| Hallervorden-Spatz disease |
A slowly progressive hereditary disorder characterized by abnormal accumulations of iron pigments in certain regions of the basal ganglia (eg, globus pallidus and substantia nigra). This autosomal recessive disorder typically becomes apparent during late childhood or adolescence. ...
출처: www.dbs-stn.org/glossary1.asp
|
|---|---|
| Hallervorden-Spatz d. |
a hereditary disorder characterized by marked reduction in the number of myelin sheaths of the globus pallidus and substantia nigra, with accumulations of iron pigment, progressive rigidity beginning in the legs, choreoathetoid movements, dysarthria, and progressive mental deterioration. Transmitted as an autosomal recessive trait, it usually begins in the first or second decade, with death usually occurring before the thirtieth year. Called also status dysmyelinatus, and status dysmyelinisatus.
출처: www.mercksource.com/pp/us/cns/cns_health_library.j...
|