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Gerstmann syndrome

MedGen UID:: 42208
•Concept ID:: C0017494
•: Mental or Behavioral Dysfunction

Synonyms:	Developmental Gerstmann syndrome; Gerstmann Badal syndrome; Gerstmann tetrad
SNOMED CT:	Aphasia-angular gyrus syndrome (36785009); Gerstmann's syndrome (36785009)

Monarch Initiative:	MONDO:0005773
Orphanet:	ORPHA221117

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. [from ORDO]

Term Hierarchy

GTR
MeSH
Orphanet

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVGerstmann syndrome

Abnormal nervous system physiology
- Abnormality of mental function
  - Abnormal experience of reality
    - Abnormal interpretation of external stimuli
      - Disturbed perception with a stimulus
        Agnosia
        Gerstmann syndrome

Follow this link to review classifications for Gerstmann syndrome in Orphanet.

Professional guidelines

PubMed

Evaluation of Human Cerebrospinal Fluid Malate Dehydrogenase 1 as a Marker in Genetic Prion Disease Patients.

Zerr I, Villar-Piqué A, Schmitz VE, Poleggi A, Pocchiari M, Sánchez-Valle R, Calero M, Calero O, Baldeiras I, Santana I, Kovacs GG, Llorens F, Schmitz M
Biomolecules 2019 Nov 28;9(12) doi: 10.3390/biom9120800. PMID: 31795176 Free PMC Article

Creutzfeldt-Jakob disease: updated diagnostic criteria, treatment algorithm, and the utility of brain biopsy.

Manix M, Kalakoti P, Henry M, Thakur J, Menger R, Guthikonda B, Nanda A
Neurosurg Focus 2015 Nov;39(5):E2. doi: 10.3171/2015.8.FOCUS15328. PMID: 26646926

Neuropathological diagnostic criteria for Creutzfeldt-Jakob disease (CJD) and other human spongiform encephalopathies (prion diseases).

Budka H, Aguzzi A, Brown P, Brucher JM, Bugiani O, Gullotta F, Haltia M, Hauw JJ, Ironside JW, Jellinger K
Brain Pathol 1995 Oct;5(4):459-66. doi: 10.1111/j.1750-3639.1995.tb00625.x. PMID: 8974629

See all (6)

Recent clinical studies

Etiology

Clinical Screening for Posterior Cortical Atrophy.

Mendez MF, Khattab YI, Yerstein O
Cogn Behav Neurol 2022 Jun 1;35(2):104-109. doi: 10.1097/WNN.0000000000000297. PMID: 35639011

Surgical treatment of parietal lobe epilepsy.

Binder DK, Podlogar M, Clusmann H, Bien C, Urbach H, Schramm J, Kral T
J Neurosurg 2009 Jun;110(6):1170-8. doi: 10.3171/2008.2.17665. PMID: 19199502

Statistical approaches to human brain mapping by functional magnetic resonance imaging.

Lange N
Stat Med 1996 Feb 28;15(4):389-428. doi: 10.1002/(SICI)1097-0258(19960229)15:4<389::AID-SIM285>3.0.CO;2-J. PMID: 8668868

Watershed cerebral infarcts: retrospective study of 24 cases.

Evrard S, Woimant F, Le Coz P, Polivka M, Cousin C, Haguenau M
Neurol Res 1992;14(2 Suppl):97-9. doi: 10.1080/01616412.1992.11740022. PMID: 1355900

Dyscalculia and elements of the developmental Gerstmann syndrome in school children.

Spellacy F, Peter B
Cortex 1978 Jun;14(2):197-206. doi: 10.1016/s0010-9452(78)80045-8. PMID: 679700

See all (20)

Diagnosis

Clinical Screening for Posterior Cortical Atrophy.

Mendez MF, Khattab YI, Yerstein O
Cogn Behav Neurol 2022 Jun 1;35(2):104-109. doi: 10.1097/WNN.0000000000000297. PMID: 35639011

Benson's Disease or Posterior Cortical Atrophy, Revisited.

Yerstein O, Parand L, Liang LJ, Isaac A, Mendez MF
J Alzheimers Dis 2021;82(2):493-502. doi: 10.3233/JAD-210368. PMID: 34057092 Free PMC Article

Gerstmann syndrome: historic and current perspectives.

Rusconi E
Handb Clin Neurol 2018;151:395-411. doi: 10.1016/B978-0-444-63622-5.00020-6. PMID: 29519471

Spatial neglect, Balint-Homes' and Gerstmann's syndrome, and other spatial disorders.

Vallar G
CNS Spectr 2007 Jul;12(7):527-36. doi: 10.1017/s1092852900021271. PMID: 17603404

Acalculia and dyscalculia.

Ardila A, Rosselli M
Neuropsychol Rev 2002 Dec;12(4):179-231. doi: 10.1023/a:1021343508573. PMID: 12539968

See all (78)

Therapy

Gerstmann syndrome complicating polycythemia secondary to anabolic steroid use.

Long N, Bassi S, Pepito D, Akhondi H
BMJ Case Rep 2019 Jun 6;12(6) doi: 10.1136/bcr-2018-229004. PMID: 31175112 Free PMC Article

Gerstmann's syndrome associated with diagnostic cerebral angiography.

Wu YT, Chen LC, Lin SL, Chang ST
Brain Inj 2013;27(2):239-41. Epub 2013 Jan 16 doi: 10.3109/02699052.2012.694569. PMID: 23324021

Acetazolamide-induced Gerstmann syndrome.

Lee YT, Wu JC, Chan FK
Int J Clin Pract 1999 Oct-Nov;53(7):560-1. PMID: 10692744

Short-term memory deficit after focal parietal damage.

Markowitsch HJ, Kalbe E, Kessler J, von Stockhausen HM, Ghaemi M, Heiss WD
J Clin Exp Neuropsychol 1999 Dec;21(6):784-97. doi: 10.1076/jcen.21.6.784.853. PMID: 10649534

Statistical approaches to human brain mapping by functional magnetic resonance imaging.

Lange N
Stat Med 1996 Feb 28;15(4):389-428. doi: 10.1002/(SICI)1097-0258(19960229)15:4<389::AID-SIM285>3.0.CO;2-J. PMID: 8668868

See all (17)

Prognosis

Is the pathology of posterior cortical atrophy clinically predictable?

Cipriano L, Oliva M, Puoti G, Signoriello E, Bonavita S, Coppola C
Rev Neurosci 2022 Dec 16;33(8):849-858. Epub 2022 Jun 6 doi: 10.1515/revneuro-2022-0003. PMID: 35659868

Safe Resection of Gliomas of the Dominant Angular Gyrus Availing of Preoperative FMRI and Intraoperative DTI: Preliminary Series and Surgical Technique.

D'Andrea G, Familiari P, Di Lauro A, Angelini A, Sessa G
World Neurosurg 2016 Mar;87:627-39. Epub 2015 Nov 5 doi: 10.1016/j.wneu.2015.10.076. PMID: 26548825

Morbidity profile following aggressive resection of parietal lobe gliomas.

Sanai N, Martino J, Berger MS
J Neurosurg 2012 Jun;116(6):1182-6. Epub 2012 Mar 23 doi: 10.3171/2012.2.JNS111228. PMID: 22443504

Does finger training increase young children's numerical performance?

Gracia-Bafalluy M, Noël MP
Cortex 2008 Apr;44(4):368-75. Epub 2008 Jan 8 doi: 10.1016/j.cortex.2007.08.020. PMID: 18387567

Finger gnosia: a predictor of numerical abilities in children?

Noël MP
Child Neuropsychol 2005 Oct;11(5):413-30. doi: 10.1080/09297040590951550. PMID: 16306017

See all (23)

Clinical prediction guides

Is the pathology of posterior cortical atrophy clinically predictable?

Cipriano L, Oliva M, Puoti G, Signoriello E, Bonavita S, Coppola C
Rev Neurosci 2022 Dec 16;33(8):849-858. Epub 2022 Jun 6 doi: 10.1515/revneuro-2022-0003. PMID: 35659868

Cognitive world: Neuropsychology of individual differences.

Ardila A, Rosselli M
Appl Neuropsychol Adult 2018 Jan-Feb;25(1):29-37. Epub 2016 Sep 20 doi: 10.1080/23279095.2016.1232264. PMID: 27649025

Typical and atypical appearance of early-onset Alzheimer's disease: A clinical, neuroimaging and neuropathological study.

Kawakatsu S, Kobayashi R, Hayashi H
Neuropathology 2017 Apr;37(2):150-173. Epub 2017 Jan 17 doi: 10.1111/neup.12364. PMID: 28093855

The enigma of Gerstmann's syndrome revisited: a telling tale of the vicissitudes of neuropsychology.

Rusconi E, Pinel P, Dehaene S, Kleinschmidt A
Brain 2010 Feb;133(Pt 2):320-32. Epub 2009 Nov 10 doi: 10.1093/brain/awp281. PMID: 19903731

Developmental Gerstmann's syndrome.

PeBenito R, Fisch CB, Fisch ML
Arch Neurol 1988 Sep;45(9):977-82. doi: 10.1001/archneur.1988.00520330063011. PMID: 3415528

See all (23)