Click Here to Download this Blog Post – Orthostatic Hypotension: Part 5 Of 6
By Dr. Nicholas L. DePace, M..D., F.A.C.C – Cardiologist specializing in autonomic dysfunction, Ehlers-Danlos syndrome and POTS.
This is Part 5 of a 6 Part Series about Orthostatic-Hypotension
Importantly, autonomic failure is often associated with movement disorders, such as Parkinson’s disease and dementia with Lewy bodies disease.
An autoimmune disorder, which can come on quickly or sub-acutely, is an autonomic failure due to autoimmune autonomic ganglionopathy, and one can diagnose this by clinical history, and oftentimes there are autoantibodies present in the blood, for which we can test.
In fact, treatments that reduce the antibody burden can improve this autoimmune disorder and lessen orthostatic drops in blood pressure in these patients.
Primary autonomic failure is a cause of autonomic neuropathy due to a neurodegenerative disorder in the brain and peripheral nerves.
Multiple system atrophy (Shy-Drager syndrome), and pure autonomic failure (Bradbury-Eggleston syndrome), and dementia with Lewy bodies are causes of autonomic neuropathy that often involve the central nervous system and are considered primary autonomic failure.
Secondary autonomic failure can occur, as mentioned, with diabetes, Parkinson’s, amyloid disease, and autoimmune disorders, rarely with Guillain-Barre syndrome, a genetic disorder known as familial dysautonomia (Riley-Day syndrome), hereditary sensory autonomic neuropathies, vitamin deficiencies such as Vitamin B12, toxic neuropathies, drug-induced neuropathy, such as from chemotherapy and infectious neuropathies.
The latter eight are rather uncommon, and normally, with peripheral neuropathies causing secondary autonomic failure, we find diabetes the most common and occasionally find amyloidosis and then Parkinson’s disease.
The primary autonomic failure ones, as mentioned above, however, are more serious and more difficult to treat and often have a very poor long-term prognosis compared to the secondary causes of autonomic failure.
This is true with multiple system atrophy, pure autonomic failure, and dementia with Lewy bodies.
With pure autonomic failure, an individual can go into an accelerated phase and develop more profound central nervous changes consistent with multiple system atrophy.
Initially, pure autonomic failure can behave as a strictly peripheral autonomic abnormality, but with worsening and transitioning stage can go into a central form.
More will be discussed about this later.
This is an extremely difficult disorder to treat. It is incurable, and results are usually poor in treatment despite aggressive pharmacological and lifestyle attempts. Multiple system atrophy has a more rapid decline, as does dementia with Lewy bodies.
About the Author
Nicholas L. DePace, MD, FACC is a board-certified cardiologist and Medical Director of Franklin Cardiovascular Associates. A graduate of the Mount Sinai School of Medicine, Dr. DePace has decades of clinical, academic, and research experience and has held faculty appointments as a Clinical Professor of Medicine, becoming one of the youngest full professors in Philadelphia at the time of his appointment.
Dr. DePace specializes in the diagnosis and treatment of autonomic nervous system dysfunction (dysautonomia), including POTS, autonomic dysfunction associated with Ehlers-Danlos syndrome (EDS), chronic fatigue, and anxiety-like conditions that are frequently misdiagnosed. He is nationally recognized for his work on parasympathetic and sympathetic (P&S) nervous system imbalance, a core mechanism underlying many complex chronic disorders.
In addition to treating patients from across the United States, Dr. DePace is a prolific clinical researcher and author of multiple nationally distributed medical textbooks published by Springer and W.W. Norton, focusing on autonomic dysfunction, mitochondrial disorders, cardiovascular disease, and mind–body medicine.
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