MCID: GRS001
MIFTS: 28

Gerstmann Syndrome

Categories: Rare diseases, Neuronal diseases

Aliases & Classifications for Gerstmann Syndrome

MalaCards integrated aliases for Gerstmann Syndrome:

Name: Gerstmann Syndrome 12 50 56 42 14 69
Developmental Gerstmann's Syndrome 69
Developmental Gerstmann Syndrome 50
Aphasia-Angular Gyrus Syndrome 12
Gerstmann Badal Syndrome 50
Gerstmann's Syndrome 51
Gerstmann Tetrad 50
Gs 50

Classifications:

Orphanet: 56  
Rare neurological diseases


External Ids:

Disease Ontology 12 DOID:4969
MeSH 42 D005862
SNOMED-CT 64 36785009
Orphanet 56 ORPHA221117
MESH via Orphanet 43 D005862
UMLS via Orphanet 70 C0017494
ICD10 via Orphanet 34 F81.2
UMLS 69 C0017494

Summaries for Gerstmann Syndrome

NINDS : 51 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 pseudohypoparathyroidism, and has symptoms including other symbolic dysfunction An important gene associated with Gerstmann Syndrome is CBS (Cystathionine-Beta-Synthase). The drugs Benzocaine and Calfactant have been mentioned in the context of this disorder. Affiliated tissues include parietal lobe and brain.

NIH Rare Diseases : 50 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. last updated: 2/29/2012

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.

Wikipedia : 72 Gerstmann syndrome is a neuropsychological disorder that is characterized by a constellation of symptoms... more...

Related Diseases for Gerstmann Syndrome

Diseases related to Gerstmann Syndrome via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 71)
id Related Disease Score Top Affiliating Genes
1 griscelli syndrome 11.6
2 pseudohypoparathyroidism 11.1
3 pseudohypoparathyroidism ic 10.9
4 gnas hyperfunction 10.9
5 finger agnosia 10.8
6 glutamine deficiency, congenital 10.8
7 megaloblastic anemia-1, finnish type 10.8
8 gitelman syndrome 10.8
9 mccune-albright syndrome, somatic, mosaic 10.7
10 pseudohypoparathyroidism, type ib 10.7
11 gilbert syndrome 10.7
12 adenoma 10.1
13 thyroiditis 10.1
14 neuroblastoma 10.0
15 glioma 10.0
16 aphasia 10.0
17 pituitary adenoma 9.9
18 cholera 9.9
19 pituitary tumors 9.9
20 neuronitis 9.8
21 multiple endocrine neoplasia 9.8
22 leukemia 9.7
23 lymphoma 9.7
24 fibrous dysplasia 9.7
25 hypothyroidism 9.7
26 prostatitis 9.7
27 acrodysostosis 9.7
28 retinitis 9.7
29 influenza 9.7
30 dysautonomia 9.7
31 systemic lupus erythematosus 9.7
32 dyscalculia 9.7
33 agnosia 9.7
34 lupus erythematosus 9.7
35 pheochromocytoma 9.5
36 colorectal cancer 9.5
37 malaria 9.5
38 hepatocellular carcinoma 9.5
39 breast cancer 9.5
40 pseudopseudohypoparathyroidism 9.5
41 gastroschisis 9.5
42 atherosclerosis 9.5
43 dilated cardiomyopathy 9.5
44 ischemic heart disease 9.5
45 giardiasis 9.5
46 sarcoma 9.5
47 pertussis 9.5
48 hypoparathyroidism 9.5
49 heart disease 9.5
50 ecthyma 9.5

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



Diseases related to Gerstmann Syndrome

Symptoms & Phenotypes for Gerstmann Syndrome

UMLS symptoms related to Gerstmann Syndrome:


other symbolic dysfunction

Drugs & Therapeutics for Gerstmann Syndrome

Drugs for Gerstmann Syndrome (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):


id Name Status Phase Clinical Trials Cas Number PubChem Id
1
Benzocaine Approved Phase 3 1994-09-7, 94-09-7 2337
2
Calfactant Approved Phase 3 183325-78-2
3 tannic acid Approved, Nutraceutical Phase 3
4 Pulmonary Surfactants Phase 3
5 Respiratory System Agents Phase 3

Interventional clinical trials:


id Name Status NCT ID Phase Drugs
1 Comparison of Aerosol Delivery of Infasurf to Usual Care in Spontaneously Breathing RDS Patients Recruiting NCT03058666 Phase 3 Aerosolized Calfactant

Search NIH Clinical Center for Gerstmann Syndrome

Cochrane evidence based reviews: gerstmann syndrome

Genetic Tests for Gerstmann Syndrome

Anatomical Context for Gerstmann Syndrome

MalaCards organs/tissues related to Gerstmann Syndrome:

39
Parietal Lobe, Brain

Publications for Gerstmann Syndrome

Articles related to Gerstmann Syndrome:

(show all 31)
id Title Authors Year
1
Gerstmann Syndrome in a Patient With Aggressive Mucormycosis. ( 28400906 )
2017
2
Mirror visual feedback for right-left discrimination in a patient with developmental Gerstmann syndrome. ( 24515713 )
2014
3
"With a Smile Through Tears": The Uprooted Career of the Man Behind Gerstmann Syndrome. ( 25259646 )
2014
4
Developmental Gerstmann syndrome in a young Taiwanese male: a case report. ( 19540897 )
2009
5
A disconnection account of Gerstmann syndrome: functional neuroanatomy evidence. ( 19938150 )
2009
6
Writing, calculating, and finger recognition in the region of the angular gyrus: a cortical stimulation study of Gerstmann syndrome. ( 14567608 )
2003
7
The Gerstmann syndrome in Alzheimer's disease. ( 11861708 )
2002
8
Gerstmann syndrome in systemic lupus erythematosus: neuropsychological, neuroimaging and spectroscopic findings. ( 11788743 )
2001
9
A pure case of Gerstmann syndrome with a subangular lesion. ( 10356063 )
1999
10
Acetazolamide-induced Gerstmann syndrome. ( 10692744 )
1999
11
Toe agnosia in Gerstmann syndrome. ( 9328265 )
1997
12
Left atrial myxoma presenting as Gerstmann syndrome. ( 8548463 )
1996
13
Gerstmann syndrome: a case report. ( 2249447 )
1990
14
Developmental Gerstmann syndrome: case report and review of the literature. ( 3611364 )
1987
15
Gerstmann syndrome without aphasia: a longitudinal study. ( 6537237 )
1984
16
Piagetian theory and the atypical case: an analysis of the developmental Gerstmann syndrome. ( 7229539 )
1981
17
Echoacousia in Gerstmann syndrome. ( 712970 )
1978
18
Disorders of oculomotor scanning and graphic orientation in developmental Gerstmann syndrome. ( 618563 )
1978
19
Dyscalculia and elements of the developmental Gerstmann syndrome in school children. ( 679700 )
1978
20
Reflections on the Gerstmann syndrome. ( 318903 )
1977
21
Gerstmann syndrome without aphasia: comments on the paper by Strub and Geschwind. ( 1204369 )
1975
22
Gerstmann syndrome without aphasia: a reply to Poeck and Orgass. ( 1204370 )
1975
23
Gerstmann syndrome without aphasia. ( 4452255 )
1974
24
Some posthumous notes on the Gerstmann syndrome. ( 4245825 )
1970
25
Developmental Gerstmann syndrome. ( 5461058 )
1970
26
Developmental Gerstmann syndrome. ( 4174929 )
1968
27
The developmental Gerstmann syndrome. ( 14032921 )
1963
28
Neuro-ophthalmic aspects of the Gerstmann syndrome. A case report. ( 14456348 )
1962
29
Some notes on the Gerstmann syndrome. ( 13483851 )
1957
30
An early description of the Gerstmann syndrome. ( 13378586 )
1956
31
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
16 ExPASy
18 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 MedGen
42 MeSH
43 MESH via Orphanet
44 MGI
46 NCI
47 NCIt
48 NDF-RT
51 NINDS
52 Novoseek
54 OMIM
55 OMIM via Orphanet
59 PubMed
60 QIAGEN
65 SNOMED-CT via HPO
66 SNOMED-CT via Orphanet
67 TGDB
68 Tocris
69 UMLS
70 UMLS via Orphanet
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