Cirrhosis and gray matter degeneration
Cirrhosis with diffuse degeneration of cerebral gray matter, Alpers: A progressive disease of the nervous system characterized by spasticity (tightness), myoclonus and dementia and by liver problems with jaundice and cirrhosis. This disorder, first described by Alpers in 1931 as “Diffuse progressive degeneration of gray matter of cerebrum”, usually begins early in life with convulsions. A continuous seizure (status epilepticus) is often the final event.
This disease is due to more than one cause. Some cases are inherited as autosomal recessive traits with both parents appearing normal but carrying one Alpers gene and each of their children, boys and girls alike, running a 1 in 4 risk of receiving both of the parental Alpers genes and suffering from this dread disease.
Other cases of Alpers disease are due to disorders of oxidative phosphorylation, including mitochondrial DNA depletion syndromes. (Phosphorylation is the addition of phosphate to an organic compound, such as the addition of phosphate to ADP [adenosine diphosphate] to form ATP [adenosine triphosphate] or the addition of phosphate to glucose to produce glucose monophosphate, through the action of enzymes known as phosphotransferases or kinases.)
Alpers disease is also called progressive infantile poliodystrophy, and Alpers progressive infantile poliodystrophy.
Read Also:
- Cisplatin
Cisplatin: An anticancer drug that belongs to the family of drugs called platinum compounds. Cisplatin is used in the treatment of a wide range of malignancies, including advanced cancer of the ovary, testis, and bladder. Cisplatin is given intravenously. Its full chemical name is cis-dichlorodiammineplatinum.
- Citrulline
Citrulline: A non-standard amino acid that is not normally present in protein. Citrulline is created in the body as an intermediate in the conversion of the amino acid ornithine to arginine in a metabolic pathway called the urea cycle. Citrulline was first isolated from watermelon. The term citrulline was coined in 1930 from citrullus, the […]
- Citrulline antibody
An immune protein (antibody) that binds to a non-standard amino acid, citrulline, which is formed by removing amino groups from the natural amino acid, arginine. Citrulline antibody is present in the blood of many patients with rheumatoid arthritis. It is used in the diagnosis of rheumatoid arthritis when evaluating patients with unexplained joint inflammation. Also […]
- Civilian Health and Medical Program of the Uniform
Civilian Health and Medical Program of the Uniformed Services: See: CHAMPUS.
- Cl (chloride)
Cl (chloride): Cl is the chemical symbol for chloride, the major anion found in the fluid outside of cells and in blood. An anion is the negatively charged part of certain substances such as table salt (sodium chloride or NaCl) when dissolved in liquid. The balance of chloride ion (Cl-) is closely regulated by the […]