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.

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.

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

BACKGROUND: Tumefactive 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 PRESENTATION: We 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. CONCLUSIONS: The 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.

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.

Gerstmann meets Geschwind: a crossing (or kissing) variant of a subcortical disconnection syndrome?

That disconnection causes clinical symptoms is a very influential concept in behavioral neurology. Criteria for subcortical disconnection usually are symptoms that are distinct from those following cortical lesions and damage to a single, long-range fiber tract. Yet, a recent study combining functional magnetic resonance imaging and fiber tracking concluded that a focal lesion in left parietal white matter provides the only tenable explanation for pure Gerstmann's syndrome, an enigmatic tetrad of acalculia, agraphia, finger agnosia, and left-right disorientation. Such a lesion would affect not only a single fiber tract but crossing or "kissing" of different fiber tracts and hence disconnect separate cortical networks. As fiber crossing is prominent in the cerebral white matter, the authors propose an extension to the subcortical disconnection framework that opens the door to ascribing a more diversified clinical phenomenology to white matter damage and ensuing disconnection than has been the case so far.

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.

[Incomplete Gerstmann syndrome with a cerebral infarct in the left middle frontal gyrus].

A 65-year-old right-handed man noted a sudden onset of numbness and weakness of the right hand. On the initial visit to our hospital, he showed severe acalculia, and transient agraphia (so called incomplete Gerstmann syndrome) and transcortical sensory aphasia. Brain MRI revealed a fresh infarct in the left middle frontal gyrus. The paragraphia and aphasia improved within 14 days after onset, but the acalculia persisted even at seven months after onset In an 123I-IMP SPECT study, the cerebral blood flow (CBF) was found to be decreased in the infarction lesion and its adjacent wide area, the ipsilateral angular and supramarginal gyri, and contralateral cerebellar hemisphere. We speculate that inactivation in the infarction lesion caused the CBF decrease in the non-infarcted areas due to diaschisis. This case indicates that Gerstmann syndrome can be caused by not only dysfunction of the angular gyrus but also of the left middle frontal gyrus in the dominant hemisphere.

[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.

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.

OBJECT: The goal of this study is to report the incidence and clinical evolution of neurological deficits in patients who underwent resection of gliomas confined to the parietal lobe. METHODS: Patient demographics, findings of serial neurological examinations, tumor location and neuroimaging characteristics, extent of resection, and surgical outcomes were tabulated by reviewing inpatient and office records, as well as all pre- and postoperative magnetic resonance (MR) images obtained in 28 consecutive patients who underwent resection of a glial neoplasm found on imaging studies to be confined to the parietal lobe. Neurological deficits were correlated with hemispheric dominance, location of the lesion within the superior or inferior parietal lobules, subcortical extension, and involvement of the postcentral gyrus. The tumors were located in the dominant hemisphere in 18 patients (64%); had a mean diameter of 39 mm (range 14-69 mm); were isolated to the superior parietal lobule in six patients (21%) and to the inferior parietal lobule in eight patients (29%); and involved both lobules in 14 patients (50%). Gross-total resection, documented by MR imaging, was achieved in 24 patients (86%). Postoperatively, nine patients (32%) experienced new neurological deficits, whereas seven (25%) had an improvement in their preoperative deficit. A correlation was noted between larger tumors and the presence of neurological deficits both before and after resection. Postoperatively higher-level (association) parietal deficits were noted only in patients with tumors involving both the superior and inferior parietal lobules in the dominant hemisphere. At the 3-month follow-up examination, five of nine new postoperative deficits had resolved. CONCLUSIONS: Neurological deterioration and improvement occur after resection of parietal lobe gliomas. Parietal lobe association deficits, specifically the components of Gerstmann syndrome, are mostly associated with large tumors that involve both the superior and inferior parietal lobules of the dominant hemisphere. New hemineglect or sensory extinction was not noted in any patient following resection of lesions located in the nondominant hemisphere. Nevertheless, primary parietal lobe deficits (for example, a visual field loss or cortical sensory syndrome) occurred in patients regardless of hemispheric dominance.

Writing, calculating, and finger recognition in the region of the angular gyrus: a cortical stimulation study of Gerstmann syndrome.

OBJECT: In an attempt to gain a better understanding of the cerebral functions represented in the angular gyrus and to spare them during surgery, the authors studied patients with brain tumors located close to the angular gyrus and mapped cortical sites by using electrostimulation. METHODS: Before undergoing tumor removal, six right-handed patients (five with left and one with right hemisphere tumors) were studied using cortical mapping with the aid of calculating, writing, finger-recognition, and color-naming tasks in addition to standard reading and object-naming tasks (for a total of 36 brain mapping studies). Strict conditions of functional site validation were applied to include only those cortical sites that produced repetitive interferences in the function tested. Preoperatively, four of the patients exhibited discrete symptoms related to Gerstmann syndrome while performing very specific tasks, whereas the other two patients presented with no symptoms of the syndrome. No patient had significant language or apraxic deficits. Distinct or shared cortical sites producing interferences in calculating, finger recognition, and writing were repeatedly found in the angular gyrus. Object- or color-naming sites and reading-interference sites were also found in or close to the angular gyrus; although frequently demonstrated, these latter results were variable and unpredictable in the group of patients studied. Finger agnosia and acalculia sites were also found elsewhere, such as in the supramarginal gyrus or close to the intraparietal sulcus. Mechanisms involved in acalculia, agraphia, or finger agnosia (either complete interferences or hesitations) during stimulation were various, from an aphasia-like form (for instance, the patient did not understand the numbers or words given for calculating or writing tasks) to an apparently pure interference in the function tested (patients understood the numbers, but were unable to perform a simple addition). CONCLUSIONS: Symptoms of Gerstmann syndrome can be found during direct brain mapping in the angular gyrus region. In this series of patients, sites producing interferences in writing, calculating, and finger recognition were demonstrated in the angular gyrus, which may or may not have been associated with object-naming, color-naming, or reading sites.

Pathologically confirmed corticobasal degeneration presenting with visuospatial dysfunction.

Corticobasal degeneration (CBD) typically manifests as progressive asymmetric rigidity and apraxia, although other non-motor presentations have been reported. We report two patients with pathologically diagnosed CBD who presented with prominent visuospatial dysfunction. The pathological changes were maximal in the visual association cortices, but absent in 31 cases of pathologically proven CBD with more typical antemortem features. Underlying CBD should be considered in the differential diagnosis of patients with findings reflecting posterior cerebral dysfunction.