Abstract Details

Title Susac Syndrome with a Unique Involvement of the Thoracic Spinal Cord

Topic Autoimmune Neurology

Presentation(s) P6 - Poster Session 6 (5:30 PM-6:30 PM)

Poster/Presentation
Number 9-007

Background Susac syndrome is a rare and frequently misdiagnosed disease of the central nervous system (CNS) commonly presenting with migraine like headaches before encephalopathy. Less than a quarter of patients present with the complete clinical triad of Susac syndrome: encephalopathy, branch retinal artery occlusions (BRAO), and hearing loss. On magnetic resonance imaging (MRI) case reports describe T2 hyperintensities in almost all areas of the CNS including the cerebrum, cerebellum, brain stem, cervical cord, and cauda equina; however, no cases of thoracic cord involvement have been documented. Similar MRI findings can appear in neuroinflammatory disorders which may lead to patients being misdiagnosed with multiple sclerosis (MS), acute disseminated encephalomyelitis (ADEM), or another neuroinflammatory entity.

Objective

Design/Methods

A woman in her late 20s presented with headaches, visual disturbances, and subacute encephalopathy. MRIs showed numerous punctate subcortical, juxtacortical, periventricular, and white matter hyperintensities with diffusion restriction, as well as enhancement of the leptomeninges surrounding the brainstem and cerebellum and two areas of signal hyperintensity in the spinal cord at the mid-thoracic level. MS and ADEM were considered most likely until she suddenly developed hearing loss.

Hearing loss, BRAO, and encephalopathy clinically confirmed Susac Syndrome. High-dose steroids were started. Her neurologic and cognitive exam began to stabilize following the fourth day of treatment. She was started on steroid sparing agents after discharge. Her vision recovered but she required a cochlear implant for hearing loss. One year later, she is stable with no reoccurrences. Repeat MRI confirmed thoracic cord involvement.

Results NA

Conclusions We report a novel finding, thoracic spinal cord involvement in Susac syndrome. This report acts to complete the radiologic picture for Susac syndrome and announce to clinicians that Susac syndrome may act on any region of the central nervous system. We hope this report helps to further define this rare syndrome and prevent misdiagnosis.

Authors/Disclosures
Robert E. Ungerer (University of Virgina Hospital) PRESENTER	Mr. Ungerer has nothing to disclose.
Rola Mahmoud, MD (Kansas City Physician Partners)	Dr. Mahmoud has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for TG Therapeutics, Genentech, Horizon, EMD Serono and Sanofi . Dr. Mahmoud has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for biogen.
Michael J. Schwartzman, DO	Dr. Schwartzman has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Argenx .