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.

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.

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 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.

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.

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.

[A 68 year-old man presenting ideomotor apraxia and incomplete Gerstmann syndrome with multiple cystic lesions in the left hemisphere].

A 68-year-old man was referred to our hospital with tension-type headaches and a 1-year history of dementia. On neurologic examination, he had ideomotor apraxia and incomplete Gerstmann syndrome that was characterized by acalculia, agraphia, and finger agnosia. On imaging, multiple cystic lesions reported as "unusually dilated perivascular spaces" were observed along the medullary arteries in the left hemisphere; some of them had adjacent hyperintense areas in fluid attenuated inversion recovery images. We assumed that the multiple cystic lesions caused his higher cerebral dysfunction, because ideomotor apraxia and Gerstmann syndrome are usually indicative of a left parietal lobe lesion. MR spectroscopy in the lesion site revealed increased lactate. On MR angiography, the left middle cerebral artery and the left posterior cerebral artery were poorly visualized without localized stenosis. Technetium-99 bicisate single-photon emission computed tomography showed severely decreased cerebral blood flow in the left hemisphere. Electroencephalography showed slow waves in the left hemisphere.

[Acquired and developmental Gerstmann syndrome. Illustration from a patient with multiple sclerosis]. / Syndrome de Gerstmann acquis ou développemental. Illustration chez une patiente atteinte de sclérose en plaques.

Gerstmann's syndrome (GS) is defined by a clinical tetrad including acalculia, finger anomia, left-right disorientation and agraphia. In this article, we describe the case of a 42-year-old woman suffering from an aggressive relapsing-remitting multiple sclerosis in which a systematic neuropsychological assessment revealed Gertsmann's syndrome amongst other cognitive disturbances. Brain MRI showed a high concentration of plaques within a left subcortical parietal region that has recently been considered as a crucial node for GS appearance. However, history, taking provided information suggesting that an important part of the GS, may have been present since childhood, evoking a possible neurodevelopmental origin in this patient. This article reviews the role of the GS concept in contemporary literature, with a special attention to pathophysiological hypotheses and to precautions necessary to study such cases.

Morbidity profile following aggressive resection of parietal lobe gliomas.

OBJECT: The impact of parietal lobe gliomas is typically studied in the context of parietal lobe syndromes. However, critical language pathways traverse the parietal lobe and are susceptible during tumor resection. The authors of this study reviewed their experience with parietal gliomas to characterize the impact of resection and the morbidity associated with language. METHODS: The study population included adults who had undergone resection of parietal gliomas of all grades. Tumor location was identified according to a proposed classification system for parietal region gliomas. Low- and high-grade tumors were volumetrically analyzed using FLAIR and T1-weighted contrast-enhanced MR imaging. RESULTS: One hundred nineteen patients with parietal gliomas were identified--34 with low-grade gliomas and 85 with high-grade gliomas. The median patient age was 45 years, and most patients (53) presented with seizures, whereas only 4 patients had an appreciable parietal lobe syndrome. The median preoperative tumor volume was 31.3 cm(3), the median extent of resection was 96%, and the median postoperative tumor volume was 0.9 cm(3). Surprisingly, the most common early postoperative neurological deficit was dysphasia (16 patients), not weakness (12 patients), sensory deficits (14 patients), or parietal lobe syndrome (10 patients). A proposed parietal glioma classification system, based on surgical anatomy, was predictive of language deficits. CONCLUSIONS: This is the largest reported experience with parietal lobe gliomas. The findings suggested that parietal language pathways are compromised at a surprisingly high rate. The proposed parietal glioma classification system is predictive of postoperative morbidity associated with language and can assist with preoperative planning. Taken together, these data emphasize the value of identifying language pathways when operating within the parietal lobe.

Intracerebral metastasis of glioblastoma multiforme. Case report and literature review.

Glioblastoma multiforme is the most common primary malignant brain tumor in adults. It's characterized by a high malignancy and rapid, frequent tendency to local recurrence. Distant metastases disseminated in the brain compared to the primary lesion and outside the central nervous system are rarely reported in the literature. The case which is being presented is of a 53 year old man operated in 2008 because of Glioblastoma multiforme IV WHO in the left periventricular parietal region, in which the main symptoms were the Gerstmann syndrome, mixed aphasia and memory disturbance. The patient was operated totally followed by adjuvant radiotherapy and chemotherapy. Two years later epileptic seizures and aphasia were intensified. Due to this adverse symptoms MRI was ordered, which revealed a tumor in the left periventricular temporal region in different location compared to the primary lesion. The patient was operated by temporal lobectomy. Histopathological diagnosis was Glioblastoma multiforme IV WHO. According to the literature we analyzed the natural GBM tumor features and factors responsible for possibility to appear of the same type of tumor in another location of the brain.

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

Eighty years ago, the Austrian neurologist Josef Gerstmann observed in a few patients a concomitant impairment in discriminating their own fingers, writing by hand, distinguishing left from right and performing calculations. He claimed that this tetrad of symptoms constituted a syndromal entity, assigned it to a lesion of the dominant parietal lobe and suggested that it was due to damage of a common functional denominator. Ever since, these claims have been debated and an astute synopsis and sceptical discussion was presented 40 years ago by MacDonald Critchley in this journal. Nonetheless, Gerstmann's syndrome has continued to intrigue both clinical neurologists and researchers in neuropsychology, and more frequently than not is described in textbooks as an example of parietal lobe damage. In this review, we revisit the chequered history of this syndrome, which can be seen as a case study of the dialectic evolution of concepts in neuropsychology. In light of several modern era findings of pure cases we conclude that it is legitimate to label the conjunction of symptoms first described by Gerstmann as a 'syndrome', but that it is very unlikely that damage to the same population of cortical neurons should account for all of the four symptoms. Instead, we propose that a pure form of Gerstmann's syndrome might arise from disconnection, via a lesion, to separate but co-localized fibre tracts in the subcortical parietal white matter, a hypothesis for which we have recently provided evidence using combined imaging of functional and structural organization in the healthy brain.

[Ictal Gerstmann's syndrome in a patient with symptomatic parietal lobe epilepsy].

A 34-year-old man with astrocytoma in the left parietal lobe had symptomatic partial epilepsy, and he presented transient episodes of acalculia, agraphia and finger agnosia. Occasionally he had difficulty in finding appropriate letters when making an e-mail, and difficulty in writing and calculation. Neurological examinations revealed ictal symptoms of Gerstmann's syndrome without right to left disorientation. No other higher cortical dysfunction or neurological deficits were noted. Scalp EEGs showed frequent, regional ictal discharges in the left parietal area lasting for 60-240 seconds. These clinico-electrographical observations strongly support that epileptic seizures produced a loss of cortical higher function manifesting Gerstmann's syndrome.

Gerstmann's syndrome: can cardiac myxoma be the cause?

Cardiac myxomas are primary cardiac tumours. Clinical presentations vary. Central nervous embolism has been a constant association. We describe a case of a 40-year-old female who presented with neurological signs and symptoms of Gerstmann's syndrome secondary to a left atrial myxoma.

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

Brain-damaged patients with lesion or dysfunction involving the parietal cortex may show a variety of neuropsychological impairments involving spatial cognition. The more frequent and disabling deficit is the syndrome of unilateral spatial neglect that, in a nutshell, consists in a bias of spatial representation and attention ipsilateral to of extrapersonal, personal (ie, the body) space, or both, toward the side of the hemispheric lesion. The deficit is more frequent and severe after damage to the right hemisphere, involving particularly the posterior-inferior parietal cortex at the temporo-parietal junction. Damage to these posterior parietal regions may also impair visuospatial short-term memory, which may be associated with and worsen spatial neglect. The neural network supporting spatial representation, attention and short-term memory is, however, more extensive, including the right premotor cortex. Also disorders of drawing and building objects (traditionally termed constructional apraxia) are a frequent indicator of posterior parietal damage in the left and in the right hemispheres. Other less frequent deficits, which, however, have a relevant localizing value, include optic ataxia (namely, the defective reaching of visual objects, in the absence of elementary visuo-motor impairments), which is typically brought about by damage to the superior parietal lobule. Optic ataxia, together with deficits of visual attention, of estimating distances and depth, and with apraxia of gaze, constitutes the severely disabling Balint-Holmes' syndrome, which is typically associated with bilateral posterior parietal and occipital damage. Finally, lesions of the posterior parietal lobule (angular gyrus) in the left hemisphere may bring about a tetrad of symptoms (left-right disorientation, acalculia, finger agnosia, and agraphia) termed Gerstmann's syndrome, that also exists in a developmental form.

What ever happened to developmental Gerstmann's syndrome? Links to other pediatric, genetic, and neurodevelopmental syndromes.

Developmental Gerstmann's syndrome is a neurodevelopmental disorder infrequently described in the literature. The limited literature might result from controversy surrounding developmental Gerstmann's syndrome as a "true syndrome." Developmental Gerstmann's syndrome requires a tetrad of symptoms: left-right confusion, finger agnosia, dyscalculia, and dysgraphia, with constructional dyspraxia often included as a fifth symptom. The etiology of developmental Gerstmann's syndrome is unknown, but several hypotheses have been proposed, and none have been conclusively confirmed. Based on the paucity of recent research on developmental Gerstmann's syndrome, individuals who meet the criteria for the disorder could be given other diagnoses. A clustering of neuropsychologic features across other seemingly related disorders suggests that the conceptualization of the tetrad of symptoms traditionally associated with developmental Gerstmann's syndrome more appropriately reflects soft signs that are commonly associated with a number of other neurodevelopmental disorders. Thus, although developmental Gerstmann's syndrome is of historical interest to neurodevelopmental specialists, there appears to be no basis for considering this disorder as a unique syndrome.

Angular gyrus syndrome mimicking depressive pseudodementia.

A 67-year-old left-handed woman with a diagnosis of pseudodementia was being treated for depression with little benefit. Neuropsychological evaluations revealed features of angular gyrus syndrome, namely, agraphia, alexia, Gerstmann's syndrome and behavioural manifestations such as depression, poor memory, frustration and irritability. A computed tomographic scan showed a right occipito-temporal infarction, which had occurred 18 months earlier. The patient demonstrated aspects of language dysfunction associated with the syndrome and showed reversed lateralization of cerebral functions. Recognizing and distinguishing between angular gyrus syndrome and depression is important because the appropriate therapies differ. The use of the term pseudodementia can be misleading.

The Gerstmann syndrome in Alzheimer's disease.

BACKGROUND: It remains unclear from lesion studies whether the four signs of the Gerstmann syndrome (finger agnosia, acalculia, agraphia, and right-left confusion) cluster because the neuronal nets that mediate these activities have anatomical proximity, or because these four functions share a common network. If there is a common network, with degeneration, as may occur in Alzheimer's disease, each of the signs associated with Gerstmann's syndrome should correlate with the other three signs more closely than they correlate with other cognitive deficits. METHODS: Thirty eight patients with probable Alzheimer's disease were included in a retrospective analysis of neuropsychological functions. RESULTS: The four Gerstmann's syndrome signs did not cluster together. Finger naming and calculations were not significantly correlated. Right-left knowledge and calculations also did not correlate. CONCLUSIONS: The four cognitive functions impaired in Gerstmann's syndrome do not share a common neuronal network, and their co-occurrence with dominant parietal lobe injuries may be related to the anatomical proximity of the different networks mediating these functions.

[Clinical analysis of 7 patients with Gerstmann syndrome secondary to cerebral vascular disease].

OBJECTIVE: To analyze clinical features of patients with Gerstmann syndrome (GS). METHODS: We retrospectively analysed the clinical manifestations of 7 patients (6 men and 1 woman) with GS secondary to cerebral vascular diseases and reviewed the literatures. RESULTS: The age ranged from 51 to 70 years with a mean of 70 years. They all had sudden onset and the tetrad of GS-finger agnosia, left-right disorientation, agraphia and acalculia, 3 patients accompanied by incomplete aphasia, 3 by anomic aphasia, 2 by alexia and 1 by constructional apraxia. Cranial computed tomographic scan showed low-density focus of the left parietal lobe in 6 cases and high-density focus of the left parietal lobe in 1 case. CONCLUSION: GS has the high value in localization and the lesion is mainly localized to angular gyrus of the dominant hemisphere.