MCID: GRS001
MIFTS: 30

Gerstmann Syndrome

Categories: Rare diseases, Neuronal diseases

Aliases & Classifications for Gerstmann Syndrome

MalaCards integrated aliases for Gerstmann Syndrome:

Name: Gerstmann Syndrome 12 76 53 59 44 15 73
Developmental Gerstmann's Syndrome 73
Developmental Gerstmann Syndrome 53
Aphasia-Angular Gyrus Syndrome 12
Gerstmann Badal Syndrome 53
Gerstmann's Syndrome 54
Gerstmann Tetrad 53
Gs 53

Classifications:

Orphanet: 59  
Rare neurological diseases


External Ids:

Disease Ontology 12 DOID:4969
MeSH 44 D005862
SNOMED-CT 68 36785009
Orphanet 59 ORPHA221117
ICD10 via Orphanet 34 F81.2
MESH via Orphanet 45 D005862
UMLS via Orphanet 74 C0017494

Summaries for Gerstmann Syndrome

NINDS : 54 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 Gerstmann-Sträussler-Scheinker disease, a type of transmissible spongiform encephalopathy. In addition to exhibiting the above symptoms, many adults also experience aphasia, (difficulty in expressing oneself when speaking, in understanding speech, or in reading and writing). There are few reports of the syndrome, sometimes called developmental Gerstmann's syndrome, in children. The cause is not known. Most cases are identified when children reach school age, a time when they are challenged with writing and math exercises. Generally, children with the disorder exhibit poor handwriting and spelling skills, and difficulty with math functions, including adding, subtracting, multiplying, and dividing. An inability to differentiate right from left and to discriminate among individual fingers may also be apparent. In addition to the four primary symptoms, many children also suffer from constructional apraxia, an inability to copy simple drawings. Frequently, there is also an impairment in reading. Children with a high level of intellectual functioning as well as those with brain damage may be affected with the disorder.

MalaCards based summary : Gerstmann Syndrome, also known as developmental gerstmann's syndrome, is related to griscelli syndrome and mccune-albright syndrome, and has symptoms including agraphia, acalculia and finger agnosia. An important gene associated with Gerstmann Syndrome is CBS (Cystathionine-Beta-Synthase). Affiliated tissues include left parietal lobe, parietal lobe and brain, and related phenotype is Reduced mammosphere formation.

Disease Ontology : 12 A nervous system disease that results_from damage located in left parietal lobe, has symptom agraphia, has symptom acalculia, has symptom finger agnosia.

NIH Rare Diseases : 53 The following summary is from Orphanet, a European reference portal for information on rare diseases and orphan drugs.Orpha Number: 221117Disease definitionGerstmann syndrome is a very rare neurological disorder characterized by the specific association of acalculia, finger agnosia, left-right disorientation, and agraphia, which is supposed to be secondary to a focal subcortical white matter damage in the parietal lobe.Visit the Orphanet disease page for more resources.

Wikipedia : 76 Gerstmann syndrome is a neuropsychiatric disorder that is characterized by a constellation of symptoms... more...

Related Diseases for Gerstmann Syndrome

Graphical network of the top 20 diseases related to Gerstmann Syndrome:



Diseases related to Gerstmann Syndrome

Symptoms & Phenotypes for Gerstmann Syndrome

Symptoms:

12
  • agraphia
  • acalculia
  • finger agnosia

UMLS symptoms related to Gerstmann Syndrome:


other symbolic dysfunction

GenomeRNAi Phenotypes related to Gerstmann Syndrome according to GeneCards Suite gene sharing:

26
# Description GenomeRNAi Source Accession Score Top Affiliating Genes
1 Reduced mammosphere formation GR00396-S 8.62 CBS CMKLR1

Drugs & Therapeutics for Gerstmann Syndrome

Search Clinical Trials , NIH Clinical Center for Gerstmann Syndrome

Cochrane evidence based reviews: gerstmann syndrome

Genetic Tests for Gerstmann Syndrome

Anatomical Context for Gerstmann Syndrome

The Foundational Model of Anatomy Ontology organs/tissues related to Gerstmann Syndrome:

19
Left Parietal Lobe

MalaCards organs/tissues related to Gerstmann Syndrome:

41
Parietal Lobe, Brain

Publications for Gerstmann Syndrome

Articles related to Gerstmann Syndrome:

(show all 33)
# Title Authors Year
1
Gerstmann syndrome: historic and current perspectives. ( 29519471 )
2018
2
Gerstmann Syndrome in a Patient With Aggressive Mucormycosis. ( 28400906 )
2017
3
Transient Gerstmann syndrome as manifestation of stroke: Case report and brief literature review. ( 29213512 )
2017
4
"With a Smile Through Tears": The Uprooted Career of the Man Behind Gerstmann Syndrome. ( 25259646 )
2014
5
Mirror visual feedback for right-left discrimination in a patient with developmental Gerstmann syndrome. ( 24515713 )
2014
6
Developmental Gerstmann syndrome in a young Taiwanese male: a case report. ( 19540897 )
2009
7
A disconnection account of Gerstmann syndrome: functional neuroanatomy evidence. ( 19938150 )
2009
8
Writing, calculating, and finger recognition in the region of the angular gyrus: a cortical stimulation study of Gerstmann syndrome. ( 14567608 )
2003
9
The Gerstmann syndrome in Alzheimer's disease. ( 11861708 )
2002
10
Gerstmann syndrome in systemic lupus erythematosus: neuropsychological, neuroimaging and spectroscopic findings. ( 11788743 )
2001
11
Acetazolamide-induced Gerstmann syndrome. ( 10692744 )
1999
12
A pure case of Gerstmann syndrome with a subangular lesion. ( 10356063 )
1999
13
Toe agnosia in Gerstmann syndrome. ( 9328265 )
1997
14
Left atrial myxoma presenting as Gerstmann syndrome. ( 8548463 )
1996
15
Gerstmann syndrome: a case report. ( 2249447 )
1990
16
Developmental Gerstmann syndrome: case report and review of the literature. ( 3611364 )
1987
17
Gerstmann syndrome without aphasia: a longitudinal study. ( 6537237 )
1984
18
Piagetian theory and the atypical case: an analysis of the developmental Gerstmann syndrome. ( 7229539 )
1981
19
Dyscalculia and elements of the developmental Gerstmann syndrome in school children. ( 679700 )
1978
20
Echoacousia in Gerstmann syndrome. ( 712970 )
1978
21
Disorders of oculomotor scanning and graphic orientation in developmental Gerstmann syndrome. ( 618563 )
1978
22
Reflections on the Gerstmann syndrome. ( 318903 )
1977
23
Gerstmann syndrome without aphasia: a reply to Poeck and Orgass. ( 1204370 )
1975
24
Gerstmann syndrome without aphasia: comments on the paper by Strub and Geschwind. ( 1204369 )
1975
25
Gerstmann syndrome without aphasia. ( 4452255 )
1974
26
Some posthumous notes on the Gerstmann syndrome. ( 4245825 )
1970
27
Developmental Gerstmann syndrome. ( 5461058 )
1970
28
Developmental Gerstmann syndrome. ( 4174929 )
1968
29
The developmental Gerstmann syndrome. ( 14032921 )
1963
30
Neuro-ophthalmic aspects of the Gerstmann syndrome. A case report. ( 14456348 )
1962
31
Some notes on the Gerstmann syndrome. ( 13483851 )
1957
32
An early description of the Gerstmann syndrome. ( 13378586 )
1956
33
The Gerstmann syndrome; case report and review of the literature. ( 20291825 )
1947

Variations for Gerstmann Syndrome

Expression for Gerstmann Syndrome

Search GEO for disease gene expression data for Gerstmann Syndrome.

Pathways for Gerstmann Syndrome

GO Terms for Gerstmann Syndrome

Sources for Gerstmann Syndrome

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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