Abstract Details

Title Cutaneous Alpha-Synuclein, Dopamine Transporter Imaging, and Cardiac MIBG Scan in Multiple System Atrophy

Topic Movement Disorders

Presentation(s) P1 - Poster Session 1 (9:00 AM-5:00 PM)

Poster/Presentation
Number 277

Objective To describe clinical, dopamine transporter imaging (DAT), [(123)I]metaiodobenzylguanidine (MIBG) cardiac scintigraphy and phosphorylated alpha-synuclein (P-SYN) skin biopsy findings in patients diagnosed with Multiple System Atrophy (MSA.

Background MSA may be confused with Parkinson’s disease (PD) or Progressive Supranuclear Palsy (PSP). DAT imaging can be abnormal in all 3. Cardiac MIBG is often normal in MSA, and abnormal in PD and PSP. Only PD and MSA are P-SYN(+).

Design/Methods Retrospective review of possible (Pos-MSA) or probable MSA (Prob-MSA) patients who underwent skin biopsy for P-SYN.

Results 26 MSA patients were identified; 13 Pos-MSA and 13 Prob-MSA. Average age at onset was 68.5 years. DAT scan was abnormal in 10/26 (38%). 21/26 (80%) had MSA-Parkinsonism (MSA-P), whereas 5/26 (20%) had MSA-Cerebellar (MSA-C). 7/21 (33%) of MSA-P and 3/5 (60%) of MSA-C had abnormal DAT scans. Dermal P-SYN was identified in 23/26 (88%); 4/23 (17%) were MSA-C and 19/23 (83%) were MSA-P. 4/5 (80%) of MSA-C were P-SYN(+) whereas 19/21 (90%) of MSA-P were P-SYN(+). Cardiac MIBG was performed in 17 patients (3 MSA-C, 14 MSA-P) and was abnormal in 11/17 (65%): severe in 6/11(54%)(5 MSA-P, 1 MSA-C) and mild in 5/11(45%)(3 MSA-P, 2 MSA-C). Regarding the 3 P-SYN(-) patients, 1 had MSA-C and 2 had MSA-P; all 3 had abnormal DAT scans, and only 1 underwent cardiac MIBG scanning which was normal. Levodopa was tried on 21/27 patients; 0/21 had a robust response and 4/21 (19%) (all MSA-P) had a mild response. 2/4 MSA-P patients with some levodopa-responsiveness had cardiac MIBG scans; 1 was normal and 1 was markedly abnormal.

Conclusions Skin biopsy for P-SYN may be a highly sensitive test for both MSA-C and MSA-P. When the DAT scan is abnormal, cutaneous P-SYN and cardiac MIBG together may better differentiate MSA from PD, or MSA from PSP.

Authors/Disclosures
Virgilio Gerald H. Evidente, MD, FAAN (Movement Disorders Center of Arizona) PRESENTER	Dr. Evidente has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Revance. Dr. Evidente has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Abbvie. Dr. Evidente has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Supernus. Dr. Evidente has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Teva. Dr. Evidente has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Medtronic. Dr. Evidente has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Neurocrine. Dr. Evidente has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Amneal. Dr. Evidente has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Abbvie. The institution of Dr. Evidente has received research support from CND. The institution of Dr. Evidente has received research support from Aeon. The institution of Dr. Evidente has received research support from Bukwang Pharmaceuticals. The institution of Dr. Evidente has received research support from Jazz Pharmaceuticals. The institution of Dr. Evidente has received research support from Scion Neurostim. The institution of Dr. Evidente has received research support from Theravance Biopharma. Dr. Evidente has received research support from Cerevance. Dr. Evidente has received research support from Ipsen. The institution of Dr. Evidente has received research support from Vima Pharmaceuticals. The institution of Dr. Evidente has received research support from Biohaven.
Christopher H. Gibbons, MD, FAAN (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.
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.
Danica Evidente	Danica Evidente has nothing to disclose.
Todd D. Levine, MD (Honor Health)	Dr. Levine has received personal compensation for serving as an employee of CND life sciences . Dr. Levine has received personal compensation in the range of $100,000-$499,999 for serving as a Consultant for Nufactor. Dr. Levine has received personal compensation in the range of $50,000-$99,999 for serving as an Expert Witness for PNA. Dr. Levine has or had stock in CND Life Sciences.Dr. Levine has or had stock in Corinthian reference lab.

��ɫ��

��ɫ��