Multiple system atrophy (MSA) is a neurodegenerative movement disorder clinically characterized by a combination of different degrees of parkinsonism, cerebellar ataxia, and autonomic dysfunction. MSA is rapidly progressive, leading patients… Click to show full abstract
Multiple system atrophy (MSA) is a neurodegenerative movement disorder clinically characterized by a combination of different degrees of parkinsonism, cerebellar ataxia, and autonomic dysfunction. MSA is rapidly progressive, leading patients to be wheelchair bound within 5 to 6 years of diagno-sis. Medications are often unable or insuf fi cient to alleviate motor symptoms, and they may worsen nonmotor symptoms, especially neurogenic orthostatic hypotension (nOH). 1 The combination of these factors contributes to falls and leads to restricted ambulation. The only US Food and Drug Administration – approved treatments for orthostatic hypotension are midodrine and droxidopa (the latter being only avail-able in the United States and Japan). Other medications such as fl udrocortisone, albeit of fl abel, are commonly used at the expense of high risk for supine hypertension, which is associ-ated with greater mortality and morbidity in MSA. 2 Squair and colleagues have been developing an electrical epidural stimulator (EES), originally meant for patients with spinal cord injury, targeting the thoracic root entry zone. By stimulating the afferent fi bers in the posterior roots that project to the sympathetic ganglia via glutamatergic interneurons, they were able to prevent orthostatic hypotension in a patient with chronic cervical spinal cord injury. 3 Because the physiopathology of nOH in MSA is attributed to a preganglionic central defect (in contrast to patients with Parkinson ’ s disease, Lewy body dementia, pure autonomic failure, or other autonomic neuropathies), 4 this intervention could be helpful for patients with MSA.
               
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