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Abstract Details

Usefulness of quantitative susceptibility mapping in ALS
Neuromuscular and Clinical Neurophysiology (EMG)
P1 - Poster Session 1 (9:00 AM-5:00 PM)
376
We designed a study to retrospectively assess the MRI/QSM correlation with MRC, FVC, El Escorial diagnosis and ALSFRS-R. 
Familial ALS is most commonly due to the C9ORF72 and SOD1 genes. Currently, we use brain MRI quantitative susceptibility mapping analysis (QSM) to examine susceptibility in the motor cortex, which is typically increased in motor neuron disease. 
We retrospectively reviewed all motor neuron disease patients seen in Neurology at Hospital for Special Surgery from 2013 through 2018 who had MRI with QSM. One neuroradiologist examined the scans and quantified relative susceptibility.
We found no distinguishing abnormalities in the brain MRIs of the genetic ALS patients reviewed. QSM of the motor cortices and corticospinal tracts showed qualitative increase in susceptibility, which is not unique to type of ALS. QSM failed to correlate with ALSFRS-R, FVC, or sum of MRC. There was, however, a significant difference between normal (35.2 ppb) and definite ALS by El Escorial Criteria (43.8 ppb). 
MRI/QSM may not be useful in distinguishing ALS patients by severity or mutation. It may be useful to distinguish ALS from mimic syndromes. The amount of protein aggregation may not be directly related to axonal loss, or it may affect certain neurons/tracts more than others. 
Authors/Disclosures
Robin Warner, DO (Robin Warner Neurology, PLLC)
PRESENTER
Dr. Warner has nothing to disclose.