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Susac's syndrome: Leptomeningeal enhancement on 3D FLAIR MRI

Renate Engisch1, David S Titelbaum2, Lara Chilver-Stainer3

  • 1Institute of Diagnostic and Interventional Neuroradiology, Bern University Hospital, Bern, Switzerland renate.engisch@insel.ch.

Multiple Sclerosis (Houndmills, Basingstoke, England)
|May 22, 2016
PubMed
Abstract

Insights

Contrast-enhanced 3D FLAIR MRI identified leptomeningeal enhancement in Susac's syndrome, correlating with disease activity. This imaging technique may help diagnose and monitor Susac's syndrome.

Area of Science:

  • Neuroimaging
  • Neurology
  • Radiology

Background:

  • Contrast-enhanced (ce) fluid-attenuated inversion recovery magnetic resonance imaging (FLAIR MRI) is emerging as a tool for detecting leptomeningeal pathology in multiple sclerosis.
  • Susac's syndrome is a rare neurological disorder characterized by encephalopathy, retinopathy, and hearing loss.

Observation:

  • This study aimed to visualize leptomeningeal enhancement using three-dimensional (3D) FLAIR in a patient diagnosed with Susac's syndrome.
  • Leptomeningeal enhancement patterns were monitored over a 20-month period.

Findings:

  • Leptomeningeal enhancement on ce 3D FLAIR was observed in the Susac's syndrome case.
  • The size, number, and location of leptomeningeal enhancement fluctuated over time.
  • These imaging findings demonstrated a correlation with the patient's clinical symptom severity.
  • The observed leptomeningeal enhancement bore a striking resemblance to retinal vasculopathy.

Implications:

  • Contrast-enhanced 3D FLAIR MRI shows promise in aiding the diagnosis of Susac's syndrome.
  • This imaging modality may enhance our understanding of the underlying pathophysiology of Susac's syndrome.
  • Leptomeningeal enhancement visualized by ce 3D FLAIR could potentially serve as a biomarker for disease activity in Susac's syndrome.
Keywords:
Susac’s syndromecontrast-enhanced FLAIRleptomeningeal pathology

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