Gerstmann Syndrome Case-Control Study: Correlation between Brain Lesions & Functional Disability.

Deep functional and structural neuroimaging of a series of Gerstmann's syndrome patients required high accuracy, and our results avoided false overlaps of heterogeneous brain lesions by handling each case of our study subjects separately as an individual case regarding functional and neuroimaging tests. Six patients with Gerstmann tetrad (one with dominant acalculia, one with dominant left and right disorientation, two with writing disabilities and two with finger agnosia) and 6 control subjects with close ages were recruited in the current study. In the main phase, we assessed brain activation in response to experimental and interventional settings using neuroimaging techniques (FMRI-Functional Magnetic Resonance Imagingwhere twelve pictures were taken on a Dell inspiration 3T all-body scanner with sequences of echo pictures, 80o angled, TE 35 ms) of the subject's brain to declare lesions existence and locations that might result in one of the four cognitive impairment domains of Gerstman's syndrome tetrad. We assessed statistically significant differences of patient images vs. control images as well as the images of patients presenting specific symptomatic cognitive dysfunction domain vs. the images of patients presenting the three other domains. Neuroimages were analyzed using multiple databases such as T1 weighted and free sequence types. Gerstmann's syndrome is mainly connected to injury in the dominant parietal lobe, so images comparisons and analysis were only restricted to the left parietal lobe region. P values <0.05were only considered as statistically significant difference in comparisons of functional tests time and accuracy of patients vs. in addition to comparisons of brain images parameters of patient group vs. control group and specific symptomatic domain patients vs. other symptomatic domains patients. Regarding functional testing, Patients group took significantly higher time compared to control group. Regarding brain images parameters, patients in each domain showed significantly different lesions compared to other domains. Moreover, control subjects showed no lesions in the left parietal lobe compared to significant lesions in the patient groups. These results oppose the theory of Gerstmann that a common brain structural injury may result in the combination of all of the four symptomatic dysfunction domains. This may be due to the fact that Gerstmann examined incomplete cases which represent a considerable criticism to his scientific basis. Moreover, he excluded patients with speech difficulties and apraxia.

White matter tract disconnection in Gerstmann's syndrome: Insights from a single case study.

It has been suggested that Gerstmann's syndrome is the result of subcortical disconnection rather than emerging from damage of a multifunctional brain region within the parietal lobe. However, patterns of white matter tract disconnection following parietal damage have been barely investigated. This single case study allows characterising Gerstmann's syndrome in terms of disconnected networks. We report the case of a left parietal patient affected by Gerstmann's tetrad: agraphia, acalculia, left/right orientation problems, and finger agnosia. Lesion mapping, atlas-based estimation of probability of disconnection, and DTI-based tractography revealed that the lesion was mainly located in the superior parietal lobule, and it caused disruption of both intraparietal tracts passing through the inferior parietal lobule (e.g., tracts connecting the angular, supramarginal, postcentral gyri, and the superior parietal lobule) and fronto-parietal long tracts (e.g., the superior longitudinal fasciculus). The lesion site appears to be located more superiorly as compared to the cerebral regions shown active by other studies during tasks impaired in the syndrome, and it reached the subcortical area potentially critical in the emergence of the syndrome, as hypothesised in previous studies. Importantly, the reconstruction of tracts connecting regions within the parietal lobe indicates that this critical subcortical area is mainly crossed by white matter tracts connecting the angular gyrus and the superior parietal lobule. Taken together, these findings suggest that this case study might be considered as empirical evidence of Gerstmann's tetrad caused by disconnection of intraparietal white matter tracts.

The making of a syndrome: Gerstmann's patients before Gerstmann syndrome.

When Gerstmann published the case report which later became known as the first case of Gerstmann syndrome, he did not claim the discovery of a new syndrome. It was only a few years later, after reporting on another two similar cases, that he isolated the famous tetrad of symptoms (finger agnosia, right-left disorientation, agraphia and acalculia) as a meaningful cluster with both localising and functional value. In this article, we provide the translation of key-excerpts of the second of Gerstmann's reports (Gerstmann, 1927) and a synoptic description of the symptoms as reported in the three original cases, which were later identified as cases of Gerstmann syndrome. The descriptions appear highly consistent across cases. Among symptoms, finger agnosia stands out for its pervasiveness, which may explain why Gerstmann considered this as the core symptom and speculated it could subtend all symptoms. However, no common functional denominator emerges from the original descriptions.

Clinical Screening for Posterior Cortical Atrophy.

BACKGROUND: Posterior cortical atrophy (PCA) is a progressive neurologic syndrome that presents with complex visual deficits. Although PCA is most commonly a form of Alzheimer disease (AD), its early diagnosis is usually delayed due to a lack of understanding for how best to clinically screen for the syndrome. OBJECTIVE: To identify neurobehavioral screening tasks for PCA-beyond simple visual constructions-that can be administered in clinic or at bedside. METHOD: We compared the performance of 12 individuals who met neuroimaging-supported consensus criteria for PCA with that of 12 matched individuals with typical AD (tAD) and 24 healthy controls (HC) on clinic/bedside tasks measuring (a) complex figure copying, (b) Balint syndrome, (c) visual object agnosia, (d) color identification, (e) figure-ground discrimination, (f) global-local processing, (g) dressing apraxia, (h) ideomotor apraxia, and (i) Gerstmann syndrome. RESULTS: All of the individuals with PCA were impaired on the figure-ground discrimination task compared with half of the tAD group and no HC. Approximately half of the PCA group had Balint syndrome, dressing apraxia, and ideomotor apraxia compared with none in the tAD group. Difficulty copying a complex figure, global-local processing impairment, and Gerstmann syndrome did not distinguish between the two dementia groups. CONCLUSION: The figure-ground discrimination task can be used successfully as an overall screening measure for PCA, followed by specific tasks for Balint syndrome and dressing and limb apraxia. Findings reinforce PCA as a predominant occipitoparietal disorder with dorsal visual stream involvement and parietal signs with spatiomotor impairments.

Benson's Disease or Posterior Cortical Atrophy, Revisited.

BACKGROUND: D. Frank Benson and colleagues first described the clinical and neuropathological features of posterior cortical atrophy (PCA) from patients in the UCLA Neurobehavior Program. OBJECTIVE: We reviewed the Program's subsequent clinical experience with PCA, and its potential for clarifying this relatively rare syndrome in comparison to the accumulated literature on PCA. METHODS: Using the original criteria derived from this clinic, 65 patients with neuroimaging-supported PCA were diagnosed between 1995 and 2020. RESULTS: On presentation, most had visual localization complaints and related visuospatial symptoms, but nearly half had memory complaints followed by symptoms of depression. Neurobehavioral testing showed predominant difficulty with visuospatial constructions, Gerstmann's syndrome, and Balint's syndrome, but also impaired memory and naming. On retrospective application of the current Consensus Criteria for PCA, 59 (91%) met PCA criteria with a modification allowing for "significantly greater visuospatial over memory and naming deficits." There were 37 deaths (56.9%) with the median overall survival of 10.3 years (95% CI: 9.6-13.6 years), consistent with a slow neurodegenerative disorder in most patients. CONCLUSION: Together, these findings recommend modifying the PCA criteria for "relatively spared" memory, language, and behavior to include secondary memory and naming difficulty and depression, with increased emphasis on the presence of Gerstmann's and Balint's syndromes.

Some evidence on Gerstmann's syndrome: A case study on a variant of the clinical disorder.

We describe the case of a bilingual patient with persistent symptoms largely, although not fully, consistent with those that are usually reported in Gerstmann's syndrome. Twenty months after a spontaneous primary intracranial hemorrhage, the patient was evaluated with a series of neuropsychological tasks and underwent an MRI investigation based on Diffusion Tensor Imaging probabilistic tractography. The patient suffered from dysgraphia (difficulty in the access to the graphemic representation of letter forms), autotopoagnosia (difficulties in locating body parts on verbal command), right-left confusion (difficulties in localizing right and left side of symmetrical body parts), and number processing/calculation impairments (predominant difficulties on transcoding tasks). Probabilistic tractography revealed a relatively spared superior longitudinal fasciculus and severe damage to the subcortical white matter connecting the angular gyrus with other parietal regions, such as the intraparietal sulcus and the supramarginal gyrus. Within the framework of the contemporary cognitive accounts of Gerstmann's syndrome, the case supports the assumption of an anatomical intraparietal disconnection more than a functional Grundstörung (core impairment).

Gerstmann's Syndrome in a Patient Double-positive for Antibodies against the N-methyl-D-aspartate Receptor and NH2-terminal of α-enolase.

We herein report a case of anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis concurrent with NH2-terminal of α-enolase (NAE) antibodies. A 36-year-old Japanese woman presented with Gerstmann's syndrome followed by jerky involuntary movements, seizure, autonomic instability, and consciousness disturbance. NAE antibodies were detected in the serum; however, NMDAR antibodies were identified in the cerebrospinal fluid with a cell-based assay, confirming the diagnosis of anti-NMDAR encephalitis. This case highlights the fact that Gerstmann's syndrome can be a manifestation of anti-NMDAR encephalitis and that NAE may be identified concurrently with NMDAR antibodies, suggesting that the diagnosis of Hashimoto encephalopathy requires the reasonable exclusion of alternative diagnoses, including anti-NMDAR encephalitis.

A case of inferior frontal gyrus infarction manifesting Gerstmann syndrome.

A 48-year-old female suffered from cerebral infarction involving the left inferior frontal gyrus. This was due to ischemic complications of endovascular treatment for subarachnoid hemorrhage. She exhibited severe acalculia, agraphia, finger agnosia, and right-left disorientation (the four features of Gerstmann syndrome), but aphasia was scarcely noticeable. Single-photon emission tomography revealed hypoperfusion in the left inferior frontal area and also in the left parietal area. It is possible that Gerstmann syndrome was caused in the present case by disruption of the association fiber connecting the inferior frontal area with the inferior parietal area.

A Rare Clinical Antity; Pure Gerstmann Syndrome.

Gerstmann syndrome is defined as a tetrad including agraphia, acalculia, finger agnosia, and right-left disorientation. In the case studies presented in the literature, it has been reported that Gerstmann syndrome usually appears as an incomplete tetrad of symptoms or accompanied by cognitive deficits including aphasia, alexia, apraxia and some perceptual disorders. Here, we present of the patient with left angular and supramarginal gyrus infarction affecting the parietal lobe. In addition to the symptoms mentioned above, the patient had alexia and anomic aphasia as well. We discussed the clinic appearance and reviewed the current literature.

Gerstmann Syndrome.

PURPOSE OF REVIEW: Gerstmann (left angular gyrus) syndrome includes the tetrad of finger agnosia (inability to distinguish, name, and recognize the fingers), agraphia (acquired disturbance in the ability to write), acalculia (loss of the ability to perform arithmetical operations and use numerical concepts), and right-left disorientation (right-left discrimination defect when using language). There is some disagreement regarding its exact localization, but it most likely involves the left angular gyrus with a probable subcortical extension. This article reviews recent research on the clinical aspects of this syndrome. RECENT FINDINGS: During the last years, just some few new reports of Gerstmann syndrome are found in neurological and neuropsychological literature. Most of the reports are single-case reports. An association between Gerstmann syndrome and the so-called semantic aphasia has been pointed out. Two different explanations to this unusual syndrome have been recently proposed: (1) the pathological process is located in the left parietal white matter disconnecting separate cortical networks and (2) it represents a disturbance in the ability to verbally mediate some spatial knowledge. Although Gerstmann syndrome continues as a controversial syndrome, and most of the reports are single case reports, recently two different explanations have been advance the understanding of this polemic but fascinating syndrome.

Left Parietal Tumors Presenting with Smartphone Icon Visual Agnosia: Two Cases of a Modern Presentation of Gerstmann Syndrome.

BACKGROUND: Gerstmanns syndrome-a clinical constellation of left-right confusion, finger agnosia, agraphia, and acalculia-is frequently attributed to pathology in the dominant inferior parietal lobe or temporo-occipital region. However, these unique clinical findings are often accompanied by more subtle signs, including aphasias, neglect, and agnosias. Associative visual agnosia, in which a patient is able to accurately perceive and describe but not recognize an object or symbol, is a well-documented but infrequently observed clinical entity. CASE DESCRIPTION: Here we detail 2 unique cases of patients who presented with the inability to recognize and use smartphone application icons. Both were found to have left temporo-occipital tumors displacing the left temporo-parietooccipital cortex. CONCLUSIONS: In the era of pervasive technology, we emphasize that smartphone icon associative visual agnosias may be recognized by discerning physicians in the clinical diagnosis of dominant parietal lobe pathology.

Gerstmann syndrome complicating polycythemia secondary to anabolic steroid use.

Anabolic steroid use is prevalent among athletes and bodybuilders. There are known cardiovascular, reproductive, musculoskeletal and neuropsychiatric risks associated with their prolonged use. Although there have been very few documented cases of strokes associated with anabolic steroid use, cardiomyopathy and secondary erythropoiesis can increase the risk of strokes in users with no other risk factors. We present a 49-year-old man with left parietal ischaemic stroke with haemorrhagic conversion resulting in Gerstmann syndrome secondary to a hypercoagulable state from chronic anabolic steroid use.

The making of a syndrome: The English translation of Gerstmann's first report.

The label Gerstmann syndrome indicates the co-occurrence of four symptoms in persons with acquired brain lesions: finger agnosia, left-right disorientation, agraphia, and acalculia. The syndrome is often associated with a lesion affecting the posterior parietal lobe of the left cerebral hemisphere. Virtually every paper discussing this tetrad of symptoms refers back to Josef Gerstmann's (1924) first report published in German. To make it accessible to the wider community of scholars and thus enable a more in-depth appreciation of the origins of this enigmatic syndrome, here we publish, for the first time in English, a translation of Gerstmann's initial report. In this paper, the syndromal construct had not yet crystallized into its four cardinal symptoms; Gerstmann's attention was mainly focused on finger agnosia and, to a lesser extent, on left-right disorientation by virtue of their significance for the body scheme theory. Although isolated agraphia and acalculia seemed to be at least as severe as finger agnosia, Gerstmann did not consider them of consequential importance (with the exception of agraphia's localisation value). It is also worth noting that the described patient presented a picture of Gerstmann syndrome associated with other symptoms such as hemianopia, balance impairment, and light memory and reasoning disorders.

Gerstmann syndrome: historic and current perspectives.

This chapter offers a perspective on the origin, operational definition, historic vicissitudes, and current status of Gerstmann syndrome. The main issues and controversy accompanying Gerstmann syndrome throughout the years are reviewed. The clinical picture of Gerstmann syndrome as it emerges from a series of modern-day pure cases is described. In current clinical practice, a diagnosis of Gerstmann syndrome indicates the concomitant presence of four acquired symptoms: finger agnosia, acalculia, left-right disorientation, and agraphia. Finally, based on empiric work conducted in recent years, the chapter concludes with a new interpretation of Gerstmann syndrome. If seen as an instance of intraparietal disconnection, this classic parietal syndrome will acquire fresh clinical and theoretic significance.

Cognitive world: Neuropsychology of individual differences.

It is proposed that depending upon the specific pattern of cognitive abilities, each individual lives in an idiosyncratic "cognitive world." Brain pathology can be associated with some disturbed abilities, and frequently experiential changes (i.e., how the world is understood) are observed. Because these patients often are aware of their intellectual changes, they may represent excellent models to illustrate the diversity of cognitive interpretations an individual can have about the surrounding environmental conditions. Four neuropsychology cases are presented to illustrate this point: (a) prosopagnosia associated with spatial agnosia; (b) Gerstmann's syndrome; (c) dysexecutive syndrome due to a head injury; and, (d) patient with Capgras' syndrome associated with a left temporal cyst. It is further emphasized that non-brain damaged people present an enormous-but usually overlooked-dispersion in different cognitive domains, resulting in specific and idiosyncratic patterns of cognitive abilities. It is concluded that the concept of "cognitive world" in neuropsychology can parallel the concept of "perceptual world" introduced by von Uexküll in biology, which assumes that different animal species live in idiosyncratic perceptual worlds, available and knowable by the differences in their sensory system abilities. That is, different individuals live in idiosyncratic cognitive worlds, owing to their differences in cognitive abilities.

Gerstmann's syndrome and unilateral optic ataxia in the emergency department / Síndrome de gerstmann e ataxia óptica unilateral na sala de emergência

ABSTRACT. A 75-year-old right-handed woman presented to the emergency department with simultanagnosia and right unilateral optic ataxia. Moreover, the patient had agraphia, acalculia, digital agnosia and right-left disorientation, consistent with complete Gerstmann's syndrome. This case highlights the concurrence of Gerstmann's syndrome and unilateral optic ataxia in the acute phase of a left middle cerebral artery stroke.

RESUMO. Uma paciente de 75 anos e destra se apresentou à sala de emergência com quadro de simultanagnosia e ataxia óptica unilateral à direita. Além disso, a paciente tinha agrafia, acalculia, agnosia digital e desorientação direita-esquerda, compatíveis com a síndrome de Gerstmann. O presente caso destaca a ocorrência simultânea da síndrome de Gerstmann com ataxia óptica unilateral na fase aguda do acidente vascular cerebral da artéria cerebral média esquerda.