Abstract Details

Title Iron accumulation correlates with disease severity in patients with Multiple System Atrophy

Topic Movement Disorders

Presentation(s) P15 - Poster Session 15 (5:30 PM-6:30 PM)

Poster/Presentation
Number 5-002

Background Iron accumulation in the lentiform nucleus (LFN), substantia nigra (SN), and dentate nucleus (DN) is elevated in Multiple Systems Atrophy (MSA). In this study, we assessed non-invasively iron accumulation in MSA, Parkinson disease (PD), and age matched healthy controls (HC), to evaluate the relationship between iron deposition and the severity of clinical symptoms in MSA

Objective Iron accumulation correlates with disease severity in patients with Multiple System Atrophy

Design/Methods All participants completed a neurologic examination and underwent 3T brain MRI. In MSA patients, the Unified Multiple System Atrophy Rating Scale (UMSARS) and Natural History and Neuroprotection in Parkinson Plus Syndromes (NNIPPS) rating scales were applied. Quantitative susceptibility maps (QSM) were calculated, and the median susceptibility values for the LFN (globus pallidum externa, interna, and putamen), SN and DN were obtained as measures of iron concentration. General linear models including age as covariate were used to compare the results between groups, and to evaluate the correlations between iron accumulation and clinical scores of disease severity (UMSARS and NNIPPS).

Results 17 MSA (Age=65±10; UMSARS=36±15), 17 PD (Age=63±6; UPDRS-III (Off)=27±10) and 17 HC participants (Age=66±7) were assessed. In comparison to PD, increased iron concentration in MSA patients was noted in the LFN (p=0.029) and DN (p=0.022). Compared to HC, MSA patients had greater iron concentration in the SN (p=0.032) and DN (p=0.002). In MSA, greater iron concentration in the SN and globus pallidum externa (GPe) positively correlated with severity as measured by UMSARS (SN: R²=0.46, p=0.031; GPe: R²=0.35, p=0.021) and NNIPPS (SN:R²=0.42, p=0.071; GPe:R²=0.34, p=0.025).

Conclusions Advanced quantitative MRI methods demonstrated pathological iron accumulation in MSA patients that relate to clinical severity of patients. These data support the use of QSM as a biomarker of disease severity in MSA.

Authors/Disclosures
David A. Stamler, MD (Alterity Therapeutics) PRESENTER	Dr. Stamler has received personal compensation for serving as an employee of Alterity Therapeutics. Dr. Stamler has stock in Alterity Therapeutics.
Daniel O. Claassen, MD, FAAN (Vanderbilt University Medical Center)	Dr. Claassen has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Teva Neuroscience. Dr. Claassen has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Spark . The institution of Dr. Claassen has received personal compensation in the range of $50,000-$99,999 for serving as a Consultant for Alterity. Dr. Claassen has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Teva Neuroscience. Dr. Claassen has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for HD Insights. The institution of Dr. Claassen has received research support from NIH. The institution of Dr. Claassen has received research support from CHDI. The institution of Dr. Claassen has received research support from HDSA. The institution of Dr. Claassen has received research support from Department of Defense. The institution of Dr. Claassen has received research support from Griffin Family Foundation. The institution of Dr. Claassen has received research support from Neurocrine. The institution of Dr. Claassen has received research support from Vaccinex. The institution of Dr. Claassen has received research support from AbbVie. The institution of Dr. Claassen has received research support from CHDI. The institution of Dr. Claassen has received research support from Genentech/ Roche. The institution of Dr. Claassen has received research support from Prilenia. The institution of Dr. Claassen has received research support from Neurocrine/ HSG.
Paula Trujillo Diaz	No disclosure on file
Jessie Iregui	Miss Iregui has nothing to disclose.
Amy Wynn, NP (Vanderbilt University Medical Center)	Ms. Wynn has nothing to disclose.
	No disclosure on file
James Silverman (Adamas Pharmaceuticals)	James Silverman has nothing to disclose.
	No disclosure on file
Hakmook Kang	No disclosure on file