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    Slowly progressive pure dysgraphia with late apraxia of speech: a further variant of the focal cerebral degeneration.

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    We report a longitudinal neuropsychological investigation of a patient with slowly progressive pure dysgraphia. Cognitive analysis of writing errors suggested a selective impairment of the graphemic buffer. After about seven years, the patient developed an apraxia of speech. No other linguistic or generalized cognitive impairment occurred subsequently, so that, twelve years after the beginning of the disease, the patient showed complete independence in daily life and still remained professionally active. Functional neuroimaging revealed hypoperfusion confined to left fronto-temporal lobe. This well-recognizable syndrome does not fit any of the cases described previously in the literature. This report therefore, adds another variant to heterogeneous clinical spectrum of focal neurodegenerative disorders, further suggesting the opportunity of their distinction from pathological processes leading to dementia

    Perception of emotions on happy/sad chimeric faces in Alzheimer disease: relationship with cognitive functions

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    Aims: To investigate the ability to recognize facial emotions in patients with Alzheimer disease (AD). Happiness and sadness, fundamental emotions that are easily recognized across cultures, were evaluated. Results were further analyzed in relation to demographic, clinical, and neuropsychologic characteristics. Methods: A test exploring recognition of facial emotions using chimeric faces based on Jaynes’ happy and sad faces was administered to 71 patients with probable AD. Results: The ability to identify positive and negative facial emotions was largely preserved in AD subjects. Impaired recognition of facial emotions was found in 27%. Poor test performance was associated with low scores on constructional praxis and nonverbal memory tasks. Conclusions: Decoding of emotional facial expressions seems to be differently affected in relation to patients’ neuropsychologic profile, as poor test performance closely correlated with nonverbal cognitive impairment. This emotion-discrimination disorder likely characterizes the behavior of a subset of AD patients with predominant right-hemisphere dysfunction. A preserved ability to process the emotional features of faces may have an important role in the management of demented patients, and suggests the use of nonverbal communication as an integrative/alternative system. The simple test used in our study may be a useful clinical tool to explore emotional behavior in demented subjects

    Modularity of music: evidence from a case of pure amnesia.

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    A case of pure amusia in a 20 year old left handed non-professional musician is reported. The patient showed an impairment of music abilities in the presence of normal processing of speech and environmental sounds. Furthermore, whereas recognition and production of melodic sequences were grossly disturbed, both the recognition and production of rhythm patterns were preserved. This selective breakdown pattern was produced by a focal lesion in the left superior temporal gyrus. This case thus suggests that not only linguistic and musical skills, but also melodic and rhythmic processing are independent of each other. This functional dissociation in the musical domain supports the hypothesis that music components have a modular organisation. Furthermore, there is the suggestion that amusia may be produced by a lesion located strictly in one hemisphere and that the superior temporal gyrus plays a crucial part in melodic processing
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