Pure autonomic failure is a neurodegenerative disorder. It typically presents in adults in their 60s, 70s, but as early as their 50s or 40s. Clinically, it’s characterized by orthostatic hypotension. When we check people’s blood pressures laying down for some time, and then after they’ve been standing for at least three minutes, we see a clear and sustained drop in their blood pressure of at least 20 millimeters of mercury...
Pure autonomic failure is a neurodegenerative disorder. It typically presents in adults in their 60s, 70s, but as early as their 50s or 40s. Clinically, it’s characterized by orthostatic hypotension. When we check people’s blood pressures laying down for some time, and then after they’ve been standing for at least three minutes, we see a clear and sustained drop in their blood pressure of at least 20 millimeters of mercury. Neuropathologically, it’s an alpha-synucleinopathy, just like in Parkinson’s disease, Lewy body dementia, and multiple system atrophy. And this is relevant because one out of three patients with pure autonomic failure will develop Parkinson’s disease, Lewy body dementia, or multiple system atrophy during their lifetime. Patients will usually report feeling dizzy or lightheaded when they first stand up, if they’ve been standing for some time, but also after doing physical activities. Sometimes they may report feeling a sensation of pressure or pain in the back of their necks, in their shoulders, usually described as a cold hunger pain. And some patients may even have some cognitive symptoms or fluctuations when their blood pressure goes down. So even though these patients are initially identified by their primary care physicians or cardiologists, they may be referred to look for autonomic neuropathies, to look for causes of postural dizziness, or for cognitive complaints. Some more informed physicians or patients may go straight to movement disorders to get evaluated for movement abnormalities.
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