GS
MCID: GRS001
MIFTS: 41

Gerstmann Syndrome (GS)

Categories: Neuronal diseases, Rare diseases

Aliases & Classifications for Gerstmann Syndrome

MalaCards integrated aliases for Gerstmann Syndrome:

Name: Gerstmann Syndrome 12 73 20 58 44 15 17 70
Developmental Gerstmann's Syndrome 70
Developmental Gerstmann Syndrome 20
Aphasia-Angular Gyrus Syndrome 12
Gerstmann Badal Syndrome 20
Gerstmann's Syndrome 53
Gerstmann Tetrad 20
Gs 20

Classifications:

Orphanet: 58  
Rare neurological diseases


External Ids:

Disease Ontology 12 DOID:4969
MeSH 44 D005862
SNOMED-CT 67 36785009
MESH via Orphanet 45 D005862
ICD10 via Orphanet 33 F81.2
UMLS via Orphanet 71 C0017494
Orphanet 58 ORPHA221117
UMLS 70 C0017494 C0936244

Summaries for Gerstmann Syndrome

NINDS : 53 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 finger agnosia and dysgraphia, and has symptoms including finger agnosia, agraphia and acalculia. An important gene associated with Gerstmann Syndrome is MAPT (Microtubule Associated Protein Tau), and among its related pathways/superpathways are Pathways of neurodegeneration - multiple diseases and Notch Signaling Pathway (WikiPathways). Affiliated tissues include left parietal lobe, parietal lobe and brain, and related phenotypes are behavior/neurological and integument

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.

GARD : 20 The following summary is from Orphanet, a European reference portal for information on rare diseases and orphan drugs. Orpha Number: 221117 Definition Gerstmann 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.

Wikipedia : 73 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 126)
# Related Disease Score Top Affiliating Genes
1 finger agnosia 31.9 PCAT2 MAPT
2 dysgraphia 30.9 MAPT GRN
3 dyscalculia 30.2 MAPT GRN APOE
4 apraxia 29.8 PSEN1 MAPT GRN APOE
5 agraphia 29.7 PRNP PCAT2 MAPT GRN APOE
6 creutzfeldt-jakob disease 29.3 PRNP MAPT APOE
7 stroke, ischemic 29.3 PSEN1 MAPT APOE
8 simultanagnosia 29.1 PSEN2 PSEN1 PCAT2 MAPT APOE
9 aphasia 29.1 PSEN2 PSEN1 PRNP MAPT GRN APOE
10 nominal aphasia 28.9 PSEN1 PCAT2 MAPT GRN APOE
11 ideomotor apraxia 28.8 PSEN2 PRNP MAPT GRN APOE
12 alexia 28.7 PSEN2 PRNP PCAT2 MAPT GRN APOE
13 visual agnosia 28.6 PSEN2 PSEN1 PCAT2 MAPT GRN APOE
14 dementia 28.3 PSEN2 PSEN1 PRNP MAPT GRN APOE
15 agnosia 10.5
16 subjective cognitive decline 10.2 MAPT APOE
17 posterior cortical atrophy 10.2 MAPT APOE
18 yemenite deaf-blind hypopigmentation syndrome 10.2
19 alzheimer disease 10 10.2 MAPT APOE
20 binswanger's disease 10.2 MAPT APOE
21 age-related hearing loss 10.2 MAPT APOE
22 mild cognitive impairment 10.2 MAPT APOE
23 alzheimer disease 7 10.2 MAPT APOE
24 arteriolosclerosis 10.1 MAPT APOE
25 associative agnosia 10.1 MAPT GRN
26 brain injury 10.1 MAPT APOE
27 apperceptive agnosia 10.1 PSEN2 GRN
28 mutism 10.1 MAPT GRN
29 rem sleep behavior disorder 10.1 MAPT APOE
30 frontotemporal dementia and/or amyotrophic lateral sclerosis 7 10.1 MAPT GRN
31 lentigines 10.1
32 noonan syndrome with multiple lentigines 10.1
33 glioma susceptibility 1 10.1
34 ataxia and polyneuropathy, adult-onset 10.1
35 malignant astrocytoma 10.1
36 acquired immunodeficiency syndrome 10.1
37 progressive multifocal leukoencephalopathy 10.1
38 learning disability 10.1
39 encephalitis 10.1
40 encephalopathy 10.1
41 echolalia 10.1 MAPT GRN
42 anosognosia 10.0 PSEN1 APOE
43 alzheimer disease 9 10.0 PSEN1 MAPT
44 perry syndrome 10.0 MAPT GRN
45 prosopagnosia 10.0 PSEN2 MAPT GRN
46 akinetic mutism 9.9 PRNP MAPT
47 fragile x syndrome 9.9
48 agenesis of corpus callosum, cardiac, ocular, and genital syndrome 9.9
49 arteriovenous malformation 9.9
50 cortical blindness 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:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 behavior/neurological MP:0005386 9.92 APOE GRN MAPT PRNP PSD2 PSEN1
2 integument MP:0010771 9.73 APOE GRN MAPT PRNP PSEN1 PSEN2
3 nervous system MP:0003631 9.7 APOE GRN MAPT PRNP PSD2 PSEN1
4 renal/urinary system MP:0005367 9.35 APOE GRN MAPT PSEN1 PSEN2
5 vision/eye MP:0005391 9.1 APOE GRN MAPT PRNP PSEN1 PSEN2

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:

40
Parietal Lobe, Brain, Eye, Cortex, Pituitary, Lung

Publications for Gerstmann Syndrome

Articles related to Gerstmann Syndrome:

(show top 50) (show all 235)
# Title Authors PMID Year
1
Some evidence on Gerstmann's syndrome: A case study on a variant of the clinical disorder. 61
33477079 2021
2
A case of inferior frontal gyrus infarction manifesting Gerstmann syndrome. 61
33175666 2020
3
Gerstmann's Syndrome in a Patient Double-positive for Antibodies against the N-methyl-D-aspartate Receptor and NH2-terminal of α-enolase. 61
33229813 2020
4
Left Parietal Tumors Presenting with Smartphone Icon Visual Agnosia: Two Cases of a Modern Presentation of Gerstmann Syndrome. 61
32673807 2020
5
A Rare Clinical Antity; Pure Gerstmann Syndrome. 61
32912538 2020
6
Manifestation of metastatic lung adenocarcinoma as Gerstmann syndrome. 61
33040040 2020
7
Gerstmann Syndrome. 61
32852667 2020
8
Transient Cerebral Vasospasm After Carotid Artery Stenting: A Case Report and Literature Review. 61
31837494 2020
9
Difficulty differentiating a case of posterior cortical atrophy from a psychogenic disturbance of vision. 61
30997705 2020
10
Negative Diffusion Weighted Imaging on Magnetic Resonance Imaging of the Brain in Creutzfeldt-Jakob Disease. 61
33414973 2020
11
Mystery Case: Parietal lobe epilepsy with ictal manifestation of Gerstmann syndrome. 61
31932514 2020
12
Seeing though the clutter: A case report of Posterior Cortical Atrophy and patient-centered care. 61
31855068 2019
13
The making of a syndrome: The English translation of Gerstmann's first report. 61
31029874 2019
14
Gerstmann syndrome complicating polycythemia secondary to anabolic steroid use. 61
31175112 2019
15
Parietal association deficits in patients harboring parietal lobe gliomas: a prospective study. 61
29726775 2018
16
The Progressive Acalculia Presentation of Parietal Variant Alzheimer's Disease. 61
29710718 2018
17
Gerstmann syndrome: historic and current perspectives. 61
29519471 2018
18
Cognitive world: Neuropsychology of individual differences. 61
27649025 2018
19
Posterior Type of Alzheimer's Dementia Presenting with Homonymous Hemianopsia. 61
30906384 2017
20
Gerstmann's syndrome and unilateral optic ataxia in the emergency department. 61
29354229 2017
21
Gerstmann's Syndrome Associated with Right Parietal Hemorrhage and Arteriovenous Malformation. 61
28516745 2017
22
Gerstmann Syndrome in a Patient With Aggressive Mucormycosis. 61
28400906 2017
23
[Brodmann Areas 39 and 40: Human Parietal Association Area and Higher Cortical Function]. 61
28424400 2017
24
Transient Gerstmann syndrome as manifestation of stroke: Case report and brief literature review. 61
29213512 2017
25
Ictal conduction aphasia and ictal angular gyrus syndrome as rare manifestations of epilepsy: The importance of ictal testing during video-EEG monitoring. 61
28913167 2017
26
Endovascular Treatment of Giant Serpentine Aneurysm of the Middle Cerebral Artery. 61
27847772 2016
27
[Profiles of cognitive and language impairment of logopenic and non-fluent variant of primary progressive aphasia]. 61
27524196 2016
28
Can patients without early, prominent visual deficits still be diagnosed of posterior cortical atrophy? 61
27423559 2016
29
The phenotypical core of Alzheimer's disease-related and nonrelated variants of the corticobasal syndrome: A systematic clinical, neuropsychological, imaging, and biomarker study. 61
26988428 2016
30
Safe Resection of Gliomas of the Dominant Angular Gyrus Availing of Preoperative FMRI and Intraoperative DTI: Preliminary Series and Surgical Technique. 61
26548825 2016
31
Progressive Multifocal Leukoencephalopathy with Balanced CD4/CD8 T-Cell Infiltration and Good Response to Mefloquine Treatment. 61
27301518 2016
32
Histologically confirmed case of cerebral vasculitis associated with Crohn's disease--a case report. 61
26390922 2015
33
"With a smile through tears": the uprooted career of the man behind Gerstmann syndrome. 61
25259646 2015
34
[Carbon monoxide poisoning: clinical features of the victims of the explosion accident of Mitsui-Miike Mikawa coal mine 50 years ago]. 61
25585432 2015
35
[Functional mapping using subdural electrodes combined with monitoring during awake craniotomy enabled preservation of function and extensive resection of a glioma adjacent to the parietal lobe language sites: a case report]. 61
25557101 2015
36
A proposed reinterpretation of Gerstmann's syndrome. 61
25377466 2014
37
Clinical Utility of Amyloid Imaging in a Complex Case of Corticobasal Syndrome Presenting with Psychiatric Symptoms. 61
26225355 2014
38
Distinct representations of symbolic ordinality and quantity: evidence from neuropsychological investigations in a Chinese patient with Gerstmann's syndrome. 61
24833400 2014
39
A rare presentation of atypical demyelination: tumefactive multiple sclerosis causing Gerstmann's syndrome. 61
24694183 2014
40
The progression of posterior cortical atrophy to corticobasal syndrome: lumping or splitting neurodegenerative diseases? 61
25009764 2014
41
Mirror visual feedback for right-left discrimination in a patient with developmental Gerstmann syndrome. 61
24515713 2014
42
Dyscalculia, dysgraphia, and left-right confusion from a left posterior peri-insular infarct. 61
24817791 2014
43
Right superior longitudinal fasciculus: implications for visuospatial neglect mimicking Gerstmann's syndrome. 61
22818517 2013
44
Acute parietal lobe infarction presenting as Gerstmann's syndrome and cognitive decline mimicking senile dementia. 61
23847420 2013
45
Is psychopathy a syndrome? Commentary on Marcus, Fulton, and Edens. 61
23339318 2013
46
Gerstmann's syndrome associated with diagnostic cerebral angiography. 61
23324021 2013
47
Corticobasal syndrome presenting with partial Gerstmann's syndrome and digit agnosia. 61
23686076 2013
48
Gerstmann'S syndrome in acute stroke patients. 61
23378691 2012
49
[Acquired and developmental Gerstmann syndrome. Illustration from a patient with multiple sclerosis]. 61
22560518 2012
50
Simultaneous surgical resections of two distant metastatic malignant melanoma lesions--case report. 61
22515124 2012

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

Cellular components related to Gerstmann Syndrome according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 endoplasmic reticulum GO:0005783 9.85 PSEN2 PSEN1 PRNP GRN APOE
2 Golgi apparatus GO:0005794 9.8 PSEN2 PSEN1 PRNP GRN APOE
3 lysosomal membrane GO:0005765 9.65 PSEN2 PSEN1 GRN
4 growth cone GO:0030426 9.54 PSEN2 PSEN1 MAPT
5 membrane raft GO:0045121 9.46 PSEN2 PSEN1 PRNP MAPT
6 dendritic shaft GO:0043198 9.43 PSEN2 PSEN1
7 dendrite GO:0030425 9.35 PSEN1 PSD2 PRNP MAPT APOE
8 ciliary rootlet GO:0035253 9.32 PSEN2 PSEN1
9 gamma-secretase complex GO:0070765 9.26 PSEN2 PSEN1
10 neuronal cell body GO:0043025 9.02 PSEN2 PSEN1 PSD2 MAPT APOE

Biological processes related to Gerstmann Syndrome according to GeneCards Suite gene sharing:

(show all 27)
# Name GO ID Score Top Affiliating Genes
1 negative regulation of gene expression GO:0010629 9.8 PSEN1 MAPT APOE
2 negative regulation of neuron apoptotic process GO:0043524 9.72 PSEN1 GRN APOE
3 response to oxidative stress GO:0006979 9.67 PSEN1 PRNP APOE
4 regulation of synaptic plasticity GO:0048167 9.63 PSEN1 MAPT
5 positive regulation of neuron apoptotic process GO:0043525 9.63 PRNP GRN
6 cellular response to amyloid-beta GO:1904646 9.62 PSEN1 PRNP
7 positive regulation of neuron death GO:1901216 9.61 PRNP MAPT
8 gene expression GO:0010467 9.61 PSEN1 APOE
9 long-term memory GO:0007616 9.6 PRNP APOE
10 positive regulation of catalytic activity GO:0043085 9.59 PSEN2 PSEN1
11 negative regulation of amyloid-beta formation GO:1902430 9.58 PRNP APOE
12 positive regulation of dendritic spine development GO:0060999 9.58 PSEN1 APOE
13 membrane protein ectodomain proteolysis GO:0006509 9.57 PSEN2 PSEN1
14 membrane protein intracellular domain proteolysis GO:0031293 9.56 PSEN2 PSEN1
15 astrocyte activation GO:0048143 9.54 PSEN1 MAPT
16 amyloid-beta metabolic process GO:0050435 9.52 PSEN2 PSEN1
17 amyloid precursor protein catabolic process GO:0042987 9.51 PSEN2 PSEN1
18 negative regulation of long-term synaptic potentiation GO:1900272 9.48 PRNP APOE
19 amyloid precursor protein metabolic process GO:0042982 9.46 PSEN1 APOE
20 neuron projection maintenance GO:1990535 9.43 PSEN1 PRNP
21 amyloid-beta formation GO:0034205 9.4 PSEN2 PSEN1
22 modulation of age-related behavioral decline GO:0090647 9.37 PSEN1 PRNP
23 learning or memory GO:0007611 9.33 PSEN1 PRNP MAPT
24 Notch receptor processing GO:0007220 9.32 PSEN2 PSEN1
25 Notch receptor processing, ligand-dependent GO:0035333 9.26 PSEN2 PSEN1
26 positive regulation of amyloid fibril formation GO:1905908 8.96 PSEN1 APOE
27 astrocyte activation involved in immune response GO:0002265 8.62 PSEN1 GRN

Molecular functions related to Gerstmann Syndrome according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 chaperone binding GO:0051087 9.33 PRNP MAPT GRN
2 aspartic-type endopeptidase activity GO:0004190 9.26 PSEN2 PSEN1
3 lipoprotein particle binding GO:0071813 8.96 MAPT APOE
4 aspartic endopeptidase activity, intramembrane cleaving GO:0042500 8.62 PSEN2 PSEN1

Sources for Gerstmann Syndrome

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 20-May-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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