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    Lesions Associated with Motor Speech

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    Apperceptive and Associative Forms of Phonagnosia

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    Purpose of review: Pronagnosia is a rare acquired or developmental pathological condition that consists of a selective difficulty to recognize familiar people by their voices. It can be distinguished into two different categories: apperceptive phonagnosia, which denotes a purely perceptual form of voice recognition disorder; and associative phonagnosia, in which patients have no perceptual defects, but cannot evaluate if the voice of a known person is or not familiar. The neural substrate of these two forms of voice recognition is still controversial, but it could concern different components of the core temporal voice areas and of extratemporal voice processing areas. This article reviews recent research on the neuropsychological and anatomo-clinical aspects of this condition. Recent findings: Data obtained in group studies or single case reports of phonagnosic patients suggest that apperceptive phonagnosia might be due to disruption of the core temporal voice areas, bilaterally located in the posterior parts of the superior temporal gyrus, whereas associative phonagnosia might result from impaired access to structures where voice representations are stored, due to a disconnection of these areas from structures of the voice extended system. Although these results must be confirmed by further investigations, they represent an important step toward understanding the nature and neural substrate of apperceptive and associative forms of phonagnosia

    Cross-modal recognition disorders for persons and other unique entities in a patient with right fronto-temporal degeneration

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    We describe a patient (CD), with a right fronto-temporal degeneration, who showed massive defects in the recognition of familiar people and severe behavioural disorders. CD scored in the normal range on tests of episodic memory, attention and visual-spatial abilities, and obtained mildly abnormal scores on naming and executive functions tests. CD was then studied and matched with a left brain-damaged patient (IG), comparable to her as for education, naming score and general cognitive impairment, on several tasks, exploring recognition of familiar people and of other instances of 'unique entities'. On specific tasks of face recognition, she obtained normal results on perceptual tests, but highly pathological scores on mnesic-associative tasks. A similar defect was found when identification was based on the person's voice or on a verbal definition. The cross-modal nature of CD's disorder was confirmed by results of a test, in which person-specific information available from photographs and from names was directly compared. In order to evaluate if CD's recognition disorder: (a) was the consequence of a general semantic defect, (b) was specific for people, or (c) also concerned other instances of 'unique entities', we matched her capacity to name and recognize the pictures of items belonging to various categories of knowledge with those concerning famous monuments and famous people. CD identified items belonging to semantic categories much better than those considered as 'unique entities' and, within the latter, obtained slightly better results with famous monuments than with famous persons. MRI showed a bilateral atrophy of the antero-inferior parts of the temporal lobes, more pronounced in the right side. About 2 years after the onset of the symptomatology, CD became untestable, due to the development of a severe motor neuron disease

    Determinants of cognitive impairment in elderly myasthenia gravis patients

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    The relationship between myasthenia gravis (MG) and cognitive dysfunction has been a matter of debate because of the possible association between peripheral and central nervous system (CNS) cholinergic dysfunction. The aim of this study was to evaluate cognitive function in a series of elderly MG patients in comparison to matched controls. In all, 100 consecutive MG patients aged over 60 years and 31 matched control subjects underwent an extensive neuropsychological test battery to explore multiple cognitive domains. There were no differences in cognitive performances between patients and controls. Severe MG was associated with impaired attention, constructional praxis, and frontal control. Logistic regression analysis showed that advanced age, diabetes, and thyroid dysfunction were independently associated with cognitive impairment. This study does not support the hypothesis of CNS cholinergic involvement in MG. The impairments of attention, memory, and control tasks in MG are related to general visual motor slowness and to the concomitant presence of other diseases

    Post-stroke depression: Main phenomenological clusters and their relationships with clinical measures

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    Objectives: To investigate the principal psychopathological dimensions of post-stroke depression (PSD) through the assessment of the factorial structure of the Post-Stroke Depression Rating Scale (PSDRS). Methods: We enrolled ninety-eight subjects with PSD, who underwent the PSDRS, MMSE and Barthel Index. Information about demographic, clinical, and neuroanatomical factors was collected. Results: The factor analysis extracted three factors accounting for 63.4% of the total variance, and identified as: 1) "Depressive and Anxious Symptoms" (DAS); 2) "Lack of Emotional Control" (LEC); 3) "Reduced Motivation" (RM). On multivariate statistics, DAS severity was predicted by previous history of mood disorders and Barthel Index; LEC severity was predicted by Barthel Index; RM severity was predicted by age. Conclusions: The PSDRS displayed a reliable factor structure that agreed with previous interpretation of PSD. In particular, core depressive symptoms seem to be related to premorbid personality and functional status, whereas apathy/anhedonia may be connected to brain aging

    Different apathy profile in behavioral variant of frontotemporal dementia and Alzheimer's disease: a preliminary investigation

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    Apathy is one of the most common behavioral symptoms of dementia; it is one of the salient features of behavioral variant of frontotemporal dementia (bvFTD) but is also very frequent in Alzheimer's disease. This preliminary investigation was aimed at assessing the type of apathy-related symptoms in a population of bvFTD and AD subjects showing comparable apathy severity. Each patient underwent a comprehensive neuropsychological assessment; behavioral changes were investigated by the neuropsychiatric inventory (NPI), using the NPI-apathy subscale to detect apathetic symptoms. At univariate analysis, bvFTD subjects showed lack of initiation (χ(2) = 4.602, p = 0.032), reduced emotional output (χ(2) = 6.493, p = 0.008), and reduced interest toward friends and family members (χ(2) = 4.898, p = 0.027), more frequently than AD subjects. BvFTD displayed higher scores than AD on NPI total score (p = 0.005) and on subscales assessing agitation (p = 0.004), disinhibition (p = 0.007) and sleep disturbances (p = 0.025); conversely, AD subjects were more impaired on memory, constructional abilities, and attention. On multivariate logistic regression, reduced emotional output was highly predictive of bvFTD (OR = 18.266; p = 0.008). Our preliminary findings support the hypothesis that apathy is a complex phenomenon, whose clinical expression is conditioned by the site of anatomical damage. Furthermore, apathy profile may help in differentiating bvFTD from AD

    Semantic Relations in a Categorical Verbal Fluency Test: An Exploratory Investigation in Mild Cognitive Impairment

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    Categorical verbal fluency tests (CFT) are commonly used to assess the integrity of semantic memory in individuals with brain damage. Persons with Dementia of the Alzheimer's Type display a reduced output on CFT, and a similar pattern has been reported in persons with amnesic Mild Cognitive Impairment (aMCI). The aims of the present study were to assess whether the semantic relations between lexical entries produced on a categorical fluency test were different between healthy persons and those with aMCI, and whether this difference was more pronounced in individuals who converted to dementia during a 3-year follow-up period. Methods: We recruited 34 individuals with aMCI and 29 matched healthy persons. During the follow-up period, 10 individuals converted to Dementia (aMCI-conv). Two measures assessing semantic relations between consecutively produced word pairs (Path length and Extended Gloss Overlap) were obtained from the Wordnet database. Results: The number of word pairs analyzed among the healthy participants (HP) and persons with aMCI were 498 (birds: 262; pieces of furniture: 236) and 395 (birds: 174; pieces of furniture: 221), respectively. Path length was lower in aMCI-conv than in HP (p = 0.035), but no differences were found between stable aMCI and HP, and between aMCI-stable and aMCI-conv. The ANOVA for lexical entries belonging to the "birds" category showed a significant effect of group (F = 5.630; p = 0.004); the post hoc analysis showed a significant difference between HP and aMCI-conv (p = 0.003). The "pieces of furniture" category was significantly affected by group (F = 4.107; p = 0.017); the post hoc test showed significant differences between aMCI-conv and healthy individuals (p = 0.049), and between aMCI-conv and stable aMCI (p = 0.001). Discussion: Individuals with aMCI who convert to dementia show a deterioration in the semantic relations between lexical entries, produced on a CFT. This phenomenon may be interpreted as a marker of a very early disruption of semantic memor
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