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Spatial span (Corsi's block-tapping test) and verbal span (Wechsler's Digits Forward test) were measured in 651 normal subjects and in three groups of extrapyramidal patients (Progressive supranuclear palsy, Parkinson, and Huntington's Chorea). Analysis showed Huntington's Chorea patients scored lower on both tests than did controls and other groups.

A 73-year-old man presented a progressive neurologic syndrome with impairment of multiple cranial nerves, ataxia and limb weakness. Neuroradiological evaluation showed multiple supra and infratentorial lesions, which completely disappeared after steroid treatment on two occasions. These neuroradiological modifications were closely correlated with clinical picture and CSF findings. Post-mortem examination showed multifocal primary CNS immunoblastic large cell lymphoma. Immunohistochemistry showed that the tumor was of B-cell origin.

A case is presented of Dejerine-Sottas disease in a 12-year-old boy in which clinical signs made diagnosis of Friedreich's ataxia seem plausible. Based on marked slowing of motor conduction velocity, the sural nerve biopsy findings of a hypertrophic neuropathy with hypo- and demyelination of the nerve fibres, as well as the clinical history, the diagnosis of Dejerine-Sottas disease was made. ABR examination suggested involvement of brain stem at the roots and/or nuclei of the eighth cranial nerve, without involvement of higher structures.

Summary Boys (n = 80) and girls (n = 80) aged 9 to 10 completed a spatial-span test and a spatial serial-learning task. Corsi's block-tapping test was used for both. Sex differences were found on each: males performed better in both cases. The better performance of males on the spatial serial-learning task was independent of their superiority in the spatial-span test.

We tested 125 normal subjects and 24 right and 22 left focal brain-damaged patients (RBD and LBD) on the Rey figure copying test and on a battery of perceptual and representational visuospatial tasks, in search of relationships between constructional and visuospatial abilities. Selected RBD and LBD were not affected by severe aphasia, unilateral spatial neglect or general intellectual defects. Both RBD and LBD showed defective performances on the constructional task with respect to normal subjects. As regards visuospatial tasks, both patient groups scored lower than normal subjects in judging angle width and mentally assembling geometrical figures; moreover, RBD, but not LBD, achieved scores significantly lower than healthy controls in judging line orientation and analyzing geometrical figures. Post-hoc comparisons did not reveal any significant differences between RBD and LBD. Multiple regression analysis showed that visuospatial abilities correlate with accuracy in copying geometrical drawings in normal subjects and in RBD, but not in LBD. From a theoretical perspective, these findings support the idea that visual perceptual and representational abilities do play a role in constructional skills.

Background In recent years several attempts have been made to distinguish frontotemporal dementia (FTD) from Alzheimer's disease (AD) on neuropsychological grounds; in particular, it has been suggested that FTD patients show spared spatial abilities with respect to AD patients. Objective We aimed at verifying whether patients with the frontal variant of frontotemporal dementia (fv-FTD) and AD patients perform differently on visuospatial and constructional tasks. Methods We assessed a wide range of visuospatial abilities and provided a qualitative analysis of constructional performances in 14 fv-FTD patients and 11 AD patients, matched for general cognitive abilities. Results The two groups of patients achieved similar scores on two copying tasks, presented similar drawing procedures in copying Rey complex figure and made a similar quantitative and qualitative pattern of errors in copying simple geometrical drawings. Moreover, no significant difference was found between fv-FTD and AD patients on a specific battery for visuospatial abilities. Conclusions Our data and a review of the literature suggest that basic visuospatial and constructional skills cannot be taken as a reliable diagnostic criterion for distinguishing fv-FTD and AD at a mild to moderate disease stage and that the clinical belief of spared spatial abilities in fv-FTD has to be referred to the lack of topographic disorientation in comparison to AD. Copyright © 2002 John Wiley & Sons, Ltd.

We report for the first time a patient with childhood-onset Tourette’s syndrome (TS) who developed alexia without agraphia, acalculia, visual agnosia for objects and faces, and preserved mnesic functions in older age. Neuroimaging studies demonstrated temporo-parieto-occipital cortical atrophy and fronto-temporo-parieto-occipital hypometabolism, both more prominent on the left side. This case fulfils the diagnostic criteria of posterior cortical atrophy (PCA). The possible link between TS and PCA is discussed.

The aim of the present paper was to investigate articulatory loop in patients with pure motor speech disturbances. A homogeneous group of 18 patients affected by ataxic dysarthria and without neuropsychological and neuroimaging evidence of cortical damage was selected. The study comprised four experiments which assessed: (i) immediate and delayed recall of word sequences with and without articulatory suppression; (ii) phonological similarity and word length effects on immediate recall; (iii) articulation rate; and (iv) verbal and spatial span. In agreement with previous studies on patients affected by different types of dysarthria, articulatory loop was found functional. However, patients had a slower articulation rate, and it was argued that this finding can represent a “capacity limitation” of articulatory rehearsal, contributing to the patients' slight verbal short-term memory defect.

We assessed neuropsychological performances of 22 patients affected by Autosomal Dominant Cerebellar Ataxia type 1. All subjects completed a comprehensive battery of standardized tests requiring a verbal response, without time constraints. In order to verify the hypothesis that disease severity is the major factor in determining the cognitive status in this syndrome, patients were divided into three groups according to the severity of the clinical picture, as evaluated by the Inherited Ataxias Progression Scale (IAPS). Statistical analysis of the three groups' raw scores showed a significant decrement in patients with more severe clinical pictures on verbal short-term memory tasks. A similar trend, but not significant, was seen for general intelligence tests and verbal learning tasks. The decrement of verbal short-term memory could be related to motor speech problems. On the other hand, the decline of cognitive abilities over the course of the Autosomal Dominant Cerebellar Ataxia type 1 was not homogeneous enough to ensure statistically reliable trends. Therefore, this cross-sectional study suggests that the progression of the disease is a necessary factor in determining cognitive decline, but it is not sufficient. Other disease-related factors (age at onset, genotypic variety) could play a critical role: among these, the size of the expanded CAG repeats is significantly related to a decline of verbal intelligence and short-term memory in SCA2 patients.

The present study aimed at controlling two variables that may affect the visuospatial short-term memory of Alzheimer patients: visuospatial coding efficiency and response modality. Thirty patients affected by Alzheimer-type dementia with relatively spared visuo-perceptual functions were tested under three conditions, all of which employed the same kind of stimuli (visuospatial patterns). At all memory tasks, patients achieved scores significantly lower than those of 30 age and education-matched normal subjects. Patients did not benefit from longer presentation time, nor did their performance improve with pointing response modality, although they performed perceptual pattern recognition as well as did controls. These data confirm that visuospatial immediate memory capacity is reduced in dementia.

This paper reports the clinical features of 2 patients affected by mixed transcortical aphasia. Both of them had extensive damage of the left hemisphere. Cerebral blood flow measurement showed a marked decrease in left-hemisphere flow. In particular, speech areas were found to be morphologically and functionally damaged. The present findings, together with a critical review of the literature, served as a starting point for a discussion of linguistic features, diagnostic criteria and classical neuroanatomical interpretation of mixed transcortical aphasia. The possible contribution of the right hemisphere in determining this aphasic syndrome is suggested.

Spatial span (Corsi's block-tapping test) and verbal spans for digits (Wechsler Digits Forward Test) and for words were measured in 30 normal subjects and in 51 demented patients, divided into two groups (mildly demented and severely demented) according to selective clinical and neuropsychological criteria. Statistical analysis showed significant differences among the three groups for spatial span. By contrast, controls' and mildly demented patients' performances on both verbal spans were not significantly different. These findings are discussed in the light of theory about working memory.