In 1924 the Austrian neuroscientist Josef Gerstmann described a rare neurological disorder which was observed by him in a few patients consisting of tetrad of symptoms; an impairment in performing calculations (acalculia), discriminating their own fingers (finger agnosia), writing by hands (agraphia) and distinguishing left from right (left-right disorientation).
This syndrome is also described in normal apparently but brain-damaged children, who present with learning disability, where it’s called developmental Gerstmann’s syndrome.
Gerstmann’s syndrome is caused by specific brain lesions which affect the posterior lobule of the parietal lobe in the dominant hemisphere, which is usually in the left hemisphere but in some patients it could be the right , especially the angular gyrus and adjacent structures (at the confluence of parietal, temporal and occipital lobes), some studies showed that it’s caused not only by lesion in the mentioned area but also in the left middle frontal lobe of the dominant hemisphere.
The cause of this syndrome may be from:-
Other diffuse etiologies are:
Other reported cases presented with Gerstmann’s syndrome, like cystic lesions with dilated perivascular spaces, as a complication of cerebral angiography, as a complication of the contrast which is used in angiography, as a complication of endovascular treatment of dural arteriovenous malformation, posterior leukoencephalopathy syndrome, necrotizing granulomatous inflammation of small-medium sized subarachnoid vessels, adverse drug reaction of Acetazolamide and embolism from left atrial myxoma.
Transient symptoms of this syndrome may occur as a result of parietal lobe epilepsy.
Because of the variation of the causes, this syndrome can occur in children, young and elderly patients, where it has been called “developmental Gerstmann’s syndrome” in children. Finding a patient with two to three symptoms of this syndrome is not rare but finding a patient with the full syndrome is rare.
The symptoms of Gerstmann’s syndrome can be explained by a disorders affecting specific areas of the brain, but some disagreements between the studies about the exact localization of the disease, for an example finger agnosia and acalculia responsible sites were found in the supramarginal gyrus or close to the intraparietal sulcus, and involvement of the superior parietal gyrus was founded to be responsible for agraphia, further studies are needed to know whether the syndrome occurs as a result of angular lesion with or without subcortical extension.
Several hypotheses have been mentioned in order to explain Gerstmann’s syndrome, but none of this hypothesis has been confirmed.
Several authors have suggested the defect which causes some symptoms (acalculia, right-left disorientation, and finger agnosia) of this syndrome is in the mental spatial rotations, it’s thought that the problem in this syndrome is in restricting the degree ’s freedom of spatial rotation; especially defective horizontal mapping.
It is thought also that pure form of Gerstmann’s syndrome is caused by disconnection from a subcortical parietal white matter lesion which affects crossing fiber tracts and disconnects separate cortical networks.
The symptoms of Gerstmann's syndrome should include; finger agnosia, acalculia in which patients understand the numbers but cannot perform a simple addition, left- right disorientation and agraphia, a study suggested that acalculia is always associated with semantic aphasia, which has a significant involvement in the angular gyrus, and the paper suggested that the syndrome should include semantic aphasia instead of agraphia, the later may occur in case of superior parietal gyrus involvement, symptoms of this syndrome may be complete or partial  and may be attributed to other cerebral symptoms and can occur in elderly and children.
This syndrome may be transient and caused as an ictal symptom in partial epilepsy, but it may occur as three symptoms out of 4.
The symptoms and signs of this disease in children may appear late in the years after perinatal asphyxia and may only suffer from other symptoms in the neonatal period; seizures.
Other symptoms and signs which may accompany this syndrome differ according to the cause of this syndrome such as; apraxia, optic ataxia, cognitive decline, numbness or weakness. Gesture imitation defects and toe agnosia may be seen as an association with finger agnosia so it may be named as digit agnosia.
So to diagnose this syndrome a complete history and physical examination should be done to guide the physician toward the cause of it.
Some special neurological examination should be done to diagnose the 4 components of Gerstmann's syndrome;
As the number of Gerstmann’s syndrome component increased the responsible brain lesion tend to be larger
Any patient with the symptoms and signs of Gerstmann’s syndrome should be evaluated by neuroimaging MRI/CT scan, abnormalities should be seen in the dominant angular gyrus with or without the involvement of the surrounding areas, abnormalities that should be seen in the imaging differs according to the cause of the syndrome.
In infarction it may be detected in CT scan as a low-density focus, but also a high-density focus in the parietal lobe has been reported in one patient with Gerstmann’s syndrome, MRI in the acute phase will show an abnormally reduced diffusivity , technetium-99 bicisate single proton emission computed tomography will also show a decreased cerebral blood flow with an increased lactate in MR spectroscopy and slowed waves in the affected hemisphere in electroencephalography, and angiography may show stenosis in the middle cerebral artery.
Studies showed the cerebral blood flow would be decreased in the non-infarcted areas also.
In patients with chronic subdural hematoma computerized tomographic (CT) scan will see an extra-axial hypodense fluid collection
In multiple sclerosis, brain MRI will show plaques in the mentioned areas, and in posterior leukoencephalopathy syndrome, MRI T2-weighted images will show an abnormal high-intensity lesion in the gray and white matter.
In necrotizing granulomatous inflammation will cause a lymphocytic pleocytosis in the cerebrospinal fluid and marked meningeal enhancement on MRI with dilated cortical venules.
Other lesions from other disorders in the parietal lobe may be detected by MRI such as JC virus, the lesions may not be limited to the parietal lobe, but may involve other lobes and other areas.
The differential diagnosis for Gerstmann’s syndrome includes:
Presence of Gerstmann’s syndrome with other disorder can make the normal life impossible, mainly due to the severe light-left disorientation. But children who present with developmental Gerstmann’s syndrome may show an improvement with intensive speech training especially when it's diagnosed early.
As the number of Gerstmann’s syndrome components increase the responsible lesion tend to be larger and the neurological impairment tend to be larger also; every patient with the 4 components of this syndrome is more likely to have severe impairment of brain function and the lesion tend to compromise the survival of the patient.
Patient with Gerstmann’s syndrome may show an excellent recovery after intensive rehabilitation and treatment, but acalculia may have a delay in the recovery. Also, children with developmental Gerstmann’s syndrome may exhibit improvement in the syndrome, but this may require early recognition and diagnosis of the syndrome.
Gerstmann’s is a neurological disorder and there is rising ethical issues with the advancing neuroscience, but overall the potential benefits of applying neuroimaging, psychopharmacology, and neurotechnology to mentally ill and healthy persons have to be carefully weighed against their potential harm.
For patients with Gerstmann’s syndrome coordination between neurologist, psychiatrist, physiotherapist, occupational therapist, home health nurses, and sometimes a neurosurgeon should be applied, in order to get a better outcome. [Level III]
|||Zeidman LA,Ziller MG,Shevell M, [PubMed PMID: 25259646]|
|||Kumar A,Dulebohn SC, Agnosia null. 2018 Jan [PubMed PMID: 29630208]|
|||João RB,Filgueiras RM,Mussi ML,de Barros JEF, Transient Gerstmann syndrome as manifestation of stroke: Case report and brief literature review. Dementia [PubMed PMID: 29213512]|
|||Zukic S,Mrkonjic Z,Sinanovic O,Vidovic M,Kojic B, Gerstmann'S syndrome in acute stroke patients. Acta informatica medica : AIM : journal of the Society for Medical Informatics of Bosnia [PubMed PMID: 23378691]|
|||PeBenito R,Fisch CB,Fisch ML, Developmental Gerstmann's syndrome. Archives of neurology. 1988 Sep [PubMed PMID: 3415528]|
|||Moore MR,Saver JL,Johnson KA,Romero JA, Right parietal stroke with Gerstmann's syndrome. Appearance on computed tomography, magnetic resonance imaging, and single-photon emission computed tomography. Archives of neurology. 1991 Apr [PubMed PMID: 2012521]|
|||Vallar G, Spatial neglect, Balint-Homes' and Gerstmann's syndrome, and other spatial disorders. CNS spectrums. 2007 Jul [PubMed PMID: 17603404]|
|||Käsmann B,Ruprecht KW, Eclamptogenic Gerstmann's syndrome in combination with cortical agnosia and cortical diplopia. German journal of ophthalmology. 1995 Jul [PubMed PMID: 7492936]|
|||Gold M,Adair JC,Jacobs DH,Heilman KM, Right-left confusion in Gerstmann's syndrome: a model of body centered spatial orientation. Cortex; a journal devoted to the study of the nervous system and behavior. 1995 Jun [PubMed PMID: 7555006]|
|||Ando Y,Sawada M,Morita M,Kawamura M,Nakano I, [Incomplete Gerstmann syndrome with a cerebral infarct in the left middle frontal gyrus]. Rinsho shinkeigaku = Clinical neurology. 2009 Sep [PubMed PMID: 19928685]|
|||Tucha O,Steup A,Smely C,Lange KW, Toe agnosia in Gerstmann syndrome. Journal of neurology, neurosurgery, and psychiatry. 1997 Sep [PubMed PMID: 9328265]|
|||Kleinschmidt A,Rusconi E, Gerstmann meets Geschwind: a crossing (or kissing) variant of a subcortical disconnection syndrome? The Neuroscientist : a review journal bringing neurobiology, neurology and psychiatry. 2011 Dec [PubMed PMID: 21670425]|
|||Kurata A,Oka H,Ohmomo T,Ozawa H,Suzuki S,Fujii K,Kan S,Miyasaka Y,Arai H, Successful stent placement for cervical artery dissection associated with the Ehlers-Danlos syndrome. Case report and review of the literature. Journal of neurosurgery. 2003 Dec [PubMed PMID: 14705737]|
|||Jeong YH,Kim JY,Koo YM,Choi JW,Whang K,Hu C,Cho SM, Endovascular Treatment of Giant Serpentine Aneurysm of the Middle Cerebral Artery. Journal of cerebrovascular and endovascular neurosurgery. 2016 Sep [PubMed PMID: 27847772]|
|||Sanjo N,Kina S,Shishido-Hara Y,Nose Y,Ishibashi S,Fukuda T,Maehara T,Eishi Y,Mizusawa H,Yokota T, Progressive Multifocal Leukoencephalopathy with Balanced CD4/CD8 T-Cell Infiltration and Good Response to Mefloquine Treatment. Internal medicine (Tokyo, Japan). 2016 [PubMed PMID: 27301518]|
|||Maeshima S,Okumura Y,Nakai K,Itakura T,Komai N, Gerstmann's syndrome associated with chronic subdural haematoma: a case report. Brain injury. 1998 Aug [PubMed PMID: 9724840]|
|||Ehrlé N,Maarouf A,Chaunu MP,Sabbagh-Peignot S,Bakchine S, [Acquired and developmental Gerstmann syndrome. Illustration from a patient with multiple sclerosis]. Revue neurologique. 2012 Nov [PubMed PMID: 22560518]|
|||Goethals M,Santens P, Posterior cortical atrophy. Two case reports and a review of the literature. Clinical neurology and neurosurgery. 2001 Jul [PubMed PMID: 11516556]|
|||Jung RE,Yeo RA,Sibbitt WL Jr,Ford CC,Hart BL,Brooks WM, Gerstmann syndrome in systemic lupus erythematosus: neuropsychological, neuroimaging and spectroscopic findings. Neurocase. 2001 [PubMed PMID: 11788743]|
|||Kato M, [Carbon monoxide poisoning: clinical features of the victims of the explosion accident of Mitsui-Miike Mikawa coal mine 50 years ago]. Brain and nerve = Shinkei kenkyu no shinpo. 2015 Jan [PubMed PMID: 25585432]|
|||Miyaji Y,Miyasaki H,Ning Z,Watanabe D,Suzuki Y,Kuroiwa Y, [A 68 year-old man presenting ideomotor apraxia and incomplete Gerstmann syndrome with multiple cystic lesions in the left hemisphere]. Rinsho shinkeigaku = Clinical neurology. 2012 [PubMed PMID: 22989905]|
|||Wu YT,Chen LC,Lin SL,Chang ST, Gerstmann's syndrome associated with diagnostic cerebral angiography. Brain injury. 2013 [PubMed PMID: 23324021]|
|||Guimaraens L,Vivas E,Fonnegra A,Sola T,Soler L,Balaguer E,Medrano J,Gandolfo C,Casasco A, Transient encephalopathy from angiographic contrast: a rare complication in neurointerventional procedures. Cardiovascular and interventional radiology. 2010 Apr [PubMed PMID: 19504154]|
|||Tsumoto T,Terada T,Tsuura M,Matsumoto H,Yamaga H,Masuo O,Itakura T, Analysis of Complications related to Endovascular Therapy for Dural Arteriovenous Fistulae. Interventional neuroradiology : journal of peritherapeutic neuroradiology, surgical procedures and related neurosciences. 2004 Mar 30 [PubMed PMID: 20587287]|
|||Yamashiro K,Kunoki M,Miura Y,Tomiyama J,Mochizuki H,Mizuno Y, [Posterior leukoencephalopathy syndrome presenting with bilateral ataxie optique: a case report]. Rinsho shinkeigaku = Clinical neurology. 2005 Jan [PubMed PMID: 15714994]|
|||Rucker JC,Biousse V,Newman NJ, Leptomeningeal enhancement and venous abnormalities in granulomatous angiitis of the central nervous system. Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society. 2003 Jun [PubMed PMID: 12782929]|
|||Lee YT,Wu JC,Chan FK, Acetazolamide-induced Gerstmann syndrome. International journal of clinical practice. 1999 Oct-Nov [PubMed PMID: 10692744]|
|||Bloom C,del Carpio-O'Donovan R,Wein T,Bégin LR, Left atrial myxoma presenting as Gerstmann syndrome. Canadian Association of Radiologists journal = Journal l'Association canadienne des radiologistes. 1996 Feb [PubMed PMID: 8548463]|
|||Sakellaridis T,Argiriou M,Koukis I,Panagiotakopoulos V,Spiliotopoulos C,Dimakopoulou A,Charitos C, Gerstmann's syndrome: can cardiac myxoma be the cause? Hellenic journal of cardiology : HJC = Hellenike kardiologike epitheorese. 2008 Jan-Feb [PubMed PMID: 18350783]|
|||Binder DK,Podlogar M,Clusmann H,Bien C,Urbach H,Schramm J,Kral T, Surgical treatment of parietal lobe epilepsy. Journal of neurosurgery. 2009 Jun [PubMed PMID: 19199502]|
|||Benton AL, THE FICTION OF THE [PubMed PMID: 21610898]|
|||Ardila A, A proposed reinterpretation of Gerstmann's syndrome. Archives of clinical neuropsychology : the official journal of the National Academy of Neuropsychologists. 2014 Dec [PubMed PMID: 25377466]|
|||Rusconi E,Pinel P,Dehaene S,Kleinschmidt A, The enigma of Gerstmann's syndrome revisited: a telling tale of the vicissitudes of neuropsychology. Brain : a journal of neurology. 2010 Feb [PubMed PMID: 19903731]|
|||Roux FE,Boetto S,Sacko O,Chollet F,Trémoulet M, Writing, calculating, and finger recognition in the region of the angular gyrus: a cortical stimulation study of Gerstmann syndrome. Journal of neurosurgery. 2003 Oct [PubMed PMID: 14567608]|
|||Roeltgen DP,Sevush S,Heilman KM, Pure Gerstmann's syndrome from a focal lesion. Archives of neurology. 1983 Jan [PubMed PMID: 6848091]|
|||Fournier del Castillo C,García-Peñas JJ,Gutiérrez-Solana LG,Ruiz-Falcó Rojas ML, [Gerstmann's syndrome in a 9 year old boy]. Revista de neurologia. 2000 Apr 16-30 [PubMed PMID: 10893737]|
|||Shimotake A,Fujita Y,Ikeda A,Tomimoto H,Takahashi J,Takahashi R, [Ictal Gerstmann's syndrome in a patient with symptomatic parietal lobe epilepsy]. Rinsho shinkeigaku = Clinical neurology. 2008 Mar [PubMed PMID: 18409543]|
|||Rusconi E,Pinel P,Eger E,LeBihan D,Thirion B,Dehaene S,Kleinschmidt A, A disconnection account of Gerstmann syndrome: functional neuroanatomy evidence. Annals of neurology. 2009 Nov [PubMed PMID: 19938150]|
|||Barbosa BJAP,de Brito MH,Rodrigues JC,Kubota GT,Parmera JB, Gerstmann's syndrome and unilateral optic ataxia in the emergency department. Dementia [PubMed PMID: 29354229]|
|||Moro V,Pernigo S,Urgesi C,Zapparoli P,Aglioti SM, Finger recognition and gesture imitation in Gerstmann's syndrome. Neurocase. 2008 [PubMed PMID: 19065283]|
|||Miller CJ,Hynd GW, What ever happened to developmental Gerstmann's syndrome? Links to other pediatric, genetic, and neurodevelopmental syndromes. Journal of child neurology. 2004 Apr [PubMed PMID: 15163095]|
|||HEIMBURGER RF,DEMYER W,REITAN RM, IMPLICATIONS OF GERSTMANN'S SYNDROME. Journal of neurology, neurosurgery, and psychiatry. 1964 Feb [PubMed PMID: 14123925]|
|||Bhattacharyya S,Cai X,Klein JP, Dyscalculia, dysgraphia, and left-right confusion from a left posterior peri-insular infarct. Behavioural neurology. 2014 [PubMed PMID: 24817791]|
|||Zhou XQ,He R,Liu Z,Wang CQ, [Clinical analysis of 7 patients with Gerstmann syndrome secondary to cerebral vascular disease]. Zhongguo yi xue ke xue yuan xue bao. Acta Academiae Medicinae Sinicae. 2002 Oct [PubMed PMID: 12905775]|
|||Russell SM,Elliott R,Forshaw D,Kelly PJ,Golfinos JG, Resection of parietal lobe gliomas: incidence and evolution of neurological deficits in 28 consecutive patients correlated to the location and morphological characteristics of the tumor. Journal of neurosurgery. 2005 Dec [PubMed PMID: 16381187]|
|||Morris HH,Lüders H,Lesser RP,Dinner DS,Hahn J, Transient neuropsychological abnormalities (including Gerstmann's syndrome) during cortical stimulation. Neurology. 1984 Jul [PubMed PMID: 6539864]|
|||Ardila A,Rosselli M,Arvizu L,Kuljis RO, Alexia and agraphia in posterior cortical atrophy. Neuropsychiatry, neuropsychology, and behavioral neurology. 1997 Jan [PubMed PMID: 9118197]|
|||Sato M,Yamamoto Y,Shimazaki S,Watanabe K, [A case of alexia with agraphia following left occipital lobe]. No to shinkei = Brain and nerve. 1987 Mar [PubMed PMID: 3580210]|
|||Areza-Fegyveres R,Caramelli P,Porto CS,Ono CR,Buchpiguel CA,Nitrini R, The syndrome of progressive posterior cortical dysfunction: A multiple case study and review. Dementia [PubMed PMID: 29213406]|
|||Wingard EM,Barrett AM,Crucian GP,Doty L,Heilman KM, The Gerstmann syndrome in Alzheimer's disease. Journal of neurology, neurosurgery, and psychiatry. 2002 Mar [PubMed PMID: 11861708]|
|||von Aster M, Developmental cognitive neuropsychology of number processing and calculation: varieties of developmental dyscalculia. European child [PubMed PMID: 11138904]|
|||Suresh PA,Sebastian S, Developmental Gerstmann's syndrome: a distinct clinical entity of learning disabilities. Pediatric neurology. 2000 Apr [PubMed PMID: 10788743]|
|||Fuchs T, Ethical issues in neuroscience. Current opinion in psychiatry. 2006 Nov [PubMed PMID: 17012939]|
|||Nicastro N,Tafer N,Schnider A,Di Pietro M, Gerstmann's Syndrome Associated with Right Parietal Hemorrhage and Arteriovenous Malformation. Journal of clinical neurology (Seoul, Korea). 2017 Jul [PubMed PMID: 28516745]|