Abstract Details

Title Spinal Cord Cervical and Thoracic Gray Matter Atrophy – an emerging imaging surrogate for Amyotrophic Lateral Sclerosis

Topic Neuromuscular and Clinical Neurophysiology (EMG)

Presentation(s) Neuromuscular and Clinical Neurophysiology (EMG) Posters (7:00 AM-5:00 PM)

Poster/Presentation
Number 045

Objective To evaluate cervical and thoracic spinal cord (SC) gray matter (GM) atrophy and its association with disability in patients with Amyotrophic Lateral Sclerosis (ALS).

Background ALS is a fatal neurodegenerative disease with a heterogeneous clinical presentation and a variable disease course. There is an urgent need for valid imaging surrogate markers for disease course monitoring, prognosis, and therapeutic response monitoring in ALS. The novel rAMIRA (Radially Sampled Averaged Magnetization Inversion Recovery Acquisitions) method enables high-resolution MR- imaging of cervical and thoracic SC GM and white matter (WM) in clinically feasible short acquisition times.

Design/Methods Using rAMIRA, we prospectively investigated 30 patients diagnosed with definitive, probable or possible ALS according to El Escorial criteria (mean age 63.05 years, SD 11.63, 11 women) and 30 age- and sex matched healthy controls (mean age 64.33 years, SD 12.09, 11 women). Total cross-sectional and SC GM areas were determined at the intervertebral disc levels C2/C3, C3/C4, C4/C5, C5/C6, T9/T10 and Tmax (level of the lumbar enlargement) using a semi-automated approach (JIM7, www.xinapse.com). In each patient, we assessed the revised ALS Functional Rating Scale (ALSFRS-R). Associations between SC imaging metrics and ALSFRS-R were assessed using multivariate regression analysis.

Results We found significant and predominant SC GM atrophy at the levels C2/C3 (relative reduction (rr)=11.7%, p<0.0001), C3/C4 (rr=15.7%, p<0.0001), C4/C5 (rr=13.2%, p=0.0010), C5/C6 (rr=14.7%, p<0.0001) and Tmax (rr=21.9%, p<0.0001) in patients compared to controls. In multivariate regression models covarying for age, sex and disease duration, SC GM areas at C3/4 and at Tmax were significantly associated with ALSFRS-R (p=0.0380, p=0.0197), explaining 53% and 54% of ALSFRS-R variance, respectively.

Conclusions SC GM atrophy evaluated by AMIRA imaging is a promising imaging surrogate candidate in ALS that correlates with disability. Ongoing longitudinal studies investigate the relation of SC GM metrics with symptom evolution and disease progression.

Authors/Disclosures
Maria Janina Wendebourg, MD (University Hospital Switzerland) PRESENTER	Dr. Wendebourg has nothing to disclose.
Matthias Weigel, PhD	The institution of Matthias Weigel, PhD has received research support from Biogen. Matthias Weigel, PhD has received intellectual property interests from a discovery or technology relating to health care.
	No disclosure on file
	No disclosure on file
	No disclosure on file
Laura Sander, MD (Universitätsspital Basel)	Dr. Sander has nothing to disclose.
Eva M. Kesenheimer, MD (University Hospital Basel)	Dr. Kesenheimer has nothing to disclose.
	No disclosure on file
	No disclosure on file
Cristina Granziera, MD, PhD (Basel University Hospital)	The institution of Prof. Granziera has received research support from Novartis. The institution of Prof. Granziera has received research support from Hoffmann La Roche. The institution of Prof. Granziera has received research support from Genzyme Sanofi. The institution of Prof. Granziera has received research support from Biogen.
Ludwig Kappos, MD, F�鶹��ýӳ�� (RC2NB, University Hospital Basel)	Dr. Kappos has nothing to disclose.
Markus Weber, MD (Kantonsspital St. Gallen)	Dr. Weber has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Biogen Idec. Dr. Weber has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Mitsubishi Tanabe.
Michael Sinnreich, MD, PhD (Basel University Hospital)	Prof. Sinnreich has nothing to disclose.
Kathi Schweikert	No disclosure on file
	No disclosure on file
	No disclosure on file

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