GS
MCID: GRS001
MIFTS: 29

Gerstmann Syndrome (GS) malady

Neuronal diseases category

Summaries for Gerstmann Syndrome

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43NINDS, 63Wikipedia, 32MalaCards
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NINDS:43 Gerstmann's syndrome is a cognitive impairment that results from damage to a specific area of the brain -- the left parietal lobe in the region of the angular gyrus. It may occur after a stroke or in association with damage to the parietal lobe. It is characterized by four primary symptoms: a writing disability (agraphia or dysgraphia), a lack of understanding of the rules for calculation or arithmetic (acalculia or dyscalculia), an inability to distinguish right from left, and an inability to identify fingers (finger agnosia). The disorder should not be confused with , a type of transmissible spongiform encephalopathy.

MalaCards: Gerstmann Syndrome, also known as developmental gerstmann's syndrome, is related to pseudohypoparathyroidism and mccune albright syndrome. An important gene associated with Gerstmann Syndrome is TBX1 (T-box 1). Affiliated tissues include brain and parietal lobe.

Wikipedia:63 Gerstmann syndrome is a neuropsychological disorder that is characterized by a constellation of symptoms... more...

Aliases & Classifications for Gerstmann Syndrome

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8Disease Ontology, 42NIH Rare Diseases, 43NINDS, 48Orphanet, 60UMLS, 56SNOMED-CT, 34MeSH, 35MESH via Orphanet, 57SNOMED-CT via Orphanet, 61UMLS via Orphanet
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Classifications:

Malacards categories (disease lists): (See all malacards categories)
Anatomical: Neuronal diseases


Aliases & Descriptions:

gerstmann syndrome 8 42 48 60
developmental gerstmann's syndrome 60
developmental gerstmann syndrome 42
aphasia-angular gyrus syndrome 8
gerstmann badal syndrome 42
gerstmann's syndrome 43
gerstmann tetrad 42
gs 42


External Ids:

Disease Ontology8 DOID:4969
MeSH34 D005862
MESH via Orphanet35 D005862
SNOMED-CT56 36785009
SNOMED-CT via Orphanet57 36785009
UMLS via Orphanet61 C0017494

Related Diseases for Gerstmann Syndrome

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17GeneCards, 18GeneDecks
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Graphical network of the top 20 diseases related to Gerstmann Syndrome:



Diseases related to gerstmann syndrome

Clinical Features for Gerstmann Syndrome

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Drugs & Therapeutics for Gerstmann Syndrome

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5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials
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Approved drugs:

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Drug clinical trials:

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Genetic Tests for Gerstmann Syndrome

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Anatomical Context for Gerstmann Syndrome

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32MalaCards
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MalaCards organs/tissues related to Gerstmann Syndrome:

32
Brain, Parietal lobe

Animal Models for Gerstmann Syndrome or affiliated genes

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Publications for Gerstmann Syndrome

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50PubMed
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Articles related to Gerstmann Syndrome:

(show top 50)    (show all 54)
idTitleAuthorsYear
1
Gerstmann-StrAoussler-Scheinker syndrome with the P102L pathogenic mutation presenting as familial Creutzfeldt-Jakob disease: a case report and review of the literature. (22494260)
2013
2
Upregulation of micro RNA-146a (miRNA-146a), a marker for inflammatory neurodegeneration, in sporadic Creutzfeldt-Jakob disease (sCJD) and Gerstmann-Straussler-Scheinker (GSS) syndrome. (22043907)
2011
3
Molecular origin of Gerstmann-StrAoussler-Scheinker syndrome: insight from computer simulation of an amyloidogenic prion peptide. (21689534)
2011
4
A distinct phenotype of leg hyperreflexia in a Japanese family with Gerstmann-StrAoussler-Scheinker syndrome (P102L). (20154442)
2010
5
Loss of anti-Bax function in Gerstmann-StrAoussler-Scheinker syndrome-associated prion protein mutants. (19680558)
2009
6
Developmental Gerstmann syndrome in a young Taiwanese male: a case report. (19540897)
2009
7
A New Transgenic Mouse Model of Gerstmann-Straussler-Scheinker Syndrome Caused by the A117V Mutation of PRNP. (19675240)
2009
8
A disconnection account of Gerstmann syndrome: functional neuroanatomy evidence. (19938150)
2009
9
A case of Gerstmann-StrAoussler-Scheinker syndrome with the P105L prion protein gene mutation presenting with ataxia and extrapyramidal signs without spastic paraparesis. (19443103)
2009
10
Hyperphosphorylated tau deposition parallels prion protein burden in a case of Gerstmann-StrAoussler-Scheinker syndrome P102L mutation complicated with dementia. (12200619)
2002
11
The Gerstmann syndrome in Alzheimer's disease. (11861708)
2002
12
Gerstmann-StrAoussler-Scheinker syndrome,fatal familial insomnia, and kuru: a review of these less common human transmissible spongiform encephalopathies. (11535002)
2001
13
Gerstmann syndrome in systemic lupus erythematosus: neuropsychological, neuroimaging and spectroscopic findings. (11788743)
2001
14
Analyses of Gerstmann-Straussler syndrome with 102Leu219Lys using monoclonal antibodies that specifically detect human prion protein with 219Glu. (10889337)
2000
15
Acetazolamide-induced Gerstmann syndrome. (10692744)
1999
16
A pure case of Gerstmann syndrome with a subangular lesion. (10356063)
1999
17
Neurofibrillary tangles in Gerstmann-StrAoussler-Scheinker syndrome with the A117V prion gene mutation. (9285466)
1997
18
Toe agnosia in Gerstmann syndrome. (9328265)
1997
19
Left atrial myxoma presenting as Gerstmann syndrome. (8548463)
1996
20
New variant prion protein in a Japanese family with Gerstmann-StrAoussler syndrome. (7637591)
1995
21
Microglia is a component of the prion protein amyloid plaque in the Gerstmann-StrAoussler-Scheinker syndrome. (8337941)
1993
22
Novel missense variants of prion protein in Creutzfeldt-Jakob disease or Gerstmann-Straussler syndrome. (8461023)
1993
23
An amber mutation of prion protein in Gerstmann-StrAoussler syndrome with mutant PrP plaques. (8097911)
1993
24
Colocalization of prion protein and beta protein in the same amyloid plaques in patients with Gerstmann-StrAoussler syndrome. (1349451)
1992
25
A prion protein missense variant is integrated in kuru plaque cores in patients with Gerstmann-StrAoussler syndrome. (1671530)
1991
26
Gerstmann syndrome: a case report. (2249447)
1990
27
Creutzfeldt-Jakob disease patients with congophilic kuru plaques have the missense variant prion protein common to Gerstmann-StrAoussler syndrome. (2180366)
1990
28
Immunochemical, molecular genetic, and transmission studies on a case of Gerstmann-StrAoussler-Scheinker syndrome. (1699173)
1990
29
Linkage of a prion protein missense variant to Gerstmann-Straussler syndrome. (2564168)
1989
30
Mutations in familial Creutzfeldt-Jakob disease and Gerstmann-StrAoussler-Scheinker's syndrome. (2572450)
1989
31
Diagnosis of Gerstmann-StrAoussler syndrome in familial dementia with prion protein gene analysis. (2567794)
1989
32
Pro-->Leu change at position 102 of prion protein is the most common but not the sole mutation related to Gerstmann-Straussler syndrome. (2783132)
1989
33
Developmental Gerstmann syndrome: case report and review of the literature. (3611364)
1987
34
Gerstmann syndrome without aphasia: a longitudinal study. (6537237)
1984
35
Spino-cerebello-cerebral degeneration with amyloid plaques (Gerstmann, StrAoussler, Scheinker syndrome). (7151028)
1982
36
Piagetian theory and the atypical case: an analysis of the developmental Gerstmann syndrome. (7229539)
1981
37
Echoacousia in Gerstmann syndrome. (712970)
1978
38
Disorders of oculomotor scanning and graphic orientation in developmental Gerstmann syndrome. (618563)
1978
39
Reflections on the Gerstmann syndrome. (318903)
1977
40
Gerstmann syndrome without aphasia: a reply to Poeck and Orgass. (1204370)
1975
41
Gerstmann syndrome without aphasia: comments on the paper by Strub and Geschwind. (1204369)
1975
42
Gerstmann syndrome without aphasia. (4452255)
1974
43
Developmental Gerstmann syndrome. (5461058)
1970
44
Some posthumous notes on the Gerstmann syndrome. (4245825)
1970
45
Developmental Gerstmann syndrome. (4174929)
1968
46
The developmental Gerstmann syndrome. (14032921)
1963
47
Neuro-ophthalmic aspects of the Gerstmann syndrome. A case report. (14456348)
1962
48
Some notes on the Gerstmann syndrome. (13483851)
1957
49
An early description of the Gerstmann syndrome. (13378586)
1956
50
The Gerstmann syndrome; case report and review of the literature. (20291825)
1947

Genetic Variations for Gerstmann Syndrome

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Expression for genes affiliated with Gerstmann Syndrome

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1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Gerstmann Syndrome

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Pathways for genes affiliated with Gerstmann Syndrome

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Compounds for genes affiliated with Gerstmann Syndrome

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GO Terms for genes affiliated with Gerstmann Syndrome

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Products for genes affiliated with Gerstmann Syndrome

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Sources for Gerstmann Syndrome

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
50PubMed
51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet