Gerstmann's Syndrome Research Articles

Posterior Cortical Atrophy with Acute Onset and Rapid Progressive Visual Symptoms: A Case Report

Posterior cortical atrophy (PCA) is a rare degenerative disease with unknown cause. In 1988, Benson et al.1 reported some patients whose cerebral images showed significant atrophy of the parieto-occipital region with progressive dementia and cortical visual dysfunction. They named this disease as PCA based on image findings. Since PCA is most often associated with the pathological findings of Alzheimer’s disease (AD), some people consider PCA as a visual variant of AD. Because PCA invades the posterior cerebral cortex, Gerstmann syndrome, Balint syndrome, and trans-cortical sensory aphasia may occur along with visual agnosia that occurs relatively early.2,3 Here we report a patient who visited our hospital with complaints of only visual symptom that rapidly progressed. We diagnosed it as PCA through clinical findings, image examinations, and other evidences. CASE REPORT

Carbon monoxide poisoning: clinical features of the victims of the explosion accident of Mitsui-Miike Mikawa coal mine 50 years ago

Clinical features of carbon monoxide poisoning have been described in 24 victims of an intense explosion accident of the Mitsui-Miike Mikawa coal mine in Japan 50 years ago; these victims were admitted to the Kyushu University Hospital as they suffered from severe poisoning. In the early stage of poisoning, all victims showed disturbed state of consciousness, varying in duration from 5.5 hours to 3 months, and the duration of unconsciousness was closely correlated to the clinical severity in the late stage. Some of the severely poisoned patients showed a transient stage of apallic syndrome. After recovery from unconsciousness, all patients presented with severe amnestic syndrome and loss of initiative. Neurologically, the extrapyramidal signs were prominent in the early stage, which gradually improved in the late stage. Variable types of agnosia and apraxia were apparent in some of the severely and moderately poisoned patients in the late stage, with prominent Gerstmann syndrome and visual-visuospatial agnosias. Since these signs showed poor improvement, the agnosia and apraxia, as well as impaired intellectual ability, remained as a sequela of the poisoning, and were one of the major causes of deficits of the patients in their daily life activities in the late stage.

Effect of the Lesions in the Globus Pallidus on Cognitive Impairment after Carbon Monoxide Poisoning

Background: Clinical manifestation of carbon monoxide (CO) poisoning depends on the concentration of CO and duration of exposure. Neurologic presentation is closely related to lesions in the brain. Case Report: A married couple suffered from CO exposure from a car engine that was used as a heater during an overnight stay in a cabin. Two weeks later, the wife developed cognitive problems. Neurologic examination was indicative of Gerstmann syndrome. However, her husband was normal. Their brain MRI showed a similar degree of diffuse white matter change. The brain MRI of the wife showed additional lesions in the globus pallidum (GP). Conclusion: The duration and amount of CO exposure experienced by the couple was the same, hence the GP lesion had a possible role in the clinical manifestations. Key Words: Carbon monoxide; Globus pallidus; White matter

A proposed reinterpretation of Gerstmann's syndrome.

Gerstmann's syndrome includes the clinical tetrad of finger agnosia, agraphia, acalculia, and right-left confusion. Some disagreement remains with regard to the exact localization of the syndrome, but most probable it involves the left angular gyrus with a subcortical extension. Several authors have suggested that a defect in mental spatial rotations could simultaneously account for acalculia, right-left disorientation, and finger agnosia. It has been also suggested that semantic aphasia is always associated with acalculia; as a matter of fact, left angular gyrus has a significant involvement in semantic processing. In this paper, it is proposed that Gerstmann's syndrome should include: acalculia, finger agnosia, right-left disorientation, and semantic aphasia, but not agraphia. When the pathology extends toward the superior parietal gyrus, agraphia can be found. A fundamental defect (i.e., an impairment in verbally mediated spatial operations) could explain these apparently unrelated clinical signs.

MS-16 * A RARE CASE OF PRIMARY CNS ANGIOSARCOMA PRESENTING AS AN INCOMPLETE GERSTMANN SYNDROME: A CASE REPORT AND REVIEW OF LITERATURE

INTRODUCTION: Primary central nervous system angiosarcoma is a very rare malignancy, with only 22 cases reported in the literature for the past 26 years. A 60-year old Filipino woman, housewife, presented with 1 week history of difficulty performing daily mathematical equations, difficulty reading words, and difficulty writing. On neurologic exam, patient was found to be oriented to person, place, and time. There was note of dyscalculia, agraphia, and right-left disorientation. There were no finger agnosia, agraphesthesia, astereognosia, nor apraxia. Cranial MRI showed a 4 x 4 x 4.1 cm mass at the left parietal lobe. Total excision of the mass done and biopsy revealed angiosarcoma of the brain. Thorough search for another systemic lesion failed to show any other angiosarcomatous lesion. The patient refused any further radiotherapy or chemotherapy. METHOD & RESULTS: Soft tissue sarcomas comprise a small percent 1% of the malignant neoplasms, and of which only 0.5% comprise the primary brain OR spine sarcoma. In the review of literature that we did, of the 22 reported cases, the tumor was commonly located supratentorially (9/22), affecting middle to late ages with 3:1 male to female ratio. It is an aggressive tumor with a <2-year (range of survival is 4 months -3½ years) survival independent of the age, location, or whether chemotherapy or radiotherapy was given. As with the best of our knowledge, there has been no recorded case of angiosarcoma which presented with Gerstmann syndrome in the literature. CONCLUSION: We described the first reported case of primary CNS angiosarcoma in the Philippines and the 23rd worldwide. We also described the first case of angiosarcoma which presented with Gerstmann synd.

P04 * A RARE CASE OF PRIMARY CNS ANGIOSARCOMA PRESENTING AS AN INCOMPLETE GERSTMANN SYNDROME: A CASE REPORT AND REVIEW OF LITERATURE

INTRODUCTION: Primary central nervous system angiosarcoma is a very rare malignancy, with only 22 cases reported in the literature for the past 26 years. A 60-year old Filipino woman, housewife, presented with 1 week history of difficulty performing daily mathematical equations, difficulty reading words, and difficulty writing. On neurologic exam, patient was found to be oriented to person, place, and time. There was note of dyscalculia, agraphia, and right-left disorientation. There were no finger agnosia, agraphesthesia, astereognosia, nor apraxia. Cranial MRI showed a 4 x 4 x 4.1 cm mass at the left parietal lobe. Total excision of the mass done and biopsy revealed angiosarcoma of the brain. Thorough search for another systemic lesion failed to show any other angiosarcomatous lesion. The patient refused any further radiotherapy or chemotherapy. METHOD & RESULTS: Soft tissue sarcomas comprise a small percent 1% of the malignant neoplasms, and of which only 0.5% comprise the primary brain OR spine sarcoma. In the review of literature that we did, of the 22 reported cases, the tumor was commonly located supratentorially (9/22), affecting middle to late ages with 3:1 male to female ratio. It is an aggressive tumor with a <2-year (range of survival is 4 months -3½ years) survival independent of the age, location, or whether chemotherapy or radiotherapy was given. As with the best of our knowledge, there has been no recorded case of angiosarcoma which presented with Gerstmann syndrome in the literature. CONCLUSION: We described the first reported case of primary CNS angiosarcoma in the Philippines and the 23rd worldwide. We also described the first case of angiosarcoma which presented with Gerstmann synd.

“With a Smile Through Tears”: The Uprooted Career of the Man Behind Gerstmann Syndrome

Austrian neuroscientist Josef Gerstmann, well known for describing Gerstmann syndrome and for pioneering works on tactile agnosia, also co-described the familial prion disorder later known as Gerstmann-Sträussler-Scheinker disease. In 1938, Nazi Germany annexed Austria (the “Anschluss”) and the three-time decorated war veteran Gerstmann was dismissed from his professorship in Vienna because of his “race.” In 1942, he unknowingly had his doctorate stripped, only to have it returned in 1955. The Gerstmann properties were seized in Vienna, resulting in a bitter postwar reclamation battle. Gerstmann immigrated to the United States quickly after the annexation and had some success in exile but never again directed a hospital. He maintained a private practice throughout his exile and, in the 1940s, had some research and consulting positions in New York. More than 75 years after the Anschluss, many questions remain unanswered about Gerstmann’s forced exile and the impact of becoming a refugee on his life and career.

Reversible homonymous hemianopia secondary to occipital lobe seizures

Occipital lobe infarcts, hemorrhages, or metastatic tumors can present with complex visual hallucinations, sensory or proprioceptive abnormalities, speech dysfunction, visual loss, and homonymous hemianopia (HH). Treatment of occipital lobe seizures is by single or multiple antiepileptic drugs. We present a case of idiopathic occipital lobe epilepsy where the seizures were controlled by antiepileptic drugs, but the HH did not subside until treated with high dose intravenous (IV) steroids.

The progression of posterior cortical atrophy to corticobasal syndrome: lumping or splitting neurodegenerative diseases?

BackgroundPosterior cortical atrophy is a clinical syndrome that is characterized by the progressive loss of visuospatial integration and is associated with neurodegenerative conditions.Case ReportWe describe a 60-year-old female with simultanagnosia, oculomotor apraxia, and optic ataxia for which she received an initial clinical diagnosis of posterior cortical atrophy. Three years later, she developed Balint's syndrome, Gerstmann's syndrome, left alien hand syndrome, smooth asymmetric (left) rigidity, cortical sensory loss, and spontaneous myoclonic jerks of the left arm, which suggested a final diagnosis of corticobasal syndrome.DiscussionThis case report indicates that corticobasal syndrome may present with visuospatial deficits.

Distinct representations of symbolic ordinality and quantity: Evidence from neuropsychological investigations in a Chinese patient with Gerstmann’s syndrome

A number of recent studies have shown conflicting evidence as to common or distinct representations between symbolic ordinality and quantity. We investigated this issue through a series of neuropsychological tests in a unique Chinese patient with the left angular gyrus and left supramarginal gyrus lesions. Behavioral experiments revealed that (1) the patient showed Gerstmann syndrome, with minimal anomia and alexia and (2) the patient showed the dissociation among number semantic representations with relatively preserved symbolic quantity knowledge and impaired processing of symbolic order meaning. Together with existing evidence in the literature, results of the current study suggest that there might be two separate cognitive representations of symbolic ordinality and quantity in logographic language according to this dissociation. Most importantly, another merit of this study is that the left angular gyrus and left supramarginal gyrus might be necessary to symbolic ordinality representation.

A rare presentation of atypical demyelination: tumefactive multiple sclerosis causing Gerstmann’s syndrome

BackgroundTumefactive demyelinating lesions are a rare manifestation of multiple sclerosis (MS). Differential diagnosis of such space occupying lesions may not be straightforward and sometimes necessitate brain biopsy. Impaired cognition is the second most common clinical manifestation of tumefactive MS; however complex cognitive syndromes are unusual.Case presentationWe report the case of a 30 year old woman who presented with Gerstmann’s syndrome. MRI revealed a large heterogeneous contrast enhancing lesion in the left cerebral hemisphere. Intravenous corticosteroids did not stop disease progression. A tumour or cerebral lymphoma was suspected, however brain biopsy confirmed inflammatory demyelination. Following diagnosis of tumefactive MS treatment with natalizumab effectively suppressed disease activity.ConclusionsThe case highlights the need for clinicians, radiologists and surgeons to appreciate the heterogeneous presentation of tumefactive MS. Early brain biopsy facilitates rapid diagnosis and management. Treatment with natalizumab may be useful in cases of tumefactive demyelination where additional evidence supports a diagnosis of relapsing MS.

Clinical Utility of Amyloid Imaging in a Complex Case of Corticobasal Syndrome Presenting with Psychiatric Symptoms.

Clinical indications of amyloid imaging in atypical dementia remain unclear. We report a 68-year-old female without past psychiatric history who was hospitalized for auditory hallucinations and persecutory delusions associated with cognitive and motor deficits. Although psychotic symptoms resolved with antipsychotic treatment, cognitive and motor impairments remained. She further showed severe visuoconstructive and executive deficits, ideomotor apraxia, elements of Gerstmann’s syndrome, bilateral agraphesthesia and discrete asymmetric motor deficits. Blood tests were unremarkable. Structural brain imaging revealed diffuse fronto-temporo-parietal atrophy, which was most severe in the parietal regions. Meanwhile, FDG-PET suggested asymmetrical fronto-temporo-parietal hypometabolism, with sparing of the posterior cingulate gyrus. A diagnosis of possible corticobasal syndrome (CBS) was made. Amyloid-PET using the novel tracer NAV4694 was ordered, and revealed significant deposition of fibrillar amyloid (SUVR 2.05). The primary diagnosis was CBS with underlying Alzheimer pathology and treatment with a cholinesterase inhibitor was initiated. Determination of underlying pathological CBS subtype is not simple even when based on extensive investigation including clinical presentation, atrophy patterns on MRI, and regional hypometabolism on FDG-PET. By contrast, amyloid imaging quickly confirmed Alzheimer pathology, and allowed rapid initiation of treatment in this complex case with early psychiatric symptoms. This case study illustrates the clinical utility of amyloid imaging in the setting of atypical cases seen in a tertiary memory clinic.

A case of developmental Gerstmann's syndrome

A case of developmental Gerstmann's syndrome

Mirror Visual Feedback for Right–Left Discrimination in a Patient With Developmental Gerstmann Syndrome

Mirror Visual Feedback for Right–Left Discrimination in a Patient With Developmental Gerstmann Syndrome

Editorial: Functional mapping

Direct cortical electrostimulation during brain surgery has been the basis of much of our understanding of functional regions of the cortex. The classic electrostimulation studies of Wilder Penfield and Herbert Jasper still guide our approaches to eloquent brain regions today. Direct cortical electrostimulation has also traditionally been the litmus test of whether neurological deficits would be incurred by tissue removal. This principle guides neurosurgeons removing lesions in the vicinity of languagebearing or sensorimotor cortex. It is also clear that removal of other known functional brain regions could result in specific neurological dysfunction (such as visual loss in occipital resection, alexia in dominant posterior temporal resection, and Gerstmann syndrome in dominant parietal lobe resection). The accompanying study by Della Puppa et al. documents an unexpected finding of mathematical processing dysfunction elicited by direct cortical electrostimulation of the nondominant parietal lobe, in the vicinity of the intraparietal sulcus.1 Data was obtained from 3 patients who were undergoing awake surgery of this area, and the authors found a reproducible dysfunction of multiplication and addition functions induced by direct cortical stimulation in the nondominant parietal lobe at relatively low amplitudes. These regions were spared and the patients had no deficit in mathematical processing postoperatively. Given that conventional wisdom holds that computational processing is housed in the dominant inferior parietal lobule, the results of this study contradict that widely held belief. Theirs is therefore an interesting and potentially important observation. It is not clear, however, that there is a 1-to-1 correlation between stimulation-induced function and focal deficit after resection. For example, the basal temporal lobe cortex on the dominant side commonly harbors language sites, yet resection of this area is routine in temporal lobe resections, and results in no detectable deficit. One must therefore interpret the results of the present study with caution. It must be remembered that mathematical processing—like many of the more complex brain functions—is likely a distributed function. The finding of a “node” in this network that could be stimulated and cause interruption in this function does not mean that this is the only such node, or even that it is a necessary one. Just like the basal temporal lobe and its relationship to language, it is possible that these areas in the nondominant parietal lobe could be safely resected despite the findings on direct cortical electrostimulation mapping. This study nicely illustrates a fairly highly localized area whose stimulation results in specific mathematical dysfunction. In order to improve our understanding of the significance of this finding, it should be followed up with focused functional MRI studies in an attempt to corroborate the significance of the direct cortical electrostimulation findings. Functional MRI could also help answer questions relating to the possibility of other brain regions involved in this function, and perhaps provide an answer to the question of where division and subtraction went. (http://thejns.org/doi/abs/10.3171/2013.4.JNS13560)

The Temporoparietal Fiber Intersection Area and Wernicke Perpendicular Fasciculus

In their recent article, Martino et al1 highlighted the anatomy and surgical importance of the temporoparietal fiber intersection area (TPFIA). We very much enjoyed reading their detailed description based upon postmortem fiber dissections, in vivo diffusion tensor imaging, and tractography as well as clinical case reports, but we noticed one significant mistake. The authors consistently mislabel the outermost, ie, lateral, of the 7 white matter tracts identified in the TPFIA as the “horizontal portion of the superior longitudinal fasciculus (SLF)” in all of their figures, the abstract and part of the text (second page of the article). Yet, the tract the authors refer to is obviously not taking a horizontal but a vertical course (cf. Figures 2 and 6 in their article). At the beginning of the results section, the authors identify it with the "posterior portion of the SLF." According to Makris et al,2 the only vertical component of the SLF belongs to its fourth subdivision (SLF IV), ie, the vertical part of the arcuate fasciculus (AF), which Martino et al1 identify with the second fiber tract in the TPFIA. So if the outermost, vertically running fiber pathways of the TPFIA do not belong to the SLF or AF, respectively, what are they and are these fibers surgically relevant? These particular association fibers had originally been discovered by Wernicke3 in monkey brains and have subsequently been called Wernicke perpendicular (or vertical occipital) fasciculus (WpF).4-7 According to the early seminal investigation by Sachs,8 a disciple of Wernicke in Wrocław (the former Silesian Breslau), WpF is part of the stratum verticale, ie, the stratum profundum convexitatis, lateral to the AF and the stratum sagittale externum which itself contains the inferior longitudinal fasciculus. The WpF connects the inferior parietal with the lower temporal and occipital lobe, and its fibers are crossed by posterior callosal commissure fibers, projection and association pathways. These crossing fibers may cause failures to identify this tract (i) in one-fourth of the postmortem dissections of Martino et al1 and (ii) in diffusion tensor imaging as well as streamline tractography. According to our own data,9 probabilistic tractography with crossing fibers modeling can reduce such false-negative trackings in the presence of perifocal tumor edema, for example. Crossing fibers also emphasize that the tracts of the TPFIA are not arranged in strictly separate but partially interwoven layers. WpF is clinically relevant and should be preserved from surgical damage whenever possible. Lesions to WpF have—in addition to those involving Mill's basotemporal language or the visual word forming area, posterior callosal fibers and the inferior longitudinal fasciculus —been associated with preangular alexia without agraphia.10-13 In this peculiar syndrome, patients are unable to read but can still write (eg, text short-message-service messages). The closer a lesion is located to the left inferior parietal lobule, the more likely it is accompanied by agraphia or other neuropsychological symptoms subsumed by Gerstmann syndrome, whereas the closer a lesion is to the inferior temporal lobe, the more likely it will involve disorders of face or color identification (eg, hyperfamiliarity for faces or color anomia).14,15 Furthermore, the anterior portion of WpF may be part of a brain network processing the age of faces.16 We have ourselves observed 4 cases in which tumors of and/or surgical access through the TPFIA (eg, to approach subsplenial or posterior hippocampal lesions from laterally) have led to alexia without agraphia that we relate to damage to the WpF. Such WpF disconnection syndrome may be transient, but the associated deficit may also persist and turn out to be quite disabling (eg, rendering a shop assistant unable to read labels of goods and to sort items onto the shelves). Therefore, we think that knowledge of the anatomy of the WpF and its correct labeling are essential, which is why we have taken the opportunity to alert the readers of Neurosurgery to the above detailed error in the otherwise excellent article of Martino et al.1 Disclosure The authors have no personal, financial, or institutional interest in any of the drugs, materials, or devices described in this article.

Gerstmann's syndrome associated with diagnostic cerebral angiography

Objective: Gerstmann's syndrome is a rare neurological disorder characterized by right–left disorientation, finger agnosia, agraphia and acalculia. Several causes for the manifestation of this rare syndrome have been reported in previous publications; however, thus far, an association between secondary diagnostic cerebral angiography and Gerstmann's syndrome has not been reported.Case report: A 48-year-old woman diagnosed with subarachnoid haemorrhage underwent a secondary diagnostic cerebral angiography 7 months after the episode. The patient showed memory impairment, agraphia, acalculia, right–left disorientation, occasional errors in speech and finger agnosia accompanied by an acute infarction in the left middle cerebral artery territory. However, she showed excellent recovery after intensive rehabilitation and conservative treatment.Conclusion: The previously reported rate of permanent neurological complications associated with diagnostic cerebral angiography was very low (0–0.5%). To the best of the authors’ knowledge, this is the first case report of Gerstmann's syndrome as a complication of cerebral angiography. This report discusses the complications associated with the neurological condition and emphasizes the need for early rehabilitation in cases of Gerstmann's syndrome.

Corticobasal Syndrome Presenting With Partial Gerstmann’s Syndrome and Digit Agnosia

Back to table of contents Previous article Next article LettersFull AccessCorticobasal Syndrome Presenting With Partial Gerstmann’s Syndrome and Digit AgnosiaMichele Poletti, Claudio Lucetti, Andrea Vergallo, and Ubaldo BonuccelliMichele PolettiSearch for more papers by this author, Claudio LucettiSearch for more papers by this author, Andrea VergalloSearch for more papers by this author, and Ubaldo BonuccelliSearch for more papers by this authorPublished Online:1 Apr 2013https://doi.org/10.1176/appi.neuropsych.12060155AboutSectionsPDF/EPUB ToolsAdd to favoritesDownload CitationsTrack Citations ShareShare onFacebookTwitterLinked InEmail To the Editor: Gerstmann’s syndrome (GS) is a clinical picture characterized by finger agnosia, left–right confusion, agraphia, and acalculia, associated with focal left posterior-inferior parietal lesions.1 Partial presentations of GS have been reported in neurodegenerative diseases,2,3 as we describe in a patient presenting with GS and digit agnosia as early symptoms.A 62-year-old, right-handed woman, with 8 years of formal education, came to our attention because of mild cognitive difficulties involving limb praxis. The neurological examination detected upper limb tremor, more left-lateralized; global bradykinesia; rigidity of left limbs; bilateral focal myoclonus, stimulus-sensitive, more evident on the left limbs; and gait apraxia; the alien limb phenomenon was not present. Instrumental examinations detected bilateral temporo-parietal alterations (EEG), cortical atrophy (RMI), bilateral hypometabolism in the frontal-temporal-parietal cortices, and bilateral striatal degeneration (DAT-SCAN).The neuropsychological assessment detected executive difficulties, impaired visuospatial functions, and bilateral ideomotor limb apraxia, and therefore focused on GS. The patient showed finger agnosia (Two-Point Finger test,1 Finger Agnosia test,1 In-Between test)1 and toe agnosia;4 that is, digit agnosia. She also presented acalculia (being unable to count backward from 10 to 0 and perform multiplication tables), Left–Right Confusion (3/5 in the two-stage command1 and 2/5 in relation to experimenter body) and agraphia (5/20 correct words in dictation; no signs of alexia), although limb motor difficulties hampered its examination.Although only a pathological examination could confirm the clinical diagnosis, her clinical picture was suggestive of a parkinsonism, probably a corticobasal syndrome (CBS); a progressive neurodegenerative disease with atrophy, gliosis, and tau-immunoreactive pathology of both gray and white matter of neocortex, basal ganglia, and substantia nigra, and clinically characterized by asymmetric akinesis and rigidity; dystonia of the upper limb; apraxia, myoclonus, and dementia.5The neuropsychological assessment evidenced digit agnosia and at least a partial GS; indeed, although her limb motor difficulties hampered the examination of agraphia, it appeared likely. Interestingly, the neuropsychological pattern of CBS partially overlaps the clinical presentation of GS as regards acalculia, number processing, and agraphia, and both CBS and GS are associated with parietal lesions.1,5 Despite their possible phenotypic overlapping, only two cases of pathologically-confirmed corticobasal degeneration and partial presentation of GS have been described.2 The current study confirmed that GS, including digit agnosia, may characterize the early clinical stages of a neurodegenerative disease affecting parietal lobes, as CBS.Dept. of Neuroscience, University of Pisa, Via Rome, Pisa, ItalyNeurology Unit, Versilia Hospital, Via Aurelia, Lido di Camaiore (LU), ItalyCorrespondence: Prof. Ubaldo Bonuccelli, Department of Neuroscience, University of Pisa, Italy; e-mail: u.[email protected]unipi.itReferences1 Rusconi E, Pinel P, Dehaene S, et al.: The enigma of Gerstmann’s syndrome revisited: a telling tale of the vicissitudes of neuropsychology. Brain 2010; 133:320–332Crossref, Medline, Google Scholar2 Wingard EM, Barrett AM, Crucian GP, et al.: The Gerstmann syndrome in Alzheimer’s disease. J Neurol Neurosurg Psychiatry 2002; 72:403–405Crossref, Medline, Google Scholar3 Tang-Wai DF, Josephs KA, Boeve BF, et al.: Pathologically confirmed corticobasal degeneration presenting with visuospatial dysfunction. Neurology 2003; 61:1134–1135Crossref, Medline, Google Scholar4 Tucha O, Steup A, Smely C, et al.: Toe agnosia in Gerstmann syndrome. J Neurol Neurosurg Psychiatry 1997; 63:399–403Crossref, Medline, Google Scholar5 Mahapatra RK, Edwards MJ, Schott JM, et al.: Corticobasal degeneration. Lancet Neurol 2004; 3:736–743Crossref, Medline, Google Scholar FiguresReferencesCited byDetailsCited BySome evidence on Gerstmann’s syndrome: A case study on a variant of the clinical disorderBrain and Cognition, Vol. 148 Volume 25Issue 2 Spring 2013Pages E70-E70 Metrics PDF download History Published online 1 April 2013 Published in print 1 April 2013

Acute parietal lobe infarction presenting as Gerstmann&amp;rsquo;s syndrome and cognitive decline mimicking senile dementia

Gerstmann’s syndrome encompasses the tetrad of finger agnosia, agraphia, acalculia, and right-left confusion. An elderly man with a history of several cardiovascular diseases was initially brought to the psychiatric outpatient department by his family because of worsening of recent memory, executive function, and mixed anxious-depressive mood. Gerstmann’s syndrome without obvious motor function impairment and dementia-like features could be observed at first. Emergent brain computed tomography scan revealed new left-middle cerebral artery infarction over the left posterior parietal lobe. This case reminds us that acute cerebral infarction involving the parietal lobe may present as Gerstmann’s syndrome accompanied by cognitive decline mimicking dementia. As a result, emergent organic workups should be arranged, especially for elderly patients at high risk for cerebral vascular accident.

Clinical Progress of Gerstmann's Syndrome with Left Frontal Lobe Lesion: Two Cases

Gerstmann’s syndrome, assigned to a lesion of the dominant parietal lobe, is a neurological disorder characterized by acalculia, agraphia, right-left disorientation and finger agnosia. Some studies report that these symptoms are also shown in other brain lesions. We report two patients who presented with this tetrad of symptoms in initial assessment. Their Brain MRI images both showed lesion of left frontal lobe. Over time, these symptoms became better but some still remained in last assessment. Accordingly, we suggest that a left frontal lesion cause Gerstmann’s syndrome. Received: December 1, 2013 Revision received: December 12, 2013 Accepted: December 12, 2013