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Gerstmann-Straussler-Scheinker syndrome
Gerstmann-Straussler-Scheinker syndromeA rare familial form of progressive dementia inherited in an autosomal dominant manner due to a mutant prion gene on chromosome 20pter-p12. Abbreviated GSS. Degeneration of the nervous system usually starts in the fourth or fifth decade of life with slowly developing dysarthria (difficulty speaking) and cerebellar ataxia (wobbliness) and later the progressive dementia become evident. Death usually occurs within 10 years of the onset of symptoms. RELATED TERMS-------------------------------------- Familial A condition that is tends to occur more often in family members than expected by chance alone. A familial disease may be genetic (such as cystic fibrosis) or environmental (such as tuberculosis). Dementia The loss, usually progressive, of cognitive and intellectual functions, without impairment of perception or consciousness; caused by a variety of disorders, most commonly structural brain disease. Characterized by disorientation, impaired memory, impaired judgment and lowered intellect. Autosomal "Pertaining to a chromosome that is not a sex chromosome; relating to any one of the chromosomes save the sex chromosomes. People normally have 22 pairs of autosomes (44 autosomes) in each cell together with two sex chromosomes (X and Y in the male and XX in the female). " Dominant A genetic trait is considered dominant if it is expressed in a person who has only one copy of that gene. (In genetic terms, a dominant trait is one that is phenotypically expressed in heterozygotes). A dominant trait is opposed to a recessive trait which is expressed only when two copies of the gene are present. (In genetic terms, a recessive trait is one that is phenotypically expressed only in homozygotes). Gene 1. A unit of DNA that carries information for the biosynthesis of a specific product in the cell. 2. Ultimate unit by which inheritable characteristics are transmitted to succeeding generations in all living organisms. Genes are contained by, and arranged along the length of, the chromosome. The gene is composed of deoxyribonucleic acid (DNA). Each chromosome of each species has a definite number and arrangement of genes, which govern both the structure and metabolic functions of the cells and thus of the entire organism. Chromosome A structural unit within a eukaryotic nucleus that carries genes. A chromosome consists of a long, continuous strand of DNA and associated proteins. Dysarthria Imperfect articulation of speech due to disturbances of muscular control or incoordination. Cerebellar Pertaining to the cerebellum, the part of the brain in the back of the head between the cerebrum and the brain stem. The cerebellum controls balance for walking and standing and other complex motor functions. Ataxia Ataxia is unsteady and clumsy motion of the limbs or trunk due to a failure of the fine coordination of muscle movements. The cerebellum is the center of the fine coordination of muscle movements, while the information emerging from it is carried to the muscles by the spinal cord and peripheral nerves, respectively. Therefore a disorder in either the spinal cord or in the peripheral nerves can cause ataxia. Death 1. The end of life. The cessation of life. (These common definitions of death ultimately depend upon the definition of life, upon which there is no consensus.) 2. The permanent cessation of all vital bodily functions. (This definition depends upon the definition of "vital bodily functions.") See: Vital bodily functions. 3. The common law standard for determining death is the cessation of all vital functions, traditionally demonstrated by "an absence of spontaneous respiratory and cardiac functions." 4. The uniform determination of death. SIMILAR TERMS-------------------------------------- PREVIOUS AND NEXT TERMS-------------------------------------- German measles immunization The standard MMR vaccine is given to prevent measles, mumps and rubella (German measles). The MMR vaccine is now given in two dosages. The first should be given at 12-15 months of age. The second vaccination should be given at 4-6 years. Most children should receive MMR vaccinations. Exceptions may include children born with an inability to fight off infection, some children with cancer, on treatment with radiation or drugs for cancer, on long term steroids (cortisone). People with severe allergic reactions to eggs or the drug neomycin should probably avoid the MMR vaccine. Pregnant women should wait until after delivery before being immunized with MMR. People with HIV or AIDS should normally receive MMR vaccine. Measles, mumps, and rubella vaccines may be administered as individual shots, if necessary, or as a measles- rubella combination. German measles vaccine A vaccine designed to prevent rubella, or German measles. The rubella vaccine has since been refined into the vaccine now known as MMR for mumps, measles and rubella. The congenital rubella syndrome is now largely a chapter in the history of medicine, thanks to the rubella vaccine. Germanium A nonessential trace element that has caused nephrotoxicity (kidney injury) and death when used chronically by humans, even at recommended levels of use. Germinoma A type of germ cell tumor. Germline mutation A heritable change in the DNA that occurred in a germ cell (a cell destined to become an egg or in the sperm) or the zygote (the conceptus) at the single-cell stage. When transmitted to a child, a germline mutation is incorporated in every cell of their body. Germline mutations play a key role in genetic diseases. They play a role, too, in certain types of cancer as, for example, the eye tumor retinoblastoma and Wilms tumor, a childhood malignancy of the kidney. A germline mutation is in contrast to a somatic mutation which is acquired in a single body cell. Gerstmann-Straussler-Scheinker syndrome Gestalt therapy A psychotherapeutic concept that stresses understanding mental processes as holistic entities (gestalts) rather than as discrete steps. Gestalt therapy often uses group therapy techniques to help patients gain this type of insight. Gestation period The period of development of the young from the time of conception until birth. For humans the full gestation period is normally 9 months. GFAP Glial fibrillary acidic protein. The degenerative brain condition called Alexander disease is caused by mutation in GFAP (glial fibrillary acidic protein). The GFAP gene provides instructions for making GFAP protein, a member of the intermediate filament family that provides support and strength to cells. Several molecules of GFAP protein bind together to form the main intermediate filament found in specialized brain cells called astrocytes. Astrocytes are star-shaped cells that support the functions of nerve cells in the brain and spinal cord (central nervous system). If the central nervous system is injured through trauma or disease, astrocytes react by rapidly producing more GFAP. GFP Green fluorescent protein. A protein that glows green under fluorescent light. Found naturally in the jellyfish Aequorea victoria, GFP fluoresces green when exposed to blue light. It has a sequence of three amino acids (serine-tyrosine-glycine) which is responsible for its fluorescence. GI 1. In medicine, commonly used abbreviation for gastrointestinal, referring collectively to the stomach and the small and large intestine. 2. Outside of medicine, GI may stand for galvanized iron, general issue or government issue (as in GI Joe). We thank you for using the Medical Glossary to search for Gerstmann-Straussler-Scheinker syndrome. If you have a better definition for Gerstmann-Straussler-Scheinker syndrome than the one presented here, please let us know by making use of the suggest a term option. This definition of Gerstmann-Straussler-Scheinker syndrome may be disputed by other professionals. Our attempt is to provide easy definitions on Gerstmann-Straussler-Scheinker syndrome and any other medical topic for the public at large.
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syndrome / gerstmxnn-straussler-scheinker syndrome / gerstmznn-straussler-scheinker syndrome / gerstmabn-straussler-scheinker syndrome / gerstmahn-straussler-scheinker syndrome / gerstmajn-straussler-scheinker syndrome / gerstmamn-straussler-scheinker syndrome / gerstma n-straussler-scheinker syndrome / gerstmanb-straussler-scheinker syndrome / gerstmanh-straussler-scheinker syndrome / gerstmanj-straussler-scheinker syndrome / gerstmanm-straussler-scheinker syndrome / gerstman -straussler-scheinker syndrome / gerstmann=straussler-scheinker syndrome / gerstmann[straussler-scheinker syndrome / gerstmannpstraussler-scheinker syndrome / gerstmann0straussler-scheinker syndrome / gerstmann-wtraussler-scheinker syndrome / gerstmann-etraussler-scheinker syndrome / gerstmann-dtraussler-scheinker syndrome / gerstmann-xtraussler-scheinker syndrome / gerstmann-ztraussler-scheinker syndrome / gerstmann-atraussler-scheinker syndrome / gerstmann-qtraussler-scheinker syndrome / gerstmann-s5raussler-scheinker syndrome / gerstmann-s6raussler-scheinker syndrome / gerstmann-syraussler-scheinker syndrome / gerstmann-shraussler-scheinker syndrome / gerstmann-sgraussler-scheinker syndrome / gerstmann-sfraussler-scheinker syndrome / gerstmann-srraussler-scheinker syndrome / gerstmann-s4raussler-scheinker syndrome / gerstmann-st4aussler-scheinker syndrome / gerstmann-st5aussler-scheinker syndrome / gerstmann-sttaussler-scheinker syndrome / gerstmann-stgaussler-scheinker syndrome / gerstmann-stfaussler-scheinker syndrome / gerstmann-stdaussler-scheinker syndrome / gerstmann-steaussler-scheinker syndrome / gerstmann-st3aussler-scheinker syndrome / gerstmann-strqussler-scheinker syndrome / gerstmann-strwussler-scheinker syndrome / gerstmann-strsussler-scheinker syndrome / gerstmann-strxussler-scheinker syndrome / gerstmann-strzussler-scheinker syndrome / gerstmann-stra7ssler-scheinker syndrome / gerstmann-stra8ssler-scheinker syndrome / gerstmann-straissler-scheinker syndrome / gerstmann-strakssler-scheinker syndrome / gerstmann-strajssler-scheinker syndrome / gerstmann-strahssler-scheinker syndrome / gerstmann-strayssler-scheinker syndrome / gerstmann-stra6ssler-scheinker syndrome / gerstmann-strauwsler-scheinker syndrome / gerstmann-strauesler-scheinker syndrome / gerstmann-straudsler-scheinker syndrome / gerstmann-strauxsler-scheinker syndrome / gerstmann-strauzsler-scheinker syndrome / gerstmann-strauasler-scheinker syndrome / gerstmann-strauqsler-scheinker syndrome / gerstmann-strauswler-scheinker syndrome / gerstmann-strauseler-scheinker syndrome / gerstmann-strausdler-scheinker syndrome / gerstmann-strausxler-scheinker syndrome / gerstmann-strauszler-scheinker syndrome / gerstmann-strausaler-scheinker syndrome / gerstmann-strausqler-scheinker syndrome / gerstmann-straussoer-scheinker syndrome / gerstmann-straussper-scheinker syndrome / gerstmann-strauss;er-scheinker syndrome / gerstmann-strauss.er-scheinker syndrome / gerstmann-strauss,er-scheinker syndrome / gerstmann-straussker-scheinker syndrome / gerstmann-straussier-scheinker syndrome / gerstmann-straussl3r-scheinker syndrome / gerstmann-straussl4r-scheinker syndrome / gerstmann-strausslrr-scheinker syndrome / gerstmann-strausslfr-scheinker syndrome / gerstmann-straussldr-scheinker syndrome / gerstmann-strausslsr-scheinker syndrome / gerstmann-strausslwr-scheinker syndrome / gerstmann-straussle4-scheinker syndrome / gerstmann-straussle5-scheinker syndrome / gerstmann-strausslet-scheinker syndrome / gerstmann-straussleg-scheinker syndrome / gerstmann-strausslef-scheinker syndrome / gerstmann-straussled-scheinker syndrome / gerstmann-strausslee-scheinker syndrome / gerstmann-straussle3-scheinker syndrome / gerstmann-straussler=scheinker syndrome / gerstmann-straussler[scheinker syndrome / gerstmann-strausslerpscheinker syndrome / gerstmann-straussler0scheinker syndrome / gerstmann-straussler-wcheinker syndrome / gerstmann-straussler-echeinker syndrome / gerstmann-straussler-dcheinker syndrome / gerstmann-straussler-xcheinker syndrome / gerstmann-straussler-zcheinker syndrome / gerstmann-straussler-acheinker syndrome / gerstmann-straussler-qcheinker syndrome / gerstmann-straussler-sxheinker syndrome / gerstmann-straussler-ssheinker syndrome / gerstmann-straussler-sdheinker syndrome / gerstmann-straussler-sfheinker syndrome / gerstmann-straussler-svheinker syndrome / gerstmann-straussler-s heinker syndrome / gerstmann-straussler-scyeinker syndrome / gerstmann-straussler-scueinker syndrome / gerstmann-straussler-scjeinker syndrome / gerstmann-straussler-scneinker syndrome / gerstmann-straussler-scbeinker syndrome / gerstmann-straussler-scgeinker syndrome / gerstmann-straussler-scteinker syndrome / gerstmann-straussler-sch3inker syndrome / gerstmann-straussler-sch4inker syndrome / gerstmann-straussler-schrinker syndrome / gerstmann-straussler-schfinker syndrome / gerstmann-straussler-schdinker syndrome / gerstmann-straussler-schsinker syndrome / gerstmann-straussler-schwinker syndrome / gerstmann-straussler-schenker syndrome / gerstmann-straussler-scheibker syndrome / gerstmann-straussler-scheihker syndrome / gerstmann-straussler-scheijker syndrome / gerstmann-straussler-scheimker syndrome / gerstmann-straussler-schei ker syndrome / gerstmann-straussler-scheiner syndrome / gerstmann-straussler-scheink3r syndrome / gerstmann-straussler-scheink4r syndrome / gerstmann-straussler-scheinkrr syndrome / gerstmann-straussler-scheinkfr syndrome / gerstmann-straussler-scheinkdr syndrome / gerstmann-straussler-scheinksr syndrome / gerstmann-straussler-scheinkwr syndrome / gerstmann-straussler-scheinke4 syndrome / gerstmann-straussler-scheinke5 syndrome / gerstmann-straussler-scheinket syndrome / gerstmann-straussler-scheinkeg syndrome / gerstmann-straussler-scheinkef syndrome / gerstmann-straussler-scheinked syndrome / gerstmann-straussler-scheinkee syndrome / gerstmann-straussler-scheinke3 syndrome / gerstmann-straussler-scheinker wyndrome / gerstmann-straussler-scheinker eyndrome / gerstmann-straussler-scheinker dyndrome / gerstmann-straussler-scheinker xyndrome / gerstmann-straussler-scheinker zyndrome / gerstmann-straussler-scheinker ayndrome / gerstmann-straussler-scheinker qyndrome / gerstmann-straussler-scheinker s6ndrome / gerstmann-straussler-scheinker s7ndrome / gerstmann-straussler-scheinker sundrome / gerstmann-straussler-scheinker sjndrome / gerstmann-straussler-scheinker shndrome / gerstmann-straussler-scheinker sgndrome / gerstmann-straussler-scheinker stndrome / gerstmann-straussler-scheinker s5ndrome / gerstmann-straussler-scheinker sybdrome / gerstmann-straussler-scheinker syhdrome / gerstmann-straussler-scheinker syjdrome / gerstmann-straussler-scheinker symdrome / gerstmann-straussler-scheinker sy drome / gerstmann-straussler-scheinker synerome / gerstmann-straussler-scheinker synrrome / gerstmann-straussler-scheinker synfrome / gerstmann-straussler-scheinker synvrome / gerstmann-straussler-scheinker syncrome / gerstmann-straussler-scheinker synxrome / gerstmann-straussler-scheinker synsrome / gerstmann-straussler-scheinker synwrome / gerstmann-straussler-scheinker synd4ome / gerstmann-straussler-scheinker synd5ome / gerstmann-straussler-scheinker syndtome / gerstmann-straussler-scheinker syndgome / gerstmann-straussler-scheinker syndfome / gerstmann-straussler-scheinker synddome / gerstmann-straussler-scheinker syndeome / gerstmann-straussler-scheinker synd3ome / gerstmann-straussler-scheinker syndr9me / gerstmann-straussler-scheinker syndr0me / gerstmann-straussler-scheinker syndrpme / gerstmann-straussler-scheinker syndrlme / gerstmann-straussler-scheinker syndrkme / gerstmann-straussler-scheinker syndrime / gerstmann-straussler-scheinker syndr8me / gerstmann-straussler-scheinker syndrone / gerstmann-straussler-scheinker syndroje / gerstmann-straussler-scheinker syndroke / gerstmann-straussler-scheinker syndro,e / gerstmann-straussler-scheinker syndro e / gerstmann-straussler-scheinker syndrom3 / gerstmann-straussler-scheinker syndrom4 / gerstmann-straussler-scheinker syndromr / gerstmann-straussler-scheinker syndromf / gerstmann-straussler-scheinker syndromd / gerstmann-straussler-scheinker syndroms / gerstmann-straussler-scheinker syndromw / | ||||||||||||||||