Foreign accent syndrome (FAS) is a rare speech disorder characterised by the emergence of a new accent, perceived by listeners as foreign. FAS has usually been described following focal brain insults, such as stroke. We describe the unusual case of a woman presenting with FAS as the earliest symptom of progressive degenerative brain disease. At presentation, she showed no language or other cognitive impairment, and functional and structural brain imaging were normal. Follow-up 1 year later revealed the emergence of mild expressive language problems. Repeat functional neuroimaging showed mild hypoperfusion of the perisylvian speech area of the left hemisphere, and structural imaging showed mild left perisylvian atrophy. We interpret the case as an unusual presentation of primary progressive non-fluent aphasia. The case provides further evidence of the variable and circumscribed nature of the clinical presentation of focal cerebral degeneration.
One of the major problems that clinical neuropsychology has had in memory clinics is to apply ecological, easily administrable and sensitive tests that can make the diagnosis of dementia both precocious and reliable. Often the choice of the best neuropsychological test is hard because of a number of variables that can influence a subject's performance. In this regard, tests originally devised to investigate cognitive functions in healthy adults are not often appropriate to analyze cognitive performance in old subjects with low education because of their intrinsically complex nature. In the present paper, we present normative values for the Rey-Osterrieth Complex Figure B Test (ROCF-B) a simple test that explores constructional praxis and visuospatial memory. We collected normative data of copy, immediate and delayed recall of the ROCF-B in a group of 346 normal Italian subjects above 40 years. A multiple regression analysis was performed to evaluate the potential effect of age, sex, and education on the three tasks administered to the subjects. Age and education had a significant effect on copying, immediate recall, and delayed recall as well as on the rate of forgetting. Correction grids and equivalent scores with cut-off values relative to each task are available. The availability of normative values can make the ROCF-B a valid instrument to assess non-verbal memory in adults and in the elderly for whom the commonly used ROCF-A is too demanding.
The presence of the applause sign in cortical dementia does not confirm the specificity of the applause sign for parkinsonian disorders. The applause sign should be interpreted as a sign of frontal lobe dysfunction rather than a form of apraxia, and can likely be detected in any kind of disease which involves frontal lobe structures to some extent.
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