Background/Purpose: To describe the evolution of neurologic symptoms and neuroradiologic findings in patients with ECDS and PRS.

Methods: The medical records database at St. Louis Children’s Hospital was searched to identify patients diagnosed with “linear scleroderma” who had been evaluated by Rheumatology, Dermatology or Neurology between 1999 and 2015.  Each chart was initially reviewed to identify children who had been diagnosed with ECDS or PRS.  The resulting charts were then reviewed to characterize skin manifestations, identify associated neurologic symptoms and evaluate for neuroradiologic abnormalities.

Results:

15 patients with ECDS or PRS were identified. Mean age of cutaneous lesion onset was 7.5 years (0-16 years). 14 patients had brain MRI performed, with serial brain MRI available in 7 patients (50%) over an average of 3 years (1-4 years). Of the 14 patients, 6 had abnormal MRI findings which were ipsilateral to the cutaneous lesion in 5 patients. The most common findings were T2/FLAIR white matter hyperintensities (83%). Brain lesions remained stable in 4 patients (80%), while 1 patient had progression in the setting of non-compliance with mycophenolate mofetil, which restabilized with compliance. All patients with abnormal imaging had neurologic symptoms, including migraines (67%), developmental delay (50%), seizures (33%) and facial palsy (17%).

Conclusion: Unilateral and commonly ipsilateral T2/FLAIR white matter hyperintensities were the most common neuroradiologic abnormalities identified. Lesions remained stable or improved over time with treatment. All patients with abnormal imaging had neurologic symptoms. Our results emphasize that patients with ECDS or PRS would benefit from serial neuroimaging, neurocognitive testing and ongoing neurologic follow-up.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/neurologic-findings-and-serial-neuroimaging-in-patients-with-linear-scleroderma-en-coup-de-sabre-ecds-and-parry-romberg-syndrome-prs/