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PubMed1992

Neuropsychological deficits in a case of H2S anoxic encephalopathy.

A comprehensive examination of the neuropsychological status of a young male patient of anoxic encephalopathy due to acute hydrogen sulfide intoxication was made. Agraphia, acalculia, deficits of associative verbal fluency and design fluency, and general intellectual deterioration were noted. Qualitative analysis of number operation revealed a specific impairment in the performance of division. Further analysis of graphic output showed a phonological substitution feature. The disorder of written language in the absence of disorders of language and communication in other modalities and praxis can be considered pure agraphia. The possible neural substrate for pure agraphia and partial acalculia was also discussed.

Journal Article↗
PubMed1995

Clinico-pathological correlation in dementias.

The object of this study is to investigate whether or not there are clinical signs and symptoms in patients with dementia that, by themselves or jointly, can be associated with the pathological diagnosis of Alzheimer's disease. Twelve patients with dementia were studied, in whom the clinical diagnosis of Alzheimer's disease was made according to established criteria. A sample of leptomeninges, cortex and subcortical white matter was obtained from each patient and was processed for light and electron microscopy. In the cases in whom neuritic plaques and neurofibrilary tangles were present, pathological changes were quantified. The diagnosis of Alzheimer's disease was confirmed in 5 cases, whereas in 3 patients spongiform encephalopathy was present. In the remaining patients, the number of neuritic plaques was within normal limits for the age of the subjects. Comparison of the data in Alzheimer (n = 5) and non-Alzheimer (n = 7) groups showed an increased, statistically significant incidence of acalculia, abnormalities of judgment, impairment of abstraction and primitive reflexes in the former. Although good fitting models were obtained, none achieved perfect discrimination. The model that included alterations of judgment and acalculia gave the best fit.

Aged↗
PubMed2006

Cognitive impairment and celiac disease.

OBJECTIVE: To characterize the clinical, radiological, and electrophysiological laboratory profiles and histological features of patients who developed cognitive impairment temporally associated with celiac disease. DESIGN: Case series. SETTING: Referral center. PATIENTS: Patients with the onset of progressive cognitive decline within 2 years of symptomatic onset or with a severe exacerbation of biopsy-proved adult celiac disease were identified from the Mayo Clinic medical records from January 1, 1970, to December 31, 2005. Patients were excluded if an alternate cause of their cognitive impairment was identified. RESULTS: Thirteen patients (5 women) were identified. The median age at cognitive impairment onset was 64 years (range, 45-79 years), which coincided with symptom onset or exacerbation of diarrhea, steatorrhea, and abdominal cramping in 5 patients. Amnesia, acalculia, confusion, and personality changes were the most common presenting features. The average initial Short Test of Mental Status score was 28 of a total of 38 (range, 18-34), which was in the moderately impaired range. The results of neuropsychological testing suggested a trend of a frontosubcortical pattern of impairment. Ten patients had ataxia, and 4 of them also had peripheral neuropathy. Magnetic resonance imaging of the head showed nonspecific T2 hyperintensities, and electroencephalography showed nonspecific diffuse slowing. Deficiencies in folate, vitamin B(12), vitamin E, or a combination were identified in 4 patients, yet supplementation did not improve their neurological symptoms. Three patients improved or stabilized cognitively with gluten withdrawal. A detailed histological analysis revealed nonspecific gliosis. CONCLUSIONS: A possible association exists between progressive cognitive impairment and celiac disease, given the temporal relationship and the relatively high frequency of ataxia and peripheral neuropathy, more commonly associated with celiac disease. Given the impact for potential treatment of similar cases, recognition of this possible association and additional studies are warranted.

Aged↗
PubMed2003

Corticobasal degeneration as a cognitive disorder.

The presence of cognitive impairment in corticobasal degeneration (CBD) is now widely recognised. Our review of the literature reveals that, although the pattern and severity of neuropsychological impairments can be highly variable across patients, several general trends can be identified. The most characteristic impairments are limb apraxia (usually ideomotor), constructional and visuospatial difficulties, acalculia, frontal dysfunction, and nonfluent aphasia. The limb apraxia is associated with deficits in drawing, copying, and handwriting, but there is emerging evidence that the problems with handwriting are not due exclusively to the apraxia. The findings with respect to episodic memory are more variable, but when there is impairment in this area, it tends to be milder than that seen in Alzheimer's disease. Semantic memory functioning appears relatively preserved but has been poorly studied. Problems with speech are common, and may be due to dysarthria or buccofacial apraxia. Aphasia, although initially considered rare, is in fact a common accompaniment of CBD, may be the presenting feature, and is typically nonfluent in type. More systematic investigation of the clinical and neuropathological overlap between progressive nonfluent aphasia (generally considered to be a form of frontotemporal dementia) and CBD is needed.

Aphasia↗
PubMed1993

Spatial agraphia.

Twenty-one patients with right hemisphere damage were studied (11 men, 10 women; average age = 41.33; age range 19-65). Subjects were divided into two groups: pre-Rolandic (6) and retro-Rolandic (15) right hemisphere damaged patients. A special writing test was given to each patient. The writing errors observed included literal substitutions, feature omissions and additions, letter omissions and additions, inability to maintain horizontal writing, inappropriate grouping and fragmentation of elements, and changes in handwriting style. Associated disorders included left-hemiparesis, visual field defects, spatial hemi-neglect, constructional apraxia, spatial alexia, and spatial acalculia. It is proposed that spatial agraphia is related to: (1) left hemi-neglect, (2) constructional deficits, (3) general spatial defects, and (4) some motor disautomatization and tendency to perseverate. In cases of right frontal damage, motor-associated deficits (iterations of features and letters) predominated, whereas in cases of posterior right hemisphere damage, spatial defects (inappropriate distribution of written material in the space, grouping of letters belonging to different words, and splitting of words) were more evident. Writing impairments are in general more noticeable in cases of retro-Rolandic damage.

Adult↗
PubMed1999

[Neuropsychology of normal pressure hydrocephalus].

Although dementia is described as one of the constituent characteristics of normal pressure hydrocephalus (NPH), alongside gait disturbances and urinary incontinence, there is a rather limited number of controlled studies concerning neuropsychological deficits in the disease. A wide range of psychopathologically relevant symptoms have been described, but the common features of most cases include mental and motor slowing, apathy, emotional indifference, anosognosia, memory and attentional impairment. A number of other functional deficits such as dyslexia, dysgraphia, acalculia, apraxia can also frequently be found. Some emphasis is put on the work of J. de Mol (Brussels) which appears to be most important for the study of neuropsychological symptoms in NPH patients. The methodological standard of a number of studies has been found to be rather low, and yet a sound neuropsychological investigation may be of utmost importance for the diagnosis and neurosurgical outcome assessment. Concerning morphological correlates of the functional deficits in NPH, various hypotheses have been formulated, but it is argued that symptoms can neither be described as predominantly "diffuse" in nature, nor can they be reduced to unilocular dysfunctions. Recommendations for future research strategies are formulated.

Adolescent↗
PubMed2006

Posterior cortical atrophy: a brief review.

Posterior cortical atrophy is a striking clinical syndrome in which a dementing illness begins with visual symptoms. Initially, the problem may seem to be loss of elementary vision, but over time the patient develops features of visual agnosia, topographical difficulty, optic ataxia, simultanagnosia, ocular apraxia (Balint's syndrome), alexia, acalculia, right-left confusion, and agraphia (Gerstmann's syndrome), and later a more generalized dementia. Occasional patients have visual hallucinations and signs of Parkinson's disease or Lewy body dementia. A number of different neuropathologic disorders are associated with posterior cortical atrophy.

Atrophy↗
PubMed1989

Calculation deficits in patients with right and left hemisphere damage.

Calculation abilities were studied in 41 left and 21 right hemisphere damaged patients. Their results were compared with a normal group matched by age, sex and educational level. Left hemisphere patients were grouped according to the type of accompanying aphasia. All patient groups presented at least a certain degree of acalculia. However, maximum difficulties were found in retrorolandic left hemisphere patients. Right hemisphere patients, especially retrorolandic patients, also presented impairments in calculation abilities, resulting mainly from spatial defects. Different types of errors were analyzed. Observed errors may be similar, but due to highly different neuropsychological dysfunctioning. Primary and secondary observed defects may be attributed to a diverse array of underlying deficits.

Adolescent↗
PubMed1981

False aneurysm with subdural hematoma and symptomatic vasospasm following head injury.

A rare case of cortical false aneurysm in a subdural hematoma and symptomatic vasospasm following head injury without skull fracture is reported. Angiography led to the correct preoperative diagnosis and successful surgical treatment, although the patient's symptoms of acalculia, agraphia, and astereognosis, mainly due to the vasospasm of the proximal portion of the middle cerebral artery, remained unchanged. The mechanism of such a cortical false aneurysm and symptomatic vasospasm is discussed briefly.

Adult↗
PubMed2004

Arithmetic and the brain.

Recent studies in human neuroimaging, primate neurophysiology, and developmental neuropsychology indicate that the human ability for arithmetic has a tangible cerebral substrate. The human intraparietal sulcus is systematically activated in all number tasks and could host a central amodal representation of quantity. Areas of the precentral and inferior prefrontal cortex also activate when subjects engage in mental calculation. A monkey analogue of these parieto-frontal regions has recently been identified, and a neuronal population code for number has been characterized. Finally, pathologies of this system, leading to acalculia in adults or to developmental dyscalculia in children, are beginning to be understood, thus paving the way for brain-oriented intervention studies.

Animals↗
PubMed2005

Dexterity with numbers: rTMS over left angular gyrus disrupts finger gnosis and number processing.

Since the original description of Gerstmann's syndrome with its four cardinal symptoms, among which are finger agnosia and acalculia, the neuro-cognitive relationship between fingers and calculation has been debated. We asked our participants to perform four different tasks, two of which involved fingers and the other two involving numbers, during repetitive transcranial magnetic stimulation (rTMS) over the posterior parietal lobe of either hemisphere. In the finger tasks, they were required to transform a tactile stimulus randomly delivered on one of their fingers into a speeded key-press response either with the same or with the homologous finger on the opposite hand. In the numerical tasks, they were asked to perform a magnitude or a parity matching on pairs of single digits, in the context of arithmetically related or unrelated numerical primes. In accordance with the original anatomical hypothesis put forward by Gerstmann [Gerstmann, J. (1924). Fingeragnosie: eine umschriebene Stoerung der Orienterung am eigenen Koerper. Wiener clinische Wochenschrift, 37, 1010-12], we found that rTMS over the left angular gyrus disrupted tasks requiring access to the finger schema and number magnitude processing in the same group of participants. In addition to the numerous studies which have employed special populations such as neurological patients and children, our data confirm the presence of a relationship between numbers and body knowledge in skilled adults who no longer use their fingers for solving simple arithmetical tasks.

Adult↗
PubMed2005

Brain magnetic resonance imaging and single photon emission computerized tomography findings in a case of relapsing polychondritis showing cognitive impairment and personality changes.

A 57-year-old female with relapsing polychondritis (RP) showing CNS complications is described with reference to the MRI and SPECT findings. At the age of 56, the patient was diagnosed with RP based on her symptoms, which included bilateral auricular chondritis, nonerosive seronegative inflammatory polyarthritis, and ocular inflammation, as well as vestibular dysfunction. During the course of the disease, she suffered from aseptic meningitis accompanied by delirium, which was resolved by treatment with cyclophosphamide, in addition to prednisolone concurrent with the improvement of her physical condition. After the meningitis improved, she developed a number of cognitive impairments, including time disorientation, agraphia, acalculia, constitutional apraxia, and personality changes. Simultaneously, we observed, via SPECT, a decrease in cerebral blood flow (CBF) in several regions, including the bilateral frontal, lateral temporal, and parietal regions. This is the first study to report on the CNS manifestations of RP with reference to MRI and SPECT findings.

Brain Mapping↗
PubMed1995

Right-left confusion in Gerstmann's syndrome: a model of body centered spatial orientation.

Gerstmann's syndrome encompasses the tetrad of finger agnosia, agraphia, acalculia and right-left confusion and is associated with lesions of the dominant angular gyrus. The localizing value of this syndrome has been questioned because multiple mechanisms can account for each of the components of the syndrome. We present the case of a man who developed Gerstmann's syndrome following a focal infarct of the left angular gyrus. The patient's right-left confusion could not be accounted for by either an aphasia or a degraded body schema. A series of experiments that investigated the patient's spatial mapping system by progressively restricting the degrees of freedom for spatial rotation revealed an isolated defect in deriving the relative position of an object along the horizontal axis. Defective horizontal mapping can account for the other components of Gerstmann's syndrome because they all share a common dependency on relative horizontal positioning.

Aged↗
PubMed1982

Calculation disturbances in adults with focal hemispheric damage.

It has been suggested that hemisphere-damaged patients with calculation disorders can be subdivided into 3 groups: agraphia or alexia for numbers, spatial dyscalculia and anarithmetia. The purpose of the present study was to evaluate the role played by visuospatial disorders and by anarithmetia in subjects with impaired calculation abilities. Seventy-six patients with focal hemispheric lesions and 26 normal controls with demonstrated ability to read and write numbers were given a written calculation task, the Token Test, the Crosses Test, a test of Constructional Apraxia and Raven's Progressive Matrices. In the calculation task, a quantitative score represented the number of digits that were correct numerically and put in correct position. A qualitative score with emphasis on visuospatial factors was obtained by scoring each problem with the criteria used in Benton's Visual Retention Test. Analysis of the results showed that both left and right hemisphere-damaged patients performed significantly worse than controls and that patients with left posterior lesions were particularly impaired even after correction of the acalculia scores by the results of the other neuropsychological tests. These results suggest that even though different factors may contribute to calculation disorders (impairment of intelligence, visuoconstructive difficulties and above all aphasia), left posterior lesions are particularly prone to produce an impairment in calculating abilities which is partially independent from the above disorders.

Aphasia↗
PubMed1987

Aphasia arising from purely deep lesions.

In this study we investigated the relationship between deep-seated lesions of the left hemisphere and the aphasic disturbances. Thirty-seven vascular patients with CT-scan, language and neuropsychological examination carried out between 15 and 60 days post-onset were studied. Presence and frequency of apraxic disorders and acalculia are also reported. A consistent relationship between the site of the lesion and the pattern of aphasic disturbances could not be established. The most striking finding was a dissociation between oral and written expression, the latter being disproportionately impaired in a number of subjects.

Aged↗
PubMed2000

Language and calculation within the parietal lobe: a combined cognitive, anatomical and fMRI study.

We report the case of a patient (ATH) who suffered from aphasia, deep dyslexia, and acalculia, following a lesion in her left perisylvian area. She showed a severe impairment in all tasks involving numbers in a verbal format, such as reading aloud, writing to dictation, or responding verbally to questions of numerical knowledge. In contrast, her ability to manipulate non-verbal representations of numbers, i.e., Arabic numerals and quantities, was comparatively well preserved, as evidenced for instance in number comparison or number bisection tasks. This dissociated impairment of verbal and non-verbal numerical abilities entailed a differential impairment of the four arithmetic operations. ATH performed much better with subtraction and addition, that can be solved on the basis of quantity manipulation, than with multiplication and division problems, that are commonly solved by retrieving stored verbal sequences. The brain lesion affected the classical language areas, but spared a subset of the left inferior parietal lobule that was active during calculation tasks, as demonstrated with functional MRI. Finally, the relative preservation of subtraction versus multiplication may be related to the fact that subtraction activated the intact right parietal lobe, while multiplication activated predominantly left-sided areas.

Aphasia↗
PubMed2003

Approximate quantities and exact number words: dissociable systems.

Numerical abilities are thought to rest on the integration of two distinct systems, a verbal system of number words and a non-symbolic representation of approximate quantities. This view has lead to the classification of acalculias into two broad categories depending on whether the deficit affects the verbal or the quantity system. Here, we test the association of deficits predicted by this theory, and particularly the presence or absence of impairments in non-symbolic quantity processing. We describe two acalculic patients, one with a focal lesion of the left parietal lobe and Gerstmann's syndrome and another with semantic dementia with predominantly left temporal hypometabolism. As predicted by a quantity deficit, the first patient was more impaired in subtraction than in multiplication, showed a severe slowness in approximation, and exhibited associated impairments in subitizing and numerical comparison tasks, both with Arabic digits and with arrays of dots. As predicted by a verbal deficit, the second patient was more impaired in multiplication than in subtraction, had intact approximation abilities, and showed preserved processing of non-symbolic numerosities.

Aged↗
PubMed2000

Developmental Gerstmann's syndrome: a distinct clinical entity of learning disabilities.

The symptom complex of finger anomia, right-left disorientation, dysgraphia, and dyscalculia constitutes Gerstmann's syndrome. It is mostly described in adults and is caused by acquired lesions of the dominant parietal lobe. It is infrequently described in children with learning disabilities and has been designated developmental Gerstmann's syndrome. Developmental Gerstmann's syndrome goes unnoticed if not specifically sought by clinicians. A detailed evaluation will reveal subtle neurologic deficits, behavioral problems, and neuropsychologic and specific speech and language abnormalities. Ten such patients are reported; six of the children demonstrated improvement with intensive speech training. Early identification and intervention is therefore crucial, and even more important in cultures in which students are required to be biliterate or triliterate, further increasing the constraints on writing. A selective writing, reading, or calculation abnormality in the presence of normal oral communication triggers several interesting possibilities for the brain mechanisms behind normal language processing. Similarly, the association of acalculia with finger anomia and agraphia with right-left disorientation may have specific implications in the neuropsychologic processing of the evolution of calculation and writing. A theoretical possibility of oral and written language processing from the observation of the language behavior of these children is also described.

Adolescent↗