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).

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.

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.

Typical and atypical appearance of early-onset Alzheimer's disease: A clinical, neuroimaging and neuropathological study.

The International Working Group (IWG) has classified Alzheimer's disease (AD) as two different types, the typical form and the atypical form, but clinicopathological studies of atypical AD are limited. Because atypical AD cases usually present with early-onset dementia, we investigated 12 patients with early-onset AD, including two patients with typical AD and 10 patients with atypical AD. Of these patients, six had the posterior variant, three had the frontal variant and one had the logopenic variant mixed with semantic dementia. We reported MRI, single-photon emission CT and neuropathological findings in six representative cases. We also described a "left temporal variant" of AD presenting with transcortical cortical sensory aphasia, which has not been reported previously and is another subtype of the posterior variant of AD. We found a significant correlation between regional cerebral blood flow and counts of NFTs in the cerebral cortices. An atypical presentation with focal neuropsychological symptoms roughly correlated with the density of NFTs in the cerebral cortex and more directly related to spongiform changes in the superficial layers of these areas. In contrast, the distribution of amyloid depositions was diffuse and did not necessarily correlate with focal neuropsychological symptoms. Braak staging or ABC score is not necessarily appropriate to evaluate atypical AD, and instead, spongiform changes in addition to tau pathology in the association cortices better explain the diversity of atypical AD. Interestingly, another patient with a posterior variant of AD had a novel type of atypical plaque, which we referred to as "lucent plaque". They were recognizable with HE staining in the circumference and dystrophic neurites were abundant with Gallyas-Braak staining. These plaques demonstrated intense immunoreactivity to both tau AT-8 and amyloid ß (Aß), suggesting a peculiar coexistence pattern of amyloid and tau in these plaques. Clinicopathological studies of atypical AD will provide a new understanding of the pathophysiology of AD.

Finger recognition and gesture imitation in Gerstmann's syndrome.

We report the association between finger agnosia and gesture imitation deficits in a right-handed, right-hemisphere damaged patient with Gerstmann's syndrome (GS), a neuropsychological syndrome characterized by finger and toe agnosia, left-right disorientation and dyscalculia. No language deficits were found. The patient showed a gestural imitation deficit that specifically involved finger movements and postures. The association between finger recognition and imitation deficits suggests that both static and dynamic aspects of finger representations are impaired in GS. We suggest that GS is a disorder of body representation that involves hands and fingers, that is, the non-facial body parts most involved in social interactions.

[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.

Gerstmann's syndrome associated with chronic subdural haematoma: a case report.

We report a patient who exhibited Gerstmann's syndrome in association with a chronic subdural haematoma. A 71-year-old right-handed woman presented with mild right arm and leg weakness that began 2 weeks prior to admission. Neurological examination on admission revealed a mild right hemiparesis. Neuropsychological examination revealed right-left disorientation, finger agnosia, agraphia, and acalculia, but no language disturbance. A computerized tomographic (CT) scan revealed a large left frontoparietal, extra-axial hypodense fluid collection containing scattered hypodense foci. A left parietal evacuation of the haematoma was performed. Following surgery the patient dramatically improved. We suggest that the direct compression by the chronic subdural haematoma or a hemispheric pressure difference caused Gerstmann's syndrome. This is an unusual report of a Gerstmann's syndrome following chronic subdural haematoma.

Gerstmann's syndrome following an acute herpes simplex encephalitis.

The authors present a rare clinical case of a woman who developed Gerstmann's syndrome following an acute Herpes simplex viral encephalitis. Clinical observation and laboratory evaluation were performed during the acute phase of the disease. After that the follow-up continued for one-year period. The localization of the pathologic process was determined by computerized tomography, conducted periodically. The characteristics of the clinical picture are interpreted in the context of the contemporary concepts of the topical diagnosis of Gerstmann's syndrome. The possibility of a sudden onset of acute Herpes simplex viral encephalitis without a preceding febrile-intoxication syndrome is worth noting. Conclusions are drawn stressing the need of an early etiologic treatment and the importance of the rehabilitation activities during the convalescence period.

Gerstmann tetrad in leopard syndrome.

We report a 12-year-old boy with multiple lentigines (Leopard) syndrome who was evaluated for learning difficulties and Gerstmann tetrad syndrome (i.e., dyscalculia, left-right disorientation, finger agnosia, and dysgraphia). Cranial computed tomography revealed left ventriculomegaly, more pronounced in the occipital horn suggesting mild atrophy of the left parietal lobe. This is the first report of an association between the Leopard and Gerstmann syndromes and one of the few to demonstrate a computed tomographic abnormality in the latter.

[Developmental Gerstmann syndrome associated with cerebellar neoplasm. Report of a case and review of the literature]. / Sindrome de Gerstmann de desenvolvimento associada a neoplasia cerebelar. Relato de um caso e revisão da literatura

A Developmental Gerstmann syndrome in a 7 years-old-boy with hyperactivity, short attention span, acalculia, agraphia, right-left confusion, finger agnosia and constructional apraxia is reported. An initial trial with methylphenidate was done with good improvement regarding hyperactivity, attention span and constructional apraxia. In the clinical course developed a cerebellar syndrome and intracranial hypertension. Surgical exploration of the cerebellum discovered a medulloblastoma. The author believes that this is the first described association of Developmental Gerstmann syndrome and cerebellar neoplasia. The clinical findings, the etiology and the topography of the Gerstmann syndrome are discussed.