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PubMed2003

Dissociation between personal and extrapersonal neglect in a crossed aphasia study.

Abstract Several reports document crossed aphasia following a right cerebral infarct. However, few of them provide a detailed investigation of associated neuropsychological disorders. A personal neglect disorder with no difficulty in orienting attention in the contralesional space has not been frequently reported independently of lesion side for the language deficit. In most cases, the deficit is described in the acute period. We report the case of a patient who showed severe crossed aphasia several months after cerebral damage. In addition to his language deficit, he suffered from finger agnosia, acalculia, and right/left confusion. Although he was able to orient attention in the contralesional space, he had a persistent personal neglect disorder with severe difficulty in attending to his own body. Results suggest that right hemisphere language dominance does not preclude ipsilateral specialisation for other functions. Furthermore, the dissociation between the two spatial functions provides further confirmation that they are subserved by two independent systems.

Agnosia↗
PubMed2006

Neuropsychological deficits associated with a tumour in the posterior corpus callosum: a report of two cases.

This study reports two patients with tumours arising from the splenium of the corpus callosum that caused a memory disturbance consistent with retrosplenial amnesia as well as a variety of cognitive deficits including agraphia, acalculia, constructional apraxia and ideomotor and ideational apraxia involving both hands, but not aphasia. Patients showed disorientation to time and place, impaired recent memory and memorizing capacity; immediate and remote memory were preserved. Despite impaired performance intelligence quotients (IQ), verbal IQ was relatively spared. Patients also showed disconnection symptoms including bilateral crossed visuomotor ataxia and alexia to stimuli presented in the right visual field. In both cases, the tumour of the splenium had provoked surrounding oedema extending to the left parietal and occipital lobes.

Brain Neoplasms↗
PubMed1998

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.

Aged↗
PubMed2005

Development of a standardized occupational therapy screening tool for visual perception in adults.

Occupational therapy assessment and treatment of visual perceptual impairments are integral to the rehabilitation of clients following stroke and other acquired brain injuries. Occupational therapists need to identify the nature of visual perceptual performance impairments in order to choose rehabilitation intervention strategies appropriate for remediation of specific problems or to compensate for limitations in daily function. This paper describes the variations in visual perception terminology and occupational therapy approaches to visual perceptual assessment. Limitations of existing assessment tools for visual perception are highlighted in terms of reliability, validity, normative information, length of time to administer, and comprehensiveness in screening for visual perceptual impairment. In response to these limitations, a battery of items, called the Occupational Therapy Adult Perceptual Screening Test (OT-APST) was selected to screen adults comprehensively for impairments of agnosia, visuospatial skills including body scheme and neglect, constructional skills, apraxia, and acalculia. It also includes a subtest to evaluate functional skills directly observed during screening. This new battery has established reliability, validity and age-stratified normative data for adults 16 to 97 years of age. The OT-APST is recommended for use in conjunction with observational assessment of activities of daily living for clients with stroke and acquired brain injury.

Adult↗
PubMed2002

Postmalaria neurological syndrome: two cases from the Gambia.

We describe 2 patients with severe Plasmodium falciparum malaria whose convalescence was complicated by fever, with acute confusion and acalculia in one patient and a triad of myoclonus, tremor, and dysphasia in the other. Inflammatory changes were found in cerebrospinal fluid samples. Postmalaria neurological syndrome was diagnosed in each patient, and a therapeutic response to oral corticosteroids was seen in the second patient.

Adrenal Cortex Hormones↗
PubMed1982

Aphasia in a prelingually deaf woman.

A left parietal infarct in a prelingually deaf person resulted in an aphasia for both American Sign Language (ASL) and written and finger-spelled English. Originally the patient had a nearly global aphasia affecting all language systems. By five to seven weeks post-onset her symptoms resembled those of hearing aphasics with posterior lesions: fluent but paraphasic signing, anomia, impaired comprehension and repetition, alexia, and agraphia with elements of neologistic jargon. In addition, there was a pronounced sequential movement copying disorder, reduced short-term verbal memory and acalculia. In general, the patient's sign errors showed a consistent disruption in the structure of ASL signs which parallels the speech errors of oral aphasic patients. We conclude that most aphasic symptoms are not modality-dependent, but rather reflect a disruption of linguistic processes common to all human languages. This case confirms the importance of the left hemisphere in the processing of sign language. Furthermore, the results indicate that the left supramarginal and angular gyri are necessary substrates for the comprehension of visual/gestural languages.

Aged↗
PubMed2001

Gerstmann syndrome in systemic lupus erythematosus: neuropsychological, neuroimaging and spectroscopic findings.

Gerstmann syndrome (GS) comprises four interlaced neuropsychological symptoms including finger agnosia, right-left confusion, agraphia, and acalculia. While GS is commonly associated with focal lesions to the region of the left angular gyrus, it has also been associated with numerous diffuse etiologies including atrophy, alcoholism, carbon monoxide poisoning, lead intoxication and anaphylactic shock. Thus, a vigorous debate has emerged as to whether GS represents a syndrome arising from general brain decline or a distinct and localizing lesion. We report a right-handed patient who developed neuropsychological dysfunction secondary to systemic lupus erythematosus (SLE). Neuropsychological evaluation found the patient to exhibit symptoms consistent with the GS tetrad, as well as general cognitive decline. Magnetic resonance imaging revealed a distinct focal lesion of the left parieto-occipital white matter underlying the angular gyrus as well as diffuse atrophy. (1)H-magnetic resonance spectroscopy revealed substantial metabolic derangement in a voxel placed within the visible lesion, although substantial metabolic derangement was observed in regions remote from the focal pathology. Thus, GS in this first case in SLE would appear to comprise a focal neurological tetrad of disorders within a more general pattern of cognitive decline and metabolic derangement.

Adult↗
PubMed1996

Higher cerebral dysfunction in a case with atypical multiple sclerosis with concentric lesions.

A patient with atypical multiple sclerosis (MS) with clear concentric structure was studied using high field magnetic resonance imaging (MRI). This case was considered to be Baló's concentric sclerosis. Magnetic resonance imaging showed diffuse multiple concentric demyelinating lesions in the bilateral centrum semiovales, which finally regressed with the necrotic lesions remaining when the patient was discharged. During his clinical course, he showed some higher cerebral dysfunctions, such as memory disturbance, constructual apraxia and acalculia. He was treated with glycerin, prednisone and rehabilitation; all of which were effective in his recovery. Over a 4 month period, the patient recovered clinically, but some intellectual impairment remained.

Adult↗
PubMedDate not supplied

Impairment of parietal cortical functions associated with episodic prolonged spike-and-wave discharges.

A case of a 16-year-old girl who showed selective impairment of higher cortical functions and very slight lowering of initiative activity without overt disturbance of consciousness, during prolonged generalized spike-and-wave discharges with a parietal predominance, is reported. Neuropsychological investigations during the episode revealed a disturbance of parietal cortical functions, such as constructional apraxia, ideational or ideomotor apraxia, finger agnosia, acalculia, right-left disorientation, disturbance of visual-spatial orientation, and agraphia. The transient impairment of parietal cortical functions associated with the parietal-dominant spike-and-wave discharges was suggested as a principal pathophysiology of the clinical picture. Emphasis is placed on the importance of neurophysiological assessment in such a case.

Adolescent↗
PubMedDate not supplied

Parietal and occipital lobe epilepsy: a review.

There has been considerable recent interest in frontal lobe epileptic syndromes, and less attention paid to occipital and parietal epilepsies. The occipital and parietal lobes have arbitrary anatomical borders. The prinicpal seizure symptomatology includes somatosensory (paresthetic, painful, thermal, sexual, apraxia, disturbances of body image); visual (amaurotic, elementary and complex hallucinations, illusions) and other phenomena (anosognosia, apraxia, acalculia, alexia, aphemia, confusional states, gustatory, vertiginous, adversive, oculoclonic and eyelid flutter). The seizure symptoms are of varying localizing and lateralizing value and seizure discharges may spread rapidly and perceived symptoms may reflect secondary spread rather than the primary site of seizure onset. Recognized parietal and occipital epilepsy syndromes include benign epilepsy of childhood with centrotemporal spikes, benign epilepsy of childhood with parietal evoked spikes, benign occipital epilepsy of childhood, migraine/epilepsy syndromes, and epilepsy with bilateral occipital calcification. In addition, occipital and parietal epilepsy may be on the basis of any underlying structural lesion. There is frequently a poor correlation between clinical and EEG features. MRI and functional imaging often reveals underlying pathology. There have been no specific trials of different antiepileptic drugs for occipital and parietal seizures. Surgical treatment has its place, with attention to the risk of causing a fixed neurological deficit.

Electroencephalography↗
PubMed1990

A case of slowly progressive aphasia without generalized dementia in a Japanese patient.

A Japanese patient with a 4-year history of slowly progressive aphasia without generalized dementia is described. From 1985 on, this 61-yr-old right-handed dentist showed insidiously progressive deterioration in his speech and auditory comprehension, but has no memory disturbance, disorientation of space, time or persons, acalculia or other impairments in his behavior. His personality changes are unremarkable. He still treats patients at his clinic. General physical and neurological examinations were normal. CT and MRI (1.5T) scans showed widening of the left sylvian fissure and lateral ventricle without any vascular lesions. A PET scan demonstrated focal hypometabolism restricted to the left temporal lobe. The clinical course and picture of our patient corresponds, well to those of slowly progressive aphasia without generalized dementia, described by Mesulam.

Aphasia↗
PubMed1991

Posterior cortical dementia with alexia: neurobehavioural, MRI, and PET findings.

A progressive disorder of relatively focal but asymmetric biposterior dysfunction is described in a 54 year old right handed male. Initial clinical features included letter-by-letter alexia, visual anomia, acalculia, mild agraphia, constructional apraxia, and visuospatial compromise. Serial testing demonstrated relentless deterioration with additional development of transcortical sensory aphasia, Gerstmann's tetrad, and severe visuoperceptual impairment. Amnesia was not an early clinical feature. Judgment, personality, insight, and awareness remained preserved throughout most of the clinical course. Extinction in the right visual field to bilateral stimulation was the sole neurological abnormality. Early CT was normal and late MRI showed asymmetrical bioccipitoparietal atrophy with greater involvement of the left hemisphere. Results from positron emission tomography (PET) showed bilaterally asymmetric (left greater than right) occipitotemporoparietal hypometabolism. The metabolic decrement was strikingly asymmetric with a 50% reduction in glucose consumption confined to the left occipital cortex. The picture of occipitotemporoparietal compromise verified by MRI, PET, and neurobehavioural testing would be unusual for such degenerative dementias as Alzheimer's (AD) and Pick's disease, although atypical AD with predominant occipital lobe involvement cannot be excluded. This case supports the concepts of posterior cortical dementia (PCD) as a clinically distinct entity and for the first time documents its corresponding metabolic deficit using PET.

Atrophy↗
PubMed2002

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.

Adult↗
PubMedDate not supplied

The isolated occlusion of the angular gyri artery. A correlative neurological and anatomical study--case report.

We examined a patient who had signs of a cerebral hemisphere lesion: right hemiparesis, facial weakness, right hemianopsy, acustico-mnestic dysphasia, "empty speech," acalculia, visuo-spatial agnosia and constructional apraxia, but without changes in consciousness. Taking into account clinical signs, computed tomography and carotid angiography findings, we concluded that our patient had an infarction zone in the left temporo-parieto-occipital region, as a consequence of the isolated angular gyri artery (ANG) occlusion. Some clinical signs were a direct effect of the ANG's occlusion. Namely, this artery supplies the cortical regions of great functional significance: the planum polare and temporale, the transverse temporal gyri, the superior and middle temporal gyri, the angular and supramarginal gyri, as well as the superior, middle and inferior occipital gyri. But the other symptoms and signs could be explained by the pathophysiological effect of the cerebral edema on regions supplied by the non-occluded branches of the middle cerebral artery.

Adult↗
PubMedDate not supplied

The syndrome of unilateral tuberothalamic artery territory infarction.

The study of 3 personal cases and 5 published cases of unilateral infarct limited to the territory of the tuberothalamic artery suggests that this syndrome should be differentiated from the other thalamic syndromes. The onset is usually sudden, with moderate contralateral weakness. Sensory changes may be present but remain mild. The patients are apathetic, show perseveration and may be disoriented. In left-sided infarcts, transcortical aphasia, verbal and visual memory impairment and sometimes acalculia are found. In right-sided infarcts, hemispatial neglect, visual memory impairment and disturbed visuospatial processing are common. A decreased level of consciousness, disturbed ocular movements, severe motor weakness and delayed abnormal movements do not occur. Involvement of the ventral lateral and dorsomedial nucleus with sparing of the intralaminar nuclei, posterolateral formation and upper midbrain may explain this picture. The fact that the tuberothalamic artery arises from the posterior communicating artery, which often receives its supply from the carotid system, further justifies considering unilateral tuberothalamic infarcts as a syndrome.

Acute Disease↗
PubMed2004

Clinical, genetic, and neuropathologic characteristics of posterior cortical atrophy.

OBJECTIVE: To examine the clinical, genetic, and neuropathologic features of posterior cortical atrophy (PCA). DESIGN/METHODS: Using a broad definition of PCA as a syndrome with the insidious onset of visual dysfunction in the absence of primary ophthalmologic causes, the authors identified and then reviewed the presenting signs and symptoms, ApoE genotypes, tau haplotypes, and neuropathologic findings when available of PCA cases from two dementia research centers collected over the past 14 years. RESULTS: The authors identified 40 PCA cases. Their mean age at symptom onset was 60.5 +/- 8.9 years. There were twice as many women as men in the series. The principal types of visual impairment were simultanagnosia (82%) and visual field defect (47.5%). Acalculia, alexia, and anomia were also common. Insight was preserved in almost all (95%) early in the disorder. Neither apoE epsilon4 nor tau haplotype frequencies were different from typical Alzheimer disease (AD). Nine patients had died and underwent postmortem examination. Seven autopsied cases had AD pathology but when compared to typical AD, the neurofibrillary tangle (NFT) densities were significantly higher in Brodmann areas 17 and 18 (p < 0.05) and significantly lower in the hippocampus (p < 0.05). Two cases had corticobasal degeneration with maximal involvement of tau positive glial pathology in the posterior parietal lobe and Brodmann areas 17 and 18. CONCLUSIONS: PCA is a distinctive dementia syndrome in which the most pronounced pathologic involvement is in the occipitoparietal regions independent of the specific underlying pathology. AD was the most common pathologic cause, but its regional distribution differed from typical AD.

Adult↗
PubMed1984

Transient neuropsychological abnormalities (including Gerstmann's syndrome) during cortical stimulation.

A patient with intractable partial seizures was intensively studied before surgical removal of the epileptogenic focus. A subdural electrode array was surgically placed over the left temporoparietal cortex to better localize the epileptogenic focus and localize cortical function. In addition to speech and sensory findings, acalculia, agraphia, right-left confusion, and finger agnosia were transiently produced by electrical stimulation in the perisylvian area. These findings and their relationship to the controversy surrounding Gerstmann's syndrome are discussed.

Adolescent↗
PubMed1975

[Developmental Gerstmann syndrome associated with cerebellar neoplasm. Report of a case and review of the literature].

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.

Cerebellar Neoplasms↗