Long-term Cognitive Outcomes in Susac Syndrome (P8-14.003)

Objective: Analyzing the cognitive impact of aggressive immunotherapy protocol in Susac syndrome rehabilitation.
Background: Susac syndrome (SuS) is a rare autoimmune disorder mediated by the occlusion of micro-blood vessels in the brain, retina, and inner ear. Approximately 15% of cases present with the classic triad of CNS dysfunction, visual disturbances, and sensorineural hearing loss. While the literature is abundant about the severe, acute encephalopathy of SuS, not much is known about the extent of cognitive sequela in the post-era of efficient immunomodulatory treatment.
Design/Methods: The registry of the Neuroimmunology Unit at Tel Aviv Sourasky Medical Center, Israel, was browsed for patients who met the suggested criteria of SuS diagnosis (definite and probable). Ten recovering SuS patients with an average of 2.9 (SD=1.41) years post-disease onset were assessed for global cognitive functions with a battery of cognitive tests. Further information was retrieved from the medical files, including scores on a cognitive screening test administered at the time of diagnosis, initial CNS involvement severity data, and brain MRI. Results: Patients showed intact delayed memory (both verbal and non-verbal) but below-average scores on tests of executive functions (Stroop), and deficits in attention (MOXO) and copying (Rey-Osterrieth Complex Figure). Eight patients (80%) regained base occupational functionality. Results are discussed in light of the initial severity and extent of corpus callosum involvement on brain MRI.
Conclusions: Study results suggest that the main cognitive sequela of SuS involves deficits in visual attention and executive functions possibly due to Corpus Callosum involvement. Additionally, this report supports a favorable prognosis for patients with SuS who receive a fast and efficacious immunomodulatory treatment protocol suggested in 2018 by Rennebohm at el.
Disclosure: Dr. Piura has received personal compensation in the range of $500-$4,999 for serving as a Consultant for BMS. Dr. Piura has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Merck. Noa Bregman has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Medison. The institution of Noa Bregman has received research support from Ionis pharmaceuticals. The institution of Prof. Kave has received research support from Israel Science Foundation. Dr. Karni has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Neopharm. Dr. Karni has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for BMS. Dr. Karni has received personal compensation in the range of $5,000-$9,999 for serving as an Expert Witness for Israeli Court. The institution of Dr. Karni has received research support from Medison. The institution of Dr. Karni has received research support from Sanofi. The institution of Dr. Karni has received research support from Novartis. The institution of Dr. Karni has received research support from Roche. Hadar Kolb has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Novartis. Hadar Kolb has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Marck. Ifat Vigiser has nothing to disclose. Tamara Shiner has nothing to disclose. Dr. Regev has nothing to disclose.