MRI Patterns and Neurologic Manifestations in Adult En Coup de Sabre: Insights from a Retrospective Cohort Study
Prashanth Rajarajan1, Maria Vazquez-Machado2, Nikki Zangenah2, Arjun Mahajan2, Shamik Bhattacharyya3, Avery LaChance2
1Mass General Brigham, 2Dermatology, Brigham and Women's Hospital, 3Brigham and Women's Hospital
Objective:
To examine MRI ordering patterns, imaging findings, and their relationship to neurologic symptoms in an adult en coup de sabre (ECDS) cohort.
Background:
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.
Design/Methods:
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.
Results:
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.
Conclusions:
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.
Disclaimer: Abstracts were not reviewed by Neurology® and do not reflect the views of Neurology® editors or staff.