Multiple system atrophy with early cognitive deficits: A case report

Abstract

We present the case of a patient with multiple system atrophy with predominant cerebellar ataxia (MSA-C) in the early stage of the disease, who was assessed using a comprehensive neuropsychological test battery. Many studies have found cognitive deficits in MSA patients assessed after 2–3 years, but not in the first stages of the disease. The aim of this paper is to stress the importance of a complete neuropsychological assessment, even at the initial stage of the disease, when instrumental examinations are not able to show cortical involvement and daily life activities have not been affected. The neuropsychological tests examined general cognition, verbal and visual memory, visuospatial and constructional ability, language, executive function, depression and functional autonomy. Results showed cognitive deficits in executive functions, above all in the control and inhibition of automatic response, planning and reasoning abilities, memory and visuoconstructional functions. However, these problems did not affect the patient’s autonomy in everyday life. MRI scan showed the involvement of the cerebellum and the fibers of the pons and raphe, with normal cerebral ventricles and sulci. The cognitive deficits in our patient could be explained by a disruption in cerebrocerebellum connections between the frontal areas and the cerebellar structures. These results show that a more comprehensive cognitive evaluation is necessary to detect early the onset of neuropsychological deficits, also in order to begin in time adequate rehabilitation programs.