Abstract

FRISON, Thirzá Baptista et al. Neuropsychological profile of an adult with Chorea-Acanthocytosis: a case study. Cuad. neuropsicol. [online]. 2012, vol.6, n.2, pp. 69-84. ISSN 0718-4123.  http://dx.doi.org/10.7714/cnps/6.2.301.

The purpose of the present study is to describe the neuropsychological profile of an adult afflicted with Choreoacanthocytosis, a subfrontocortical dementia marked by neuropsychiatric symptoms, cognitive decline, choreic oro-bucco-facial movements, tongue lacerations, stereotyped behavior, personality alterations, epileptic seizures, basal ganglia degeneration and presence of acanthocytes in the peripheral blood smear. Neuropsychological assessment indicated deficits in concentrated attention, verbal memory, visual memory, planning and cognitive flexibility in this condition. In Theory of Mind tasks, the patient shown difficulties to infer actions, intentions and internal states of others. Along the assessment process, were found deficits of Theory of Mind, narrative discourse, indirect speech acts interpretation and prevalence of repetition in the acquisition of new information not discussed in previous studies. It is expected this paper to offer new insight for the better understanding of this affliction, as well as for the development of neuropsychological interventions for it.

Keywords : Chorea-acanthocytosis; Neuroacanthocytosis; cognition; neuropsychological assessment; Theory of Mind; case study.

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