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.

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.

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.

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.

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.

[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.

A disconnection account of Gerstmann syndrome: functional neuroanatomy evidence.

OBJECTIVE: To examine the functional neuroanatomy that could account for pure Gerstmann syndrome, which is the selective association of acalculia, finger agnosia, left-right disorientation, and agraphia. METHODS: We used structural and functional neuroimaging at high spatial resolution in healthy subjects to seek a shared cortical substrate of the Grundstörung posited by Gerstmann, ie, a common functional denominator accounting for this clinical tetrad. We construed a functional activation paradigm that mirrors each of the four clinical deficits in Gerstmann syndrome and determined cortical activation patterns. We then applied fiber tracking to diffusion tensor images and used cortical activation foci in the four functional domains as seed regions. RESULTS: None of the subjects showed parietal overlap of cortical activation patterns from the four cognitive domains. In every subject, however, the parietal activation patterns across all four domains consistently connected to a small region of subcortical parietal white matter at a location that is congruent with the lesion in a well-documented case of pure Gerstmann syndrome. INTERPRETATION: Our functional neuroimaging findings are not in agreement with Gerstmann's postulate of damage to a common cognitive function underpinning clinical semiology. Our evidence from intact functional neuroanatomy suggests that pure forms of Gerstmann's tetrad do not arise from lesion to a shared cortical substrate but from intraparietal disconnection after damage to a focal region of subcortical white matter.

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.

Pure global acalculia following a left subangular lesion.

We describe the case of a right-handed patient who presented a severe acalculia in the context of a pure Gerstmann syndrome following a subangular lesion that spared the left inferior parietal lobule (IPL). The patient showed impairments in Arabic and verbal codes, in number production and comprehension, as well as in numerical facts and problem solving. By using the EC301 calculation battery, semantic and syntactic tasks in Arabic and verbal codes, we tested the different hypotheses raised by the cognitive neuropsychological models of acalculia. The patients' difficulties, which were not associated with a general intellectual deterioration, and those affecting number processing as a particular semantic class, were indicative of a "global acalculia". This deficit, which exceeded the anarithmetia usually described in Gerstmann syndrome following left IPL lesion, suggested that the isolation of this area may constitute a sufficient condition for producing such a global acalculia. These results are discussed in terms of a disorder in the manipulation of mental images of spatially related objects.

Posterior cortical atrophy. Two case reports and a review of the literature.

We present two cases of progressive early-onset dementia with apraxia and visuospatial disability as initial manifestations. In the later stages of the illness Gerstmann's and Balint's syndromes developed. Structural neuroimaging demonstrated parieto-occipital atrophy and functional imaging revealed bilateral hypometabolism and hypoperfusion in these areas. These cases fulfil the diagnostic criteria of posterior cortical atrophy (PCA). Frontal lobe involvement became evident as the disease progressed. Alzheimer's disease also typically features this anterior spread and possibly this is the underlying pathological substrate for this clinical syndrome, although definite pathology is lacking. In this report, we describe longitudinal evolution in these two cases of PCA.

Toe agnosia in Gerstmann syndrome.

The following case report presents a patient exhibiting Gerstmann syndrome accompanied by toe agnosia. A 72 year old right handed woman had a focal lesion in the angular gyrus of the left hemisphere which was caused by a glioblastoma multiforme. The first symptom she had complained of was severe headache. Standardised neuropsychological tests of intelligence, memory, attention, fluency, apraxia, and language functions as well as tests for the assessment of agraphia, acalculia, right-left disorientation, and digit agnosia were performed. The patient displayed all four symptoms of the Gerstmann syndrome--namely, agraphia, acalculia, right-left disorientation, and finger agnosia. The patient did not display aphasia, constructional apraxia, or any other neuropsychological impairment. In addition to the four symptoms of the Gerstmann syndrome an agnosia of the toes was found. Further studies should determine whether finger agnosia in Gerstmann syndrome is usually accompanied by toe agnosia. Finger agnosia in the context of this syndrome may be better named digit agnosia.

[Prion diseases in men]. / Les maladies à prions chez l'homme.

Since the outbreak of "mad cow disease" and, more recently, the occurrence of cases of iatrogenic Creutzfeldt-Jakob disease in children who received pituitary extracts, there has been increasing public awareness and concern regarding the spongiform encephalopathies. These disorders appear to be caused by an extraordinary agent, unlike any previously described, called a "prion". All are progressive dementing diseases which are not associated with any specific immune or inflammatory response. At present there is no effective treatment.

Gerstmann's syndrome.

Recent case reports describe the occurrence of a more or less pure Gerstmann syndrome in association with a focal lesion in the posterior perisylvian territory of the brain's left hemisphere. In addition, an electrocortical stimulation study reported the Gerstmann symptom combination and a number of other symptom combinations on stimulation of small areas in the left posterior parietotemporal cortex. The neuropsychological implications of these and other recent findings are considered in light of the variety of "syndromes" produced by lesions in this region, the rare occurrence of Gerstmann's syndrome, and its appearance as a consequence of lesions in diverse cerebral areas.

Right parietal stroke with Gerstmann's syndrome. Appearance on computed tomography, magnetic resonance imaging, and single-photon emission computed tomography.

We examined a patient who exhibited Gerstmann's syndrome (left-right disorientation, finger agnosia, dyscalculia, and dysgraphia) in association with a perioperative stroke in the right parietal lobe. This is the first description of the Gerstmann tetrad occurring in the setting of discrete right hemisphere pathologic findings. A well-localized vascular lesion was demonstrated by computed tomography, magnetic resonance imaging, and single-photon emission computed tomographic studies. The patient had clinical evidence of reversed functional cerebral dominance and radiologic evidence of reversed anatomic cerebral asymmetries.

[A case of spongiform encephalopathy with ataxia and amyloid plaques].

CASE: A 63-year-old man was admitted to our hospital because of gait disturbance. His daughter died of the same disorder at age 29. In 1974, at age 59, the patient noticed mild recent memory disturbance and clumsiness in handwriting. In 1976, his gait was markedly unstable, but he could walk without assistance, and his speech became dysarthric. In spring, 1977, he was unable to walk without assistance. From Jan. 9, 1978, through Feb. 14, 1978, he was hospitalized in our hospital. Neurological examination revealed an ataxic gait and scanning speech. Deep tendon reflexes of the upper extremities and ankle jerks were normal, but knee jerks were absent. Pathological reflexes were not elicited. Both superficial and deep sensations were normal. He was alert and showed no overt dementia. Laboratory data including CSF, EEG and brain CT were normal except positive TPHA in serum. Thereafter, he was followed up with the diagnosis of spinocerebellar degeneration. In 1979, he developed a limb ataxia. In spring, 1980, he became very irritable and was easily excited. He gradually developed dementia and urinary incontinence. In 1981, his illness progressed to an akinetic mutism and died of pneumonia on July 19, 1981. Myoclonus and periodic synchronous discharges in EEG were not observed until his death. The brain was 1,075 g in weight after formalin fixation and the cerebrum was generally soft. The gyri showed no evidence of abnormalities or atrophy, while the cerebellum and brain stem, especially the pons, were atrophic. The arterial and venous systems showed no remarkable changes. The spinal cord was externally unremarkable. The ventricles were dilated.(ABSTRACT TRUNCATED AT 250 WORDS)