好色先生

好色先生

Explore the latest content from across our publications
Join The AAN

Log In

Forgot Password?
Create New Account

Loading... please wait

Abstract Details

Predicting Phenoconversion in Pure Autonomic Failure
Neuromuscular and Clinical Neurophysiology (EMG)
S19 - Autonomic Disorders (4:18 PM-4:30 PM)
005

To determine predicting factors and frequency of phenoconversion from pure autonomic failure (PAF) into a synucleinopathy with motor or cognitive involvement of multiple system atrophy (MSA), Parkinson disease (PD), or dementia with Lewy bodies (DLB).

Pure autonomic failure (PAF) is an α-synucleinopathy characterized by orthostatic hypotension while some patients with PAF later develop motor or cognitive symptoms.  Identifying PAF patients at risk for phenoconverting to MSA or Lewy body disorders has prognostic implications and represents an opportunity for early intervention with future disease-modifying therapies.
We performed a retrospective review of all PAF patients from 2001-2011 evaluated at Mayo Clinic, Rochester. Clinical follow-up and patient telephone calls were used to assess for development of symptoms and diagnosis of MSA, PD or DLB. Clinical and laboratory variables were extracted with factors predictive of evolution assessed using group comparison, odds ratio, and logistical regression. 
Among 275 patients with PAF at presentation, 67 (24%) phenoconverted to a synucleinopathy with motor or cognitive involvement; 34 met criteria for MSA while 33 met criteria for PD or DLB. Age of onset was younger in MSA phenoconverters. Clinical features at presentation influenced phenoconversion: severe bladder symptoms were more common in MSA phenoconverters; subtle motor signs were more frequent in MSA and PD/DLB phenoconverters. MSA phenoconverters were more likely to have higher supine norepinephrine levels and preganglionic pattern of anhidrosis.  Presentation variables predicting MSA phenoconversion included: subtle motor signs, supine norepinephrine levels, severe bladder symptoms and dream enactment behavior. Presentation variables predictive of PD/DLB phenoconversion included: subtle motor signs, dream enactment behavior and constipation.    

Our findings suggest that at least a quarter of PAF patients phenoconvert to MSA, PD or DLB. Presentation features determine patients at risk for evolution with specific patterns indicative of phenoconversion to MSA versus PD/DLB.

Authors/Disclosures
Elizabeth A. Coon, MD, FAAN (Mayo Clinic)
PRESENTER 		Dr. Coon has nothing to disclose.
Jayawant N. Mandrekar, PhD Dr. Mandrekar has nothing to disclose.
Sarah E. Berini, MD (Mayo Clinic) Dr. Berini has nothing to disclose.
Eduardo E. Benarroch, MD, FAAN (Mayo Clinic) Dr. Benarroch has nothing to disclose.
Paola Sandroni, MD, PhD, FAAN (Mayo Clinic) Dr. Sandroni has nothing to disclose.
Phillip A. Low, MD, FAAN (Mayo Clinic) Dr. Low has nothing to disclose.
Wolfgang Singer, MD, FAAN (Mayo Clinic) Dr. Singer has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Amneal. Dr. Singer has received personal compensation in the range of $500-$4,999 for serving as a Consultant for UniQure. Dr. Singer has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Biohaven. Dr. Singer has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Theravance. The institution of Dr. Singer has received research support from NIH. The institution of Dr. Singer has received research support from FDA. The institution of Dr. Singer has received research support from Michael J. Fox Foundation. Dr. Singer has received intellectual property interests from a discovery or technology relating to health care.