MCID: GRS001
MIFTS: 35

Gerstmann Syndrome

Categories: Neuronal diseases, Rare diseases

Aliases & Classifications for Gerstmann Syndrome

MalaCards integrated aliases for Gerstmann Syndrome:

Name: Gerstmann Syndrome 12 77 54 60 45 15 17 74
Developmental Gerstmann's Syndrome 74
Developmental Gerstmann Syndrome 54
Aphasia-Angular Gyrus Syndrome 12
Gerstmann Badal Syndrome 54
Gerstmann's Syndrome 55
Gerstmann Tetrad 54
Gs 54

Classifications:

Orphanet: 60  
Rare neurological diseases


External Ids:

Disease Ontology 12 DOID:4969
MeSH 45 D005862
SNOMED-CT 69 36785009
MESH via Orphanet 46 D005862
ICD10 via Orphanet 35 F81.2
UMLS via Orphanet 75 C0017494
Orphanet 60 ORPHA221117

Summaries for Gerstmann Syndrome

NINDS : 55 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 finger agnosia, agraphia and acalculia. An important gene associated with Gerstmann Syndrome is TBX1 (T-Box 1). Affiliated tissues include parietal lobe, brain and prostate, and related phenotypes are craniofacial and limbs/digits/tail

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 : 54 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 : 77 Gerstmann syndrome is a neuropsychiatric 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 144)
# Related Disease Score Top Affiliating Genes
1 griscelli syndrome 12.1
2 mccune-albright syndrome 11.6
3 pseudohypoparathyroidism 11.4
4 pseudohypoparathyroidism, type ic 11.2
5 finger agnosia 11.1
6 megaloblastic anemia 1 11.1
7 gitelman syndrome 11.1
8 glutamine deficiency, congenital 11.1
9 gilbert syndrome 11.1
10 pseudohypoparathyroidism, type ii 11.1
11 pseudohypoparathyroidism, type ib 11.1
12 albright's hereditary osteodystrophy 10.3
13 glioma 10.2
14 leopard syndrome 10.2
15 noonan syndrome with multiple lentigines 10.2
16 aphasia 10.2
17 fibrous dysplasia 10.1
18 sarcoma 10.1
19 cholera 10.1
20 adenoma 10.1
21 myeloid sarcoma 10.1
22 pituitary tumors 10.1
23 pseudohypoparathyroidism, type ia 10.0
24 pseudopseudohypoparathyroidism 10.0
25 radin blood group antigen 10.0
26 major affective disorder 1 10.0
27 osseous heteroplasia, progressive 10.0
28 major affective disorder 8 10.0
29 major affective disorder 7 10.0
30 major affective disorder 9 10.0
31 leukemia 10.0
32 conn's syndrome 10.0
33 adenocarcinoma 10.0
34 pituitary adenoma 10.0
35 multiple endocrine neoplasia 10.0
36 fragile x syndrome 10.0
37 ataxia and polyneuropathy, adult-onset 10.0
38 agraphia 10.0
39 dysgraphia 10.0
40 acquired immunodeficiency syndrome 10.0
41 progressive multifocal leukoencephalopathy 10.0
42 learning disability 10.0
43 encephalitis 10.0
44 corticobasal degeneration 10.0
45 herpes simplex encephalitis 10.0
46 tumefactive multiple sclerosis 10.0
47 alzheimer disease 9.9
48 systemic lupus erythematosus 9.9
49 dyscalculia 9.9
50 agnosia 9.9

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



Diseases related to Gerstmann Syndrome

Symptoms & Phenotypes for Gerstmann Syndrome

Symptoms:

12
  • finger agnosia
  • agraphia
  • acalculia

UMLS symptoms related to Gerstmann Syndrome:


other symbolic dysfunction

MGI Mouse Phenotypes related to Gerstmann Syndrome:

47
# Description MGI Source Accession Score Top Affiliating Genes
1 craniofacial MP:0005382 9.16 CBSL TBX1
2 limbs/digits/tail MP:0005371 8.96 CBSL TBX1
3 respiratory system MP:0005388 8.62 CBSL TBX1

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

MalaCards organs/tissues related to Gerstmann Syndrome:

42
Parietal Lobe, Brain, Prostate, Heart, Pituitary, Lung, Bone

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

20
Left Parietal Lobe

Publications for Gerstmann Syndrome

Articles related to Gerstmann Syndrome:

(show all 38)
# 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 )
2015
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
What ever happened to developmental Gerstmann's syndrome? Links to other pediatric, genetic, and neurodevelopmental syndromes. ( 15163095 )
2004
9
Writing, calculating, and finger recognition in the region of the angular gyrus: a cortical stimulation study of Gerstmann syndrome. ( 14567608 )
2003
10
The Gerstmann syndrome in Alzheimer's disease. ( 11861708 )
2002
11
Gerstmann syndrome in systemic lupus erythematosus: neuropsychological, neuroimaging and spectroscopic findings. ( 11788743 )
2001
12
Developmental Gerstmann's syndrome: a distinct clinical entity of learning disabilities. ( 10788743 )
2000
13
A pure case of Gerstmann syndrome with a subangular lesion. ( 10356063 )
1999
14
Acetazolamide-induced Gerstmann syndrome. ( 10692744 )
1999
15
Toe agnosia in Gerstmann syndrome. ( 9328265 )
1997
16
Left atrial myxoma presenting as Gerstmann syndrome. ( 8548463 )
1996
17
Gerstmann syndrome: a case report. ( 2249447 )
1990
18
Fragile X syndrome: a genetic etiology for developmental Gerstmann's syndrome. ( 2590008 )
1989
19
Developmental Gerstmann's syndrome. ( 3415528 )
1988
20
Developmental Gerstmann syndrome: case report and review of the literature. ( 3611364 )
1987
21
Gerstmann syndrome without aphasia: a longitudinal study. ( 6537237 )
1984
22
Piagetian theory and the atypical case: an analysis of the developmental Gerstmann syndrome. ( 7229539 )
1981
23
Dyscalculia and elements of the developmental Gerstmann syndrome in school children. ( 679700 )
1978
24
Disorders of oculomotor scanning and graphic orientation in developmental Gerstmann syndrome. ( 618563 )
1978
25
Echoacousia in Gerstmann syndrome. ( 712970 )
1978
26
Reflections on the Gerstmann syndrome. ( 318903 )
1977
27
Gerstmann syndrome without aphasia: a reply to Poeck and Orgass. ( 1204370 )
1975
28
Gerstmann syndrome without aphasia: comments on the paper by Strub and Geschwind. ( 1204369 )
1975
29
Gerstmann syndrome without aphasia. ( 4452255 )
1974
30
Some posthumous notes on the Gerstmann syndrome. ( 4245825 )
1970
31
Developmental Gerstmann syndrome. ( 5461058 )
1970
32
Developmental Gerstmann syndrome. ( 4174929 )
1968
33
The developmental Gerstmann syndrome. ( 14032921 )
1963
34
Neuro-ophthalmic aspects of the Gerstmann syndrome. A case report. ( 14456348 )
1962
35
THE FICTION OF THE "GERSTMANN SYNDROME". ( 21610898 )
1961
36
Some notes on the Gerstmann syndrome. ( 13483851 )
1957
37
An early description of the Gerstmann syndrome. ( 13378586 )
1956
38
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 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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