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Background: Neurologic injury is still a frequent cause of mortality, morbidity, and long-lasting disability in patients undergoing an aortic arch operation with hypothermic circulatory arrest. The aim of this analysis was to evaluate short- and long-term outcomes in neurologic and cognitive functions in this group of high-risk patients., Methods: A total of 333 patients undergoing an aortic arch operation between February 2004 and June 2010 were retrospectively reviewed. Cerebral protection was obtained with deep hypothermic circulatory arrest in 220 patients (66%) or with moderate hypothermic circulatory arrest in 113 cases (34%). Straight deep hypothermic circulatory arrest was adopted in 35 cases (11%), while the association with antegrade cerebral perfusion was adopted in 271 cases (81%) and with retrograde cerebral perfusion in 27 cases (8%). Seventy-eight patients were enrolled in a case control prospective study (mean follow-up time = 42 months) and underwent neuropsychologic evaluations; data were compared with those of a matched-control group of hypertensive patients without history of cardiac operations., Results: Forty-one out of 333 patients experienced permanent neurologic dysfunction (12%) and 83 experienced temporary neurologic dysfunctions (25%). Acute aortic dissection and deep hypothermic circulatory arrest were significant predictors of mortality and permanent neurologic dysfunction. Acute aortic dissection and hypothermic circulatory arrest duration >30 minutes were significant predictors of temporary neurologic dysfunction, while antegrade cerebral perfusion was protective on mortality. Neuropsychologic evaluations showed no significant differences between the groups. The operative group showed worse verbal and working memory (P = .003), worse semantic fluency (P = .036), higher degree of alexithymia (P = .004), and a lower quality of life (P = .007)., Conclusion: Although moderate hypothermic circulatory arrest with antegrade cerebral perfusion demonstrated a lower mortality compared with deep hypothermic arrest, neurocognitive testing demonstrated no difference between the groups. Additionally, patients undergoing an aortic arch operation demonstrated long-term cognitive deficits and psychological dysfunction when compared to a matched cohort of nonoperative patients., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Patients with cerebellar lesions present some affective and cognitive disorders, defining a peculiar pattern of cognitive impairment, so-called cerebellar cognitive affective syndrome. This pattern has been confirmed in many genotypes of spinocerebellar ataxias (SCA), a group of genetically defined pathologies characterized by the degeneration of the cerebellum and its connections. Recently, in SCA patients, some authors focused the interest on social cognition evidencing an impairment of theory of mind and basic emotion recognition by verbal material. The recognition of emotions in faces is an essential component of social cognition; therefore, we assessed this ability in SCA patients, expanding the study from the basic verbal emotions to the basic and social visual emotion recognition. We assessed facial emotion recognition using two basic and social emotion tasks in a group of SCA patients together with a complete clinical and neuropsychological evaluation. We compared results with the performance of a control group. We demonstrated a significant difference between patients and controls both in basic and social emotion recognition, although we found a specific impairment only for social emotions. The deficit was not correlated to clinical and demographic features. The cognitive and psychological profile did not explain the impairment in emotion recognition. This result supports the hypothesis that the impairment in social emotion recognition could be specifically related to a defect in the corticocerebellar network.

We administered a large battery of neuropsychological tests to an heterogeneous cohort of genetically defined spinocerebellar ataxia (SCA) patients in order to assess their cognitive profile and to compare cognitive impairment among different SCA genotypes, particularly between SCA with the classical pattern of olivo-ponto-cerebellar atrophy (SCA1 and SCA2) and those with a relatively "pure" olivo-cerebellar atrophy (SCA6 and SCA8). Our data revealed a neuropsychological picture characterized by fronto-parietal involvement with mnestic, linguistic, visuospatial, attentional, executive, and mood changes, in agreement with the cerebellar cognitive affective syndrome definition. We found a homogeneous neuropsychological profile among SCA subgroups with a prominent role of frontal dysfunction--particularly, attention, memory, and executive functions. We analyzed the possible interactions between neuropsychological pattern and clinical, demographical, and genetic variables. We found the presence of a cognitive impairment at the early stages of the disease, without visuospatial alterations, which appeared later. Age and education represented the most important demographic factors to predict the neuropsychological performance in SCA and in controls, but their effect in patients had definitely more impact. In our sample education could represent a protective factor and a marker of an enriched environment or a better developmental cognitive differentiation. We demonstrated that in our patients there was a distinct subgroup of high functional subjects and that triplet repeats modulated the effect of aging on cognition and progression of motor disability.

Cerebellar agenesis is a rare disorder. We present the neurological and neuropsychological features of a patient with partial cerebellar agenesis (TZ), together with SPECT perfusion and fMRI activation during a finger tapping task. TZ shows only mild cerebellar signs, while neuropsychological testing discloses severe deficits in many domains, in accordance with the theorized role of the cerebellum in cognition. FMRI and SPECT demonstrate an activation and a symmetrical perfusion of the cerebellar remnants, that can be related to the residual cerebellar motor function. The left frontal and parieto-temporal cortex hypoperfusion can explain the severe cognitive impairment and could be linked to the abnormal cerebellar development.

Objective: This study deals with executive functions such as abstraction, use of strategy, and response flexibility (focused attention) seen in patients with anorexia nervosa. The aim was to highlight any correlation between neuropsychological functioning and the degree of pathology for food style, body image, and physical state., Method: A modified Stroop test, Wisconsin Card Sorting test, Body Shape Questionnaire, and Eating Disorder Inventory-2 were administered to 20 anorexia nervosa restricters (AN-R) and 20 control subjects., Results: The AN-R subjects showed nonspecific attentional biases, in addition to disorders regarding abstraction and flexibility of thought, similar to those seen in patients with obsessive-compulsive disorder. The disturbance of body image showed a direct correlation to frontal alterations., Discussion: This evidence suggested that in the AN-R subjects the disturbances of body image might be linked to the alterations of abstraction and critical abilities and with an obsessive frontal functioning., (Copyright 2002 by Wiley Periodicals, Inc.)

Spinocerebellar ataxias type 2 (SCA2) is a rare genetic disorder characterised by the degeneration of the Cerebellum, its connections and many Brainstem areas. A voxel-based morphometry (VBM) analysis was performed on 12 genetically determined SCA2 patients and 31 controls, normalising the brains with two different atlases: one was created in-house with DARTEL (a diffeomorphic registration method) and the other was SUIT (an exclusive Cerebellum atlas). We administered two versions of a popular executive/planning functions test: the Tower of London, in the traditional and in a computerised version that does not require the use of hands, to correlate the regional atrophy with the tests' performances and to discover the different associations of Cerebellum's areas to cognitive dysfunctions. SCA2 showed a diffuse infratentorial atrophy with the whole Cerebellum and Brainstem affected, the overall patterns were highly overlapping between atlases with some minor differences. The DARTEL VBM also allowed detecting two sovratentorial clusters of atrophy, one in the left Inferior Parietal Lobule and the other in the Corticospinal Tracts. Additional analyses revealed a partial involvement of many White Matter tracts and of the Thalamus in the pathology. The classical Tower of London version correlated maximally with the right Lobule IV-V, when the computerised version correlated with the right Crus 1. The correlations of different versions of the test suggested a dissociation between the dysfunctions in SCA2: the Posterior Cerebellum was linked to the executive dysfunction while the Anterior Cerebellum was linked to the coordinative dysfunction. [ABSTRACT FROM AUTHOR]