Aug 30, · Adult neuronal ceroid lipofuscinosis is a rare condition that affects the nervous system. Signs and symptoms usually begin around age 30, but they can develop anytime between adolescence and late adulthood. Adult neuronal ceroid lipofuscinosis (ANCL) is a general term for several rare genetic disorders that belong to a group of progressive, degenerative neurometabolic disorders known as the neuronal ceroid lipofuscinoses (NCLs). These disorders share certain similar symptoms and are distinguished in part by the age at which such symptoms appear.
20 rows · Sep 07, · Neuronal ceroid lipofuscinosis (NCL) refers to a group of conditions that . Adult-onset neuronal ceroid lipofuscinosis, also known as Kufs disease, is a neurodegenerative disorder without retinal involvement. There are 2 overlapping phenotypes: type A, characterized by progressive myoclonic epilepsy, and type B, characterized by dementia and a variety of motor-system signs (summary by Arsov et al., ).
Adult neuronal ceroid-lipofuscinosis. Goebel HH(1), Braak H. Author information: (1)Division of Neuropathology, University of Mainz, FRG. Among the different clinical forms of neuronal ceroid-lipofuscinosis (NCL), the adult type is the least frequent, most Cited by: The neuronal ceroid lipofuscinoses (NCLs) are a heterogeneous group of hereditary neurodegenerative disorders in which progressive tonic–clonic and myoclonic seizures as well as progressive cognitive decline are associated with abnormal lipopigments from lysosomal inclusion bodies in Cited by:
The neuronal ceroid lipofuscinoses (NCLs or Batten disease) is the collective name for a group of at least 10 fatal inherited lysosomal storage disorders, which affect mostly children and young adults.