Abstract Details

Title T2 Relaxation Time in Friedreich Ataxia: A Prospective Study

Topic Movement Disorders

Presentation(s) P06 - (-)

Poster/Presentation
Number 087

Objective

Background BACKGROUND: In FRDA, iron accumulates inside mitochondria leading to oxidative damage and neuronal death. Clusters of iron are paramagnetic and cause inhomogeneities in a magnetic field. This results in increased decay times, thus reducing T2 relaxation times.

Design/Methods DESIGN/METHODS: Twenty patients with FRDA and 19 age-and-sex-matched controls underwent two MRI scans in a 3T scanner (Philips Achieva) one year apart. We used a T2 multi-echo sequence to calculate relaxation times using a validated software (aftervoxel). Patients were evaluated with Friedreich Ataxia Rating Scale - FARS. We used paired sudent's t-test to compare T2 between groups and pearson's coefficient to evaluate possible correlations between T2 and clinical parameters.

Results RESULTS: Mean age of patients and duration were 26.2 and 11.4 years, respectively. Mean length of (GAA)1 and (GAA)2 alleles were 1069 and 900, respectively. There was significant reduction of T2 in FRDA in the left dentate nuclei (57 ± 8.6 ms vs 53.1 ± 6.2 ms, p<0.001), but not in controls (62.1 ± 5.0 ms vs 61.5 ± 5.1 ms, p=0.37). There was no significant difference in both groups regarding left putamen and SN T2 (patients: p=0.43 and 0.66; controls: p=0.68 and p=0.25, respectively). Variation of left dentate nuclei T2 correlated with age and age of onset (r=-0.51, p=0.02 and r=-0.51, p=0.02, respectively), but not FARS (p=0.78) and duration (p=0.12).

Conclusions CONCLUSIONS: In FRDA, there is progressive reduction of T2 relaxation time in dentate nucleus, which is more severe in early onset patients. T2 relaxation time may be used as biomarker in this disease.

Authors/Disclosures
PRESENTER	No disclosure on file
	No disclosure on file
Anelyssa Cysne Frota D' Abreu, MD, PhD, MPH, FAAN (Corewell Health Outpatient Neurology)	The institution of Dr. Cysne Frota D' Abreu has received research support from U.S. NIH Institute on Aging. The institution of Dr. Cysne Frota D' Abreu has received research support from Biogen. The institution of Dr. Cysne Frota D' Abreu has received research support from American College Of Radiology. The institution of Dr. Cysne Frota D' Abreu has received research support from COGNITION THERAPEUTICS, INC.. The institution of Dr. Cysne Frota D' Abreu has received research support from Eli Lilly. The institution of Dr. Cysne Frota D' Abreu has received research support from Jansen. Dr. Cysne Frota D' Abreu has received personal compensation in the range of $10,000-$49,999 for serving as a Lecturer with Pri_med.
Fernando Dangond, MD, FAAN	Dr. Dangond has received personal compensation in the range of $1,000,000+ for serving as a Head, Global Clinical Development (employee for 12 years) with EMD Serono.
Iscia Lopes-Cendes, MD, PhD (University of Campinas - UNICAMP)	No disclosure on file
Marcondes C. Franca, Jr., MD	Dr. Franca has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Pfizer. Dr. Franca has received personal compensation in the range of $500-$4,999 for serving as a Consultant for PTC. Dr. Franca has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Janssen. Dr. Franca has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Novartis. The institution of Dr. Franca has received research support from FARA.

��ɫ��

��ɫ��