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The accumulation of α-synuclein (αSN) is the neuropathologic hallmark of neurodegenerative diseases including dementia with Lewy bodies (DLB), Parkinson disease (PD), and rarer conditions such as multiple system atrophy (MSA) and pure autonomic failure (PAF).1 Symptoms depend on the distribution of αSN, ranging from cognitive dysfunction in DLB with diffuse neocortical localization of αSN to PAF with deposition of αSN in the brainstem.2 The latter condition is characterized by orthostatic hypotension and is associated with other symptoms of autonomic failure, including syncope, urinary or erectile dysfunction, constipation, dysphagia, dysarthria, and apnea syndrome.3
Footnotes
Author contributions: H.J. Gilhuis: corresponding author, principal author, designed study, conceptualized study, interpreted data in study, drafted manuscript. A. Panwar: coauthor, conceptualized study, analyzed data in study, interpreted data in study. S.G. Van Duinen: coauthor, analyzed data in study, interpreted data in study, revised manuscript. F.J. De Jong: coauthor, designed study, conceptualized study, interpreted data in study, drafted manuscript.
Study funding: No targeted funding reported.
Disclosure: The authors report no disclosures relevant to the manuscript. Go to Neurology.org for full disclosures.
Editor's Note: Dr. Panwar's surrogate, Dr. de Bruijn, signed Dr. Panwar's required forms due to the author's extended illness.
- Received February 25, 2014.
- Accepted in final form July 16, 2014.
- © 2014 American Academy of Neurology
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