Multiple system atrophy (MSA) is an adult-onset, sporadic, idiopathic, a rarely seen neurodegenerative disease. MSA is clinically characterized by a variable combination of autonomic dysfunction, parkinsonism, cerebellar ataxia and pyramidal symptoms. One of the symptoms of autonomic dysfunction is orthostatic hypotension. Orthostatic hypotension can worsen due to sepsis and severe hypotension requiring intravenous vasopressor therapy can develop. If these patients are dependent on vasopressors, long-term treatment options must be considered. One of the treatment options is pyridostigmine. Pyridostigmine is a cholinesterase inhibitor recently started to be used in the treatment of orthostatic hypotension. In this case report, we presented a patient admitted to our intensive care unit with the diagnosis of urosepsis in whom vasopressor dependent hypotension due to MSA developed. Although the findings of sepsis were retreat in the follow-up, the need for vasopressor continued. Pyridostigmine was used to correct hypotension associated with autonomic dysfunction in this patient resistant to weaning from vasopressors. Increasing of the pyridostigmine dose to 180 mg/day resulted in stabilization of the mean arterial pressure and the vasopressor therapy was gradually reduced and discontinued.

Keywords: Autonomic dysfunction, intensive care unit, multiple system atrophy