Cerebellar ataxia in a family with recurrent epidermolysis bullosa dystrophica.
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Biomedical subjects
Publications and source records attributed to S F Cappa.
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The performances on language and verbal memory tests of 26 right-hemisphere damaged subjects (RHD) have been compared with normal controls and 11 non-aphasic left-hemisphere damaged subjects. While phonological and syntactic performance was unimpaired, a small subgroup of RHD patients, with CT scan evidence of subcortical lesion, was mildly impaired in naming tasks. The performance of RHD patients on free recall was characterized by a reduced recency effect. The implications of these findings for theories about the participation of the right hemisphere in linguistic functions are discussed.
Forty one moderately impaired patients with clinically confirmed multiple sclerosis (MS) and a relapsing-remitting course were submitted to a neuropsychological battery and magnetic resonance imaging (MRI) to correlate the neuropsychological performances with the degree of cerebral demyelination. The neuropsychological results were indicative of a very mild overall impairment. The patients were subdivided into two groups (extensive periventricular demyelination or discrete lesions on MRI) and the results of neuropsychological tests compared. Patients with extensive periventricular demyelination had an inferior performance on concept formation, non-verbal reasoning and verbal memory tests.
The effects of congenital deficiency of gonadal hormones on verbal and spatial performance and on the establishment of hemispheric asymmetries were investigated in a group of patients with idiopathic hypogonadotropic hypogonadism. The patients showed a left hemispheric advantage for verbal material and were mildly impaired, mainly on tasks involving a short-term memory load, in comparison with a matched control group. These results do not indicate a specific role of gonadal hormones on the establishment of hemispheric asymmetries. Lack of exposure during brain development results in a decrease in memory functions which is not specific for verbal or spatial material.
Cortical regional cerebral perfusion was assessed by N, N, N1-trimethyl-N1-(2)-hydroxy-3-methyl-5-(I-123) iodobenzyl-1, 3-propanediamine 2 HCl I-123 (HIPDM) and single photon emission computerised tomography (SPECT) in six aphasic and two neglect patients with unilateral subcortical vascular lesions. Assessments were carried out both in the acute phase and after a period ranging from 1 to 6 months after stroke onset. In all patients an almost complete spontaneous recovery occurred and was associated with a significant improvement of cortical perfusion. A relationship between severity of aphasia and degree of cortical hypoperfusion was found, in both the acute and the follow up assessments, in the aphasic subgroup.
Jargonagraphia (i.e., fluent, unintelligible writing with abundant paragraphias and preserved mechanics of writing) is considered to be an infrequent symptom of Wernicke's aphasia. Six cases fulfilling the definition of jargonagraphia were found in a retrospective series of 1076 consecutive aphasics and the neuropyschological and clinical correlates of this phenomenon were analysed. Jargonagraphia was not qualitatively uniform, and its appearance seems to be conditioned by the interaction of an increased "impulse to write" with disrupted language mechanisms.
Five right-handed patients suffering from a recent CT-assessed left posterior thalamic hemorrhage are reported. In all patients a standard language examination performed within one month after stroke onset did not reveal aphasic disturbances. While the present series argues for the role of intrathalamic lesion localization, a review of the literature suggests that lesion site per se does not always account for the presence or absence of aphasia in the individual case. The importance of reduced blood flow and metabolism in cortical areas which appear undamaged on CT scan is briefly discussed.
A case of CT-diagnosed brain-stem haematoma associated with signs of dorsal medullary involvement is reported. The patient recovered completely within 1 month.
We describe a 31-year-old man who has been living in a 'locked-in' condition for the past 12 years. Extensive testing, using both the patient's residual eye movements and a writing apparatus, showed preserved cognitive abilities in spite of the long-standing de-efferentation. The emotional consequences of this condition are discussed in relation with the results of personality testing.
Two clinical-CT scan studies were undertaken in order to investigate the frequency and the characteristics of aphasia following strictly subcortical left hemisphere lesions. In Study 1, all patients whose CT scan, carried out within a given period, showed subcortical lesions in both hemispheres, were evaluated for presence and type of aphasia; conversely, in Study 2 the records of all patients referred to our Aphasia Unit for language evaluation within a given period, were examined for presence of subcortical lesions on the CT scan. In Study 1 aphasia was found in two out of six patients. In Study 2 stabilized, strictly subcortical lesions of the left hemisphere were found in six out of 250 patients referred to the Aphasia Unit; 4 out of 6 were aphasics. Closer scrutiny of the type of language disorder in the aphasics from both studies (N = 6) suggests the possibility of differentiating two types of defect: an atypical non fluent aphasia, sometimes associated with anterior capsular-putaminal lesions, and a mild fluent aphasia, sometimes associated with posterior capsular-putaminal lesions.
CT scan studies of lesion localization in aphasic patients have in general confirmed the traditional locus of damage within the left hemisphere for the major syndromes. Some interesting exceptions have come to light, such as the possible occurrence of global aphasia with a partial lesion (anterior or posterior) of the language zone. The study of correlations in vivo has underlined the importance of considering time since onset in the evaluation of the clinical picture. Description of atypical aphasias associated with subcortical lesions and correlation of lesion site with specific, partial aspects of linguistic impairment are two areas where CT scan studies are increasing our understanding of the language machinery in the brain.
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In a series of 14 thalamic hemorrhage documented by Computerized Axial Tomography (CT) scans, aphasia was present in seven out of eight patients with left lesions while it was absent in the six patients with right lesions. In three cases where detailed language testing was performed, aphasia was characterized by reduction of spontaneous speech with semantic paraphasias, preserved repetition and partially defective auditory verbal comprehension. The language disturbance was persistent in two patients, while it recovered spontaneously within four weeks in one patient. The clinical picture in these patients is similar to the classical "transcortical" aphasias, which are usually due to damage of the marginal language areas. It is suggested that the left thalamus contributes to the semantic level of verbal behavior, which is possibly subserved by these areas.
In order to investigate neuropsychological differences in patients with mild AD, we carried out a pilot study on 28 patients with a clinical diagnosis of mild dementia (CDR: 0.5-1) using an extensive neuropsychological battery, in comparison with 28 normal controls. The results of a cluster analysis, applied on the neuropsychological variables, showed the existence of at least two main subgroups of patients. Cluster 1 patients had a mean age of 61.1 years and showed a greater impairment on measures of language, abstract reasoning and verbal fluency; cluster 2 patients, with a mean age of 72.0, had more severe impairment in memory function. These preliminary results may suggest the existence of different subtypes of AD characterized by selective neuropsychological deterioration in the early stages of the disease.
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