malfunction, and problems with sight. Kufs Disease is linked to excess accumulations of pigments (lipofuscins) dissolved in fat tissues that are found throughout the central nervous system. Kufs Disease, Batten Disease, Bielchowsky Disease, and Santavuori-Haltia Disease are different forms of the same family of disorders (neuronal ceroid lipofuscinoses [NCL]) that are differentiated by the age of onset. The various forms of this disorder are often extremely difficult to differentiate from other progressive degenerative diseases of the central nervous system.