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Abstract Details

Cerebellar and parkinsonian phenotypes of multiple system atrophy: differences and similarities at baseline from the Natural History Study of the Synucleinopathies
Neuromuscular and Clinical Neurophysiology (EMG)
P5 - Poster Session 5 (8:00 AM-9:00 AM)
1-004

To compare the baseline characteristics of patients with the cerebellar (MSA-C) and parkinsonian (MSA-P) variants of multiple system atrophy enrolled in the Natural History Study of the Synucleinopathies (NHSS).

Whether the diagnostic delay and the severity of autonomic symptoms are different in MSA-P and MSA-C remains controversial.

Patients with probable or possible MSA were enrolled in the NHSS, a global multicenter prospective study. Demographic, clinical, quality of life, sleep and autonomic parameters were assessed at baseline, including the Unified MSA Rating Scale, the University of Pennsylvania Smell Identification Test (UPSIT) and the Montreal Cognitive Assessment (MoCA).

Three hundred and forty-two MSA patients (173 with MSA-C and 169 with MSA-P) were enrolled. Sex distribution, and age at enrollment and at presentation were similar. MSA-C patients had shorter diagnostic delay (MSA-C 3.8±3.8; MSA-P 4.3±4.9 years; p=0.048), and disease duration (MSA-C: 4.7±3.7; MSA-P 5.6±5.1 years; p<0.01). UMSARS scores, RBD prevalence and MoCA were similar. The burden of autonomic symptoms was higher in MSA-P (COMPASS-31, MSA-C 33±16; MSA-P 39±15; p=0.015) and patients with MSA-P required urinary catherization more frequently (MSA-C 32%; MSA-P 51%; p<0.001) and reported worse quality of life (MSA-C 64±26; MSA-P 73±66; p=0.015). Autonomic testing showed similar prevalence of abnormal Valsalva (95% in both), orthostatic hypotension (MSA-C 74%; MSA-P 79%), symptomatic orthostatic hypotension (MSA-C 32%: MSA-P 42%), supine hypertension (MSA-C 57%; MSA-P 55%) and blood pressure drop upon tilt (MSA-C: -41±27; MSA-P: -45±31 mmHg). Norepinephrine did not increase in the supine and head-up tilt positions in neither MSA-C (486±500 pg/dl and 466±270 pg/dl) nor MSA-P (360±284 pg/dl and 434±290 pg/dl).

Patients with MSA-P had higher symptomatic burden of autonomic dysfunction, predominantly urinary symptoms, with a higher need for bladder catheterization, lower quality of life and lengthier diagnostic delay than patients with MSA-C. These findings have implications for clinical trials.
Authors/Disclosures
Patricio Millar Vernetti, MD (NYU Dysautonomia Center)
PRESENTER 		The institution of Dr. Millar Vernetti has received research support from NIH.
Jose-Alberto Palma, MD, PhD, FÂ鶹´«Ã½Ó³»­ (New York University Grossman School of Medicine) Dr. Palma has received personal compensation for serving as an employee of Eli Lilly. The institution of Dr. Palma has received research support from National Institutes of Health. Dr. Palma has received publishing royalties from a publication relating to health care.
Alessandra Fanciulli, MD, PhD (Medical University of Innsbruck) Alessandra Fanciulli, MD, PhD has received personal compensation in the range of $0-$499 for serving as a Consultant for Theravance Biopharma. Alessandra Fanciulli, MD, PhD has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Sanofi. Alessandra Fanciulli, MD, PhD has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Theravance Biopharma. Alessandra Fanciulli, MD, PhD has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Healthware. Alessandra Fanciulli, MD, PhD has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Abbvie. Alessandra Fanciulli, MD, PhD has received personal compensation in the range of $0-$499 for serving on a Speakers Bureau for Stopp-HSP. Alessandra Fanciulli, MD, PhD has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Otto Loewi Society. Alessandra Fanciulli, MD, PhD has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Bial . Alessandra Fanciulli, MD, PhD has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Elsevier. The institution of Alessandra Fanciulli, MD, PhD has received research support from Parkinson Fond. The institution of Alessandra Fanciulli, MD, PhD has received research support from Tuba Foundation. The institution of Alessandra Fanciulli, MD, PhD has received research support from MSA Coalition. The institution of Alessandra Fanciulli, MD, PhD has received research support from Austrian Exchange Programme. The institution of Alessandra Fanciulli, MD, PhD has received research support from Austrian Science Fund. Alessandra Fanciulli, MD, PhD has received publishing royalties from a publication relating to health care.
No disclosure on file
Wolfgang Singer, MD, FÂ鶹´«Ã½Ó³»­ (Mayo Clinic) Dr. Singer has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Biohaven. The institution of Dr. Singer has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Lundbeck. Dr. Singer has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Ionis. Dr. Singer has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Yoda. Dr. Singer has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Theravance. Dr. Singer has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Ferrer. 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.
Phillip A. Low, MD, FÂ鶹´«Ã½Ó³»­ (Mayo Clinic) Dr. Low has nothing to disclose.
No disclosure on file
No disclosure on file
No disclosure on file
Amanda C. Peltier, MD, MS (Vanderbilt University) Dr. Peltier has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Alnylam. Dr. Peltier has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for astrazeneca. Dr. Peltier has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for CSL Behring. Dr. Peltier has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Csl Behring. Dr. Peltier has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for argenx. Dr. Peltier has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for alnylam. Dr. Peltier has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for astrazeneca. Dr. Peltier has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for Goldman Ismail. The institution of Dr. Peltier has received research support from NIH. The institution of Dr. Peltier has received research support from CSL Behring.
No disclosure on file
Maria J. Marti No disclosure on file
No disclosure on file
Marcelo Merello No disclosure on file
David S. Goldstein, MD, PhD (National Institutes of Health) Dr. Goldstein has received publishing royalties from a publication relating to health care.
Roy L. Freeman, MD (Beth Israel Deaconess Hosp) Dr. Freeman has received personal compensation in the range of $50,000-$99,999 for serving as a Consultant for Cutaneous Diagnostic Life Sciences. Dr. Freeman has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Vertex. Dr. Freeman has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Theravance. Dr. Freeman has received personal compensation in the range of $10,000-$49,999 for serving as an officer or member of the Board of Directors for Inhibikase. Dr. Freeman has received personal compensation in the range of $10,000-$49,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Elsevier. The institution of Dr. Freeman has received research support from NIH. The institution of Dr. Freeman has received research support from Theravance. The institution of Dr. Freeman has received research support from Biohaven. The institution of Dr. Freeman has received research support from Lundbeck. Dr. Freeman has received research support from Regeneron.
Christopher H. Gibbons, MD, FÂ鶹´«Ã½Ó³»­ (Beth Israel Deaconess Medical Center) Dr. Gibbons has received personal compensation for serving as an employee of CND Life Sciences. Dr. Gibbons has or had stock in CND Life Sciences.Dr. Gibbons has received publishing royalties from a publication relating to health care.
Steven Vernino, MD, PhD, FÂ鶹´«Ã½Ó³»­ (UT Southwestern Medical Center) Dr. Vernino has received personal compensation in the range of $500-$4,999 for serving as a Consultant for lundbeck. Dr. Vernino has received personal compensation in the range of $500-$4,999 for serving as a Consultant for CSL Behring. Dr. Vernino has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Regeneron. Dr. Vernino has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for argenx. Dr. Vernino has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alterity. Dr. Vernino has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Autonomic Neuroscience (Elsevier). The institution of Dr. Vernino has received research support from Takeda. The institution of Dr. Vernino has received research support from NIH/NHLBI. The institution of Dr. Vernino has received research support from Lundbeck. The institution of Dr. Vernino has received research support from Regeneron.
No disclosure on file
Gregor K. Wenning, MD No disclosure on file
Horacio C. Kaufmann, MD, FÂ鶹´«Ã½Ó³»­ (NYU Langone Health - NYU Dysautonomia Center) Dr. Kaufmann has received personal compensation in the range of $50,000-$99,999 for serving as a Consultant for Theravance. Dr. Kaufmann has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Teva Pharmaceuticals. Dr. Kaufmann has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Curasen Therapeutics. Dr. Kaufmann has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Lundbeck. Dr. Kaufmann has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for AskBio. Dr. Kaufmann has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for BioArctic. Dr. Kaufmann has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Sanofi. Dr. Kaufmann has received personal compensation in the range of $5,000-$9,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Spinger. The institution of Dr. Kaufmann has received research support from Biogen. The institution of Dr. Kaufmann has received research support from Vaxxinity. Dr. Kaufmann has received publishing royalties from a publication relating to health care.