Nina A. Fragassi

Unawareness of dyskinesias in Parkinson's and Huntington's diseases.

Abstract We performed a clinical study to evaluate the unawareness of dyskinesias in patients affected by Parkinsons disease (PD) and Huntingtons disease (HD). Thirteen PD patients with levodopa-induced dyskinesias and 9 HD patients were enrolled. Patients were asked to evaluate the presence of dyskinesias while performing specific motor tasks. The Abnormal Involuntary Movement Scale (AIMS) and Goetz dyskinesia rating scale were administered to determine the severity of dyskinesias. The Unified Parkinsons disease rating scale (UPDRS) and Unified Huntingtons Disease Rating Scale (UHDRS) were used in PD and HD patients, respectively. In PD we found a significant negative relationship between unawareness score at hand pronation-supination and AIMS score for upper limbs. In HD we found a significant positive relationship between total unawareness score and disease duration. In PD the unawareness seems to be inversely related with severity of dyskinesias, while in HD it is directly related to disease duration and severity.

Visual associative agnosia and optic aphasia. A single case study and a review of the syndromes.

The case is presented of a patient who showed visual naming disturbances caused by a left occipital infarction. His performance on tests of visual naming, of recognition not requiring a verbal response, and of verbal-visual matching demonstrated a wide range of qualitatively different errors, including complete inability to recognize the object, access to partial semantic knowledge, and mere name finding difficulty. On the basis of the present case and of a review of the recent literature, the clinical distinction between visual associative agnosia and optic aphasia and the relation of these disorders with the anatomical site of lesion are discussed.

Spatial cognition in children. I. Development of drawing-related (visuospatial and constructional) abilities in preschool and early school years

The present study aimed to investigate the acquisition of visuospatial and graphomotor capacities during the pre-school and early schooling years in order to follow the normal development of drawing-related abilities and spatial cognition. Eighty children aged 3-5 years, divided in four subgroups each different for a 6-month period, and 80 children aged 8-9 years were administered a neuropsychological battery for visuospatial and visuoconstructional analysis. The battery explored five cognitive domains: visual scanning, visuospatial perceptual and representational abilities, visuomotor control and graphomotor skills. Results showed that the total scores significantly improved in each group of children with respect to the previous one, but the pattern of skill acquisition was not homogeneous. We observed a gradient from explorative and visuomotor to perceptive, representational and graphomotor abilities. Explorative and visuomotor abilities were almost mature at a time when visuoperceptual capacities began to develop. On the contrary, at that time we found very low performances at representational and constructional tasks. Our findings could suggest that constructional abilities need both perceptual and representational competences to develop properly.

Relationships between Constructional and Visuospatial Abilities in Normal Subjects and in Focal Brain-damaged Patients

ABSTRACT 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 abstract geometrical figures; moreover, RBD, but not LBD, achieved scores significantly lower than healthy controls in judging line orientation and analyzing geometrical abstract 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.

Spatial cognition in children. II. Visuospatial and constructional skills in developmental reading disability

Cognitive models for developmental dyslexia are nowadays centered on the hypothesis of a specific deficit within the phonologic module of the language system. To ascertain whether defects of spatial cognition are associated with developmental reading disability, we investigated a sample of 43 school children (aged 8-9 years) found to be reading impaired during a wide screening survey for developmental dyslexia in the province of Naples, Italy. After one year all children were tested again and only 9/43 still presented reading impairment, while the remaining had achieved a variable range of spontaneous recovery. A detailed analysis was performed on all children to characterize their cognitive performances using on one hand classical conventional tests for constructional praxis, visuospatial cognition, and visuospatial memory and on the other a specific neuropsychological battery for constructional disorders. The results of our study demonstrated that children with long-lasting reading impairment exhibited normal performances on spatial cognition tasks. Moreover, one single child was found with relevant visuospatial deficits pointing to the possible existence of a visuospatial subtype for developmental dyslexia.

Transient Muteness Followed by Dysarthria in Patients with Pontomesencephalic Stroke

Dysarthria is the principal motor abnormality following vascular damage to pontine paramedian structures, owing to the involvement of corticobulbar fibres. Here we describe 2 cases of adults affected by dysarthria following transient muteness as the result of a stroke in pontomesencephalic structures. Their clinical outcome was very similar to that of young patients who have undergone surgery of the 4th ventricle. Recently the importance of pons involvement has also been underlined in these cases. This case report suggests the existence of a functional network for speech, in which the pontomesencephalon is an important station for the triggering and the efficacy of verbal production.

The selective inability to draw horizontal lines: a peculiar constructional disorder

A patient is described who was affected by degenerative dementia and who developed severe constructional apraxia. She showed a dissociation between the construction of horizontal lines (impaired) and oblique or vertical lines (spared) which has never been reported previously. A battery of tests disclosed that this phenomenon was consistent across a range of experimental conditions and that a similar dissociation was evident in perceptual and representational domains. This peculiar clinical finding suggests that mental representations of horizontal and vertical spatial relations in an egocentric coordinate system are functionally dissociated.

Posterior cortical atrophy with prominent alexia without agraphia in a Tourette syndrome

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.

Ad hoc Reviewers in 1998

Accessible online at: http://BioMedNet.com/karger 73 H. Ackermann, Bad Urach R.G.A. Ackerstaff, Nimwegen J.M. Bamford, Leeds H. Barnett, London, Ont. R. Baumgartner, Zürich J. Biller, Indianapolis, Ind. S. Blecic, Brussels P. Burckhardt, Lausanne A. Carolei, L’Aquila M. Daffertshofer, Mannheim A. Davalos, Girona S. Demchuk, Houston, Tex. G. Devuyst, Brussels H.C. Diener, Essen P. Duchosal, Lausanne K. Einhäupl, Berlin G. Ertl, Mannheim K. Fassbender, Mannheim F. Fazekas, Graz H.J. Freund, Düsseldorf M. Giroud, Dijon A. Grau, Heidelberg R. Hart, San Antonio, Tex. M. Harrison, London P. Jallon, Geneva M. Kaps, Lübeck C. Kase, Boston, Mass. P. König, Bad Salzhausen P. Langhorne, Glasgow K. Leenders, Gruningen J. Lodder, Maastricht P. Magistretti, Lausanne H. Markus, London J. Mau, Düsseldorf L. Mayfrank, Aachen S. Meairs, Mannheim P. de Merloose, Geneva W.H. Mess, Maastricht P. Moore, Los Angeles, Calif. E. Mori, Himeji T. Moulin, Besançon P. Nicod, Lausanne J. de Reuck, Gent R. Sacco, New York, N.Y. P. Sandercock, Edinburgh P. Schmiedek, Mannheim S. Schwab, Heidelberg D. Seidel, München W. Steinke, Düsseldorf M. Sturzenegger, Bern A. Uské, Lausanne J.M. Valdueza, Berlin F. Vingerhoets, Lausanne J. Wade, London C.-W. Wallesch, Magdeburg C. Weiller, Jena B. Widder, Günzburg E. Wijdicks, Rochester, Minn.

Residual reading ability in a deeply dyslexic patient

Abstract The case of a deeply dyslexic patient is reported. The patient, Italian, showed an unusual reading ability. He was unable to read nonwords aloud, but he matched nonwords to orally presented stimuli and read aloud the recognized nonwords. Lastly, after a 1-minute interval, the patient could not re-read aloud the stimuli previously recognized and read. An attempt is made to explain this residual reading ability.