ORPHANET® Ceroid Lipofuscinosis, Neuronal ORPHANET. Orphanet database access. ceroid lipofuscinosis, neuronal. Direct access to data Alias Batten disease, infantile (ceroid http://www.orpha.net/static/GB/ceroidlipofuscinosis.html
Orthoguide.com Neuronal Ceroid-Lipofuscinosis Search results for neuronal ceroidlipofuscinosis . NO MATCHES FOUND-Please select a different keyword or category OR. Search AltaVista http://www.mymedline.com/ortho/Neuronal_Ceroid-Lipofuscinosis.php3
NEURORETINAL Juvenile neuronal ceroidlipofuscinosis, or Batten disease, is a form of neuronal ceroid-lipofuscinosis that is characterized by onset of neuroretinal symptoms http://mind-brain.com/abstracts.php?qa=neuroretinal
Extractions: The neuronal ceroid-lipofuscinoses is a group of diseases characterized by a widespread accumulation in the body of pigments believed to be end-products of lipid-peroxidation damaged organelles. It was recently shown that cerebrospinal fluid from patients with infantile and juvenile neuronal ceroid-lipofuscinosis were less protective against superoxide radical-induced hydroxyl radical formation compared with controls. The content of superoxide dismutase isoenzymes in cerebrospinal fluid and in plasma from patients with different forms of neuronal ceroid-lipofuscinosis was analysed. No significant difference from controls could be demonstrated in samples from patients with juvenile neuronal ceroid-lipofuscinosis. The few samples from patients with infantile and late infantile neuronal ceroid-lipofuscinosis analysed all fell within the range defined by the controls.
Extractions: @import url(http://www.nlm.nih.gov/medlineplus/images/advanced.css); Skip navigation Other encyclopedia topics: A-Ag Ah-Ap Aq-Az B-Bk ... Z Contents of this page: Alternative names Lipofuscinoses; Batten disease; Jansky-Bielschowsky; Kufs' disease; Spielmeyer-Vogt Definition Return to top The neuronal ceroid lipofuscinoses (NCLS) are a group of rare, inherited neurodegenerative disorders. They associated with the accumulation of an abnormal pigment in the brain called lipofuscin. These disorders can be associated with severe diseases including blindness, mental retardation, and early death. There are three main types, depending on the age it begins late infantile (Jansky-Bielschowsky), juvenile (Batten disease), and adult (Kufs or Parry's disease). Causes, incidence, and risk factors Return to top Lipofuscin is the generic name of an abnormal pigment that builds up in brain cells in this group of diseases. It is a consequence of, and marker for, the disease rather than the cause of the problem. The genetic bases of multiple types of this disease are now known, but evidence indicates that there are problems in the ability of brain cells to remove and recycle brain proteins. The disorder may be evident at birth. More commonly it is diagnosed some time after the second year of life, in the teens, or as an adult. Children develop muscle incoordination (