Alpha-synuclein Accumulation in a Patient with Auerbach's Plexus of Pure Autonomic Failure

Pure autonomic failure is considered to be a degenerative condition of the autonomic nervous resulting from accumulation of alpha-synuclein in autonomic ganglia [1,2]. Lack of CNS involvement distinguishes this disorder from other synucleopathies with CNS involvement like MSA, DLB and PD. However, long term follow-up of PAF patients has shown that some of these cases convert to one of these disorders most commonly MSA [3]. Also, recent data, albeit limited in nature has shown the association of REM sleep disorder in PAF patients challenging the previous notion that PAF is a disease restricted to autonomic nervous system. This data has led to the discovery of a clinical syndrome with a position in the middle of the spectrum of these disorders with PAF at one end of the spectrum and MSA, DLB and PD at the other end. We hereby present a case report of one such patient with PAF associated with RBD but without any other CNS manifestations.

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Pure autonomic failure without alpha-synuclein pathology: an evolving understanding of a heterogeneous disease

Clinical Autonomic Research ◽

10.1007/s10286-017-0410-1 ◽

2017 ◽

Vol 27 (2) ◽

pp. 67-68 ◽

Cited By ~ 5

Author(s):

Elizabeth A. Coon ◽

Phillip A. Low

Keyword(s):

Autonomic Failure ◽

Alpha Synuclein ◽

Pure Autonomic Failure

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Abstract P381: Understanding the Symptoms of Neurogenic Orthostatic Hypotension: Results From a Survey of Patients and Caregivers

Hypertension ◽

10.1161/hyp.70.suppl_1.p381 ◽

2017 ◽

Vol 70 (suppl_1) ◽

Author(s):

Lawrence A Hewitt ◽

Charles H Adler ◽

Daniel O Claassen ◽

Christopher H Gibbons ◽

Satish R Raj

Keyword(s):

Multiple System Atrophy ◽

Parkinson Disease ◽

Orthostatic Hypotension ◽

Autonomic Failure ◽

Alpha Synuclein ◽

Pure Autonomic Failure ◽

Motor Symptoms ◽

Neurogenic Orthostatic Hypotension ◽

The Past ◽

Increased Risk

Objective: To understand the burden and impact of symptoms of neurogenic orthostatic hypotension (nOH) on patients Background: nOH and its symptoms such as dizziness/lightheadedness are common in patients with Parkinson disease (PD) and other forms of autonomic dysfunction. Methods: An author-designed, US-based survey was conducted by Harris Poll. Eligible participants were aged ≥18 years with PD, multiple system atrophy (MSA), or pure autonomic failure and ≥1 of the following: orthostatic hypotension, nOH, low BP, OH/nOH symptoms, or were caregivers of eligible patients. Results: Most patients (90%) had a diagnosis of PD, and most caregivers (88%) cared for a patient with PD (Table 1) . Patients (34%) and caregivers (49%) reported experiencing nOH symptoms before PD or MSA motor symptoms and >40% indicated that nOH symptoms were more troublesome than motor manifestations of PD or MSA. Less than a quarter (22%) of respondents suggested symptoms were most severe in the morning; more (30%) reported a consistent severity throughout the day. Patients (40%) and caregivers (63%) reported trouble managing symptoms during the day. In the past 12 months, a fall due to nOH symptoms was reported by 57% of patients and 80% of caregivers. Conclusions: These findings suggest that nOH symptoms may predate the onset of motor symptoms in neurodegenerative conditions linked to alpha-synuclein pathology. Many respondents report nOH symptoms are the same severity through the day. Patients with nOH may have trouble managing symptoms and note an increased risk for falls.

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