Kufs disease

Kufs disease
the adult form of neuronal ceroid-lipofuscinosis, beginning usually before the age of 40 and characterized by progressive neurologic degeneration, excessive storage of lipofuscin in the central nervous system, and shortened life expectancy. Unlike other forms of neuronal ceroid lipofuscinosis, it does not cause blindness.

Medical dictionary. 2011.

Игры ⚽ Поможем сделать НИР

Look at other dictionaries:

  • Kufs' disease — ku̇fs n a hereditary lipid metabolism defect with onset usu. in adolescence and characterized by progressive convulsions, paralysis, ataxia, and dementia Kufs H. (1871 1955) German neurologist …   Medical dictionary

  • Kufs disease — (koofs) [H. Kufs, German psychiatrist, 1871–1955] see under disease …   Medical dictionary

  • Disease — Illness or sickness often characterized by typical patient problems (symptoms) and physical findings (signs). Disruption sequence: The events that occur when a fetus that is developing normally is subjected to a destructive agent such as the… …   Medical dictionary

  • Kufs — Hugo, German psychiatrist, 1871–1955. See K. disease …   Medical dictionary

  • Neuronal ceroid lipofuscinosis — Classification and external resources ICD 10 E75.4 ICD 9 330.1 …   Wikipedia

  • Dementia — For other uses, see Dementia (disambiguation). Dementia Classification and external resources ICD 10 F00 F07 ICD 9 …   Wikipedia

  • sphingolipidosis — Collective designation for a variety of diseases characterized by abnormal sphingolipid metabolism, e.g., gangliosidosis, Gaucher disease, Niemann Pick disease. SYN: sphingolipodystrophy. cerebral s. any one of a group of …   Medical dictionary

  • lipofuscinosis — Abnormal storage of any one of a group of fatty pigments. ceroid l. SYN: Batten disease. neuronal ceroid l. a group of diseases characterized by accumulation of abnormal pigments in tissue (previously classified as …   Medical dictionary

  • CLN5 — Ceroid lipofuscinosis, neuronal 5, also known as CLN5, is a human gene.cite web | title = Entrez Gene: CLN5 ceroid lipofuscinosis, neuronal 5| url = http://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene Cmd=ShowDetailView TermToSearch=1203| accessdate …   Wikipedia

  • ceroid-lipofuscinosis — neuronal ceroid lipofuscinosis a term for several genetic lipidoses of diverse biochemical and clinical characteristics, all characterized by progressive neurodegeneration, loss of vision, and a fatal course; the infantile type is Haltia… …   Medical dictionary

Share the article and excerpts

Direct link
Do a right-click on the link above
and select “Copy Link”