Background: Clinical diagnosis of multiple system atrophy (MSA) relays on signs and symptoms that are often difficult to identify particularly at early stage. Indeed neuropathological studies have demonstrated that Parkinson variant of MSA (MSA-P) is the first cause of misdiagnosis in a cohort of patients presenting with parkinsonian features. But accurate diagnosis of these disorders is important for deciding on treatment, appropriate advice and prognosis since atypical parkinsonian disorders are characterized by poor response to dopaminergic treatment and more rapid disease progression. Therefore, we conducted this study to investigate difference of perfusion Single Photon Emission Computed Tomography (SPECT) in patients with the early phase of MSA-P using SPM program. Methods: We recruited consecutively 21 patients with MSA-P and 48 age-matched healthy controls. All subjects underwent Tc-99m HMPAO perfusion SPECT and this perfusion images were analyzed. Results: For MSA-P, only hypoperfusion was seen in the middle frontal gyrus of left frontal lobe, superior frontal gyrus of right frontal lobe, precentral gyrus of left frontal lobe, middle frontal gyrus of right frontal lobe and precentral gyrus of right frontal lobe with respect to healthy subjects. Conclusions: We cautiously assume that perfusion SPECT may offer significant advantages compared to other imaging techniques in the assessment of neuronal degeneration in MSA-P and may help the clinician in the diagnostic characterization of patients presenting with atypical parkinsonism. Received: May 16, 2014 Revision received: May 28, 2014 Accepted: May 28, 2014