Multiple System Atrophy Masquerading as Drug-Resistant Orthostatic Hypotension.

A 54-year-old male presented with a two-year history of recurrent syncope. Although he was treated with droxidopa and amezinium metilsulfate, his syncope was drug-resistant. Blood tests, cardiac evaluation and brain computed tomography (CT) were unremarkable. As his Schellong test was positive, orthostatic hypotension and syncope were the tentative diagnoses. Upon evaluating the patient neurologically while carefully avoiding syncope induction, mild cerebellar ataxia was observed. However, a brain magnetic resonance imaging (MRI) scan revealed a possible "cross sign" in the pons. Subsequent dopamine transporter single-photon emission computed tomography (DAT-SPECT) showed reduced uptake in the basal ganglia. Based on these findings, a clinical diagnosis of probable multiple system atrophy of the cerebellar type (MSA-C) was made, and treatment with taltirelin hydrate was initiated. This case underscores the importance of considering multiple system atrophy (MSA) in patients with persistent orthostatic hypotension and syncope, even before more obvious neurodegenerative symptoms emerge.

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Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work.