Syndrome, Alpers

Syndrome, 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. Alpers syndrome (or 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 Alpers progressive infantile poliodystrophy, progressive infantile poliodystrophy, diffuse degeneration of cerebral gray matter with hepatic cirrhosis and Alpers diffuse degeneration of cerebral gray matter with hepatic cirrhosis.

Medical dictionary. 2011.

Игры ⚽ Поможем решить контрольную работу

Look at other dictionaries:

  • Alpers' disease — Infobox Disease Name = Alpers disease Caption = DiseasesDB = 29298 ICD10 = ICD10|G|31|8|g|30 ICD9 = ICD9|330.8 ICDO = OMIM = 203700 MedlinePlus = eMedicineSubj = eMedicineTopic = MeshID = D002549 Alpers disease, also called Alpers syndrome,Naudé …   Wikipedia

  • Alpers syndrome — Al·pers syndrome (alґpərz) [Bernard Jacob Alpers, American neurologist, 1900–1981] see under syndrome …   Medical dictionary

  • Rett syndrome — Classification and external resources ICD 10 F84.2 ICD 9 330.8 …   Wikipedia

  • Neuroleptic malignant syndrome — Classification and external resources ICD 10 G21.0 ICD 9 333.92 …   Wikipedia

  • Stiff person syndrome — (SPS) (or stiff man syndrome; also known as Stiffperson s Syndrome or Moersch Woltman Condition) is a rare neurologic disorder of unknown etiology characterized by progressive rigidity and stiffness, primarily of the axial musculature, that is… …   Wikipedia

  • Morvan's syndrome — Classification and external resources ICD 10 G60.8 ICD 9 336.0 …   Wikipedia

  • Meige's syndrome — Classification and external resources ICD 10 G24.4 ICD 9 333.82 …   Wikipedia

  • Ramsay Hunt syndrome type I — For other uses, see Ramsay Hunt syndrome. Ramsay Hunt syndrome type I Classification and external resources ICD 10 G11.1 ICD 9 …   Wikipedia

  • Clinically isolated syndrome — A clinically isolated syndrome (CIS) is an individual s first neurological episode, caused by inflammation or demyelination of nerve tissue. An episode may be monofocal, in which symptoms present at a single site in the central nervous system, or …   Wikipedia

  • Cerebellopontine angle syndrome — Classification and external resources ICD 9 191.6 Neoplasms of brain: Cerebellum NOS: Cerebellopontine angle The cerebellopontine angle is the anatomic space between the cerebellum and the pons. This is a common site for the growth of acoustic… …   Wikipedia

Share the article and excerpts

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