Multi System Atrophy: Report Of Two Cases

Multiple system atrophy (MSA) is a sporadic, neurodegenerative disorder, clinically characterized by Parkinsonian, autonomic, cerebellar and pyramidal signs. We describe two patients showing different presentations of the same disease. The patient on case 1 presents features of MSA-C or olivopontocerebellar atrophy with the pontine "Cross sign" on brain MRI. The second case reports a patient presenting MSA-P or striatonigral degeneration and the brain MRI shows lenticular nucleus sign alteration. Multiple system atrophy (MSA) is a sporadic, neurodegenerative disorder, characterized by several combinations of parkinsonian, autonomic, cerebellar and pyramidal signs. 1-5. The incidence is 0.6 cases/100000/year and the prevalence ranges from 1.86 to 4.9 cases/1000001. The term MSA defines a distinct clinicopathological entity, including olivopontocerebellar atrophy, striatonigral degeneration and Shy-Drager syndrome, which have been described as different diseases for many years and still lead to terminology confusion. The actual consensus tries to define the diagnostic criterions and recommends the term MSA-P when parkinsonian features predominate, and the term MSA-C when cerebellar signs rules the clinical picture. There is some doubt if the term Shy-Drager syndrome should be substitute, as most cases of MSA patients develop autonomic dysfunction during their course. 6 Horimoto et al. 7 believe that some patients present a distinct form of MSA in which dysautonomic symptoms predominate, considering the utility to classify that form as MSA-A. Recently, neuroimaging studies, especially the MRI, have showed some alterations that, although not specific, may help the diagnostic of different forms of MSA. We report two MSA cases with different clinical features and their characteristic MRI alterations.