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Abstract Details

MRI Patterns and Neurologic Manifestations in Adult En Coup de Sabre: Insights from a Retrospective Cohort Study
Autoimmune Neurology
P5 - Poster Session 5 (11:45 AM-12:45 PM)
2-003
To examine MRI ordering patterns, imaging findings, and their relationship to neurologic symptoms in an adult en coup de sabre (ECDS) cohort.
ECDS is a rare subtype of linear morphea that primarily affects the frontoparietal forehead and scalp. This subtype is associated with neurological symptoms or brain lesions on neuroimaging. Since ECDS is rare and mainly presents in childhood, literature is largely limited to pediatric reports.
We used the patient data registry to identify patients aged ≥18 years diagnosed with ECDS at two large academic centers. We reviewed MRIs to identify abnormalities, assess their potential relationship to ECDS, and determine whether they could be associated with neurologic symptoms. We used Fisher’s exact tests to determine association with MRI findings and presence of neurologic symptoms. 

We identified 94 patients (mean age 47.1 years, 87.2% female). Neurologic symptoms were reported by 53 patients (56.4%): most frequently headache (86.8%), seizures (13.2%), and sensory loss (13.2%). 64 patients (68.1%) underwent MRI, with intracranial findings reported and images available for review in 33 cases. Of these, 18 (54.5%) were deemed unlikely related to ECDS while 15 (45.5%) were possibly/likely related. Of these 15 patients, 86.7% had T2 white matter hyperintensities (WMH), 60.0% with microhemorrhages, 13.3% with parenchymal enhancement, and 6.7% with meningeal enhancement—all ipsilateral to the ECDS lesion. There was no significant association between presence of WMH and/or microhemorrhages with ECDS-related neurologic symptoms. Moreover, 8/15 patients had first MRI performed as baseline in absence of symptoms.
Asymmetric WMH, microhemorrhages, and enhancement ipsilateral to ECDS can be seen in this patient population; however, WMH and microhemorrhages were not explanatory of neurologic symptoms nor were they statistically associated with them. Guidelines on when to order imaging could improve diagnostic yield, reduce unnecessary costs, and prevent overutilization.
Authors/Disclosures
Prashanth Rajarajan, MD, PhD (Brigham and Women's Hospital)
PRESENTER 		Dr. Rajarajan has nothing to disclose.
Maria C. Vazquez-Machado, BS Miss Vazquez-Machado has nothing to disclose.
Maria C. Vazquez-Machado, BS Miss Vazquez-Machado has nothing to disclose.
Arjun Mahajan Mr. Mahajan has nothing to disclose.
Shamik Bhattacharyya, MD, FAAN (Brigham and Women's Hospital) Dr. Bhattacharyya has received personal compensation in the range of $500-$4,999 for serving as a Consultant for NeuroLambda. Dr. Bhattacharyya has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion Pharmaceuticals. Dr. Bhattacharyya has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Amgen. Dr. Bhattacharyya has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for TG Therapeutics. Dr. Bhattacharyya has received personal compensation in the range of $5,000-$9,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Continuum. Dr. Bhattacharyya has received personal compensation in the range of $10,000-$49,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Wiley. Dr. Bhattacharyya has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for Merck. The institution of Dr. Bhattacharyya has received research support from Alexion Pharmaceuticals. The institution of Dr. Bhattacharyya has received research support from National Institute of Health. The institution of Dr. Bhattacharyya has received research support from UCB. The institution of Dr. Bhattacharyya has received research support from Genentech. Dr. Bhattacharyya has received publishing royalties from a publication relating to health care. Dr. Bhattacharyya has received publishing royalties from a publication relating to health care.
Avery J. LaChance, MD, MPH Mrs. LaChance has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Pfizer. Mrs. LaChance has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Odyssey. Mrs. LaChance has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Guidepoint. Mrs. LaChance has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Johnson and Johnson. Mrs. LaChance has received personal compensation in the range of $500-$4,999 for serving as an Expert Witness for HBS Atlanta.