Abstract Details

Title Superficial Siderosis Presenting as Sensory Myelopathy in a Patient with Marfan Syndrome

Topic General Neurology

Presentation(s) P11 - Poster Session 11 (8:00 AM-9:00 AM)

Poster/Presentation
Number 2-010

Objective We report a patient with Marfan Syndrome and dural ectasia who presented with a dorsal column-predominant sensory myelopathy secondary to superficial siderosis (SS).

Background SS of the CNS results from subpial hemosiderin deposition due to chronic bleeding into the subarachnoid space. This is commonly secondary to dural pathology, such as a tear. These tears may be due to trauma (eg. surgical or osteophyte-related). Dural ectasia is a rare cause of SS but common accompaniment of Marfan syndrome. SS classically causes gait ataxia and hearing impairment. Myelopathy is a known manifestation, but a dorsal-column myelopathy from SS has not been reported.

Design/Methods Case Report

Results A 46-year-old woman presented with one year of progressive imbalance that worsened in the dark. She had intermittent non-postural headaches. Her exam demonstrated sensory ataxia and distal large-fiber predominant sensation loss in the lower limbs with preserved reflexes. Nerve conduction studies and EMG were normal. Somatosensory evoked potentials showed central slowing in proprioceptive pathways that localized to the cord. MRIs of the neuraxis revealed prominent SS affecting the spinal cord, brainstem/cerebellum, and cerebral cortex. She also had marked sacral dural ectasia and multiple thoraco-lumbar meningeal diverticula. On conventional energy-integrating detector and photon-counting detector CT myelograms, no dural leak was found. This included decubitus myelography to search for a CSF-venous fistula or lateral dural tear, as well as prone myelography to search for a ventral dural tear.

Conclusions Dural ectasia is associated with connective tissue diseases such as Marfan syndrome, and rarely associated with SS. Meningeal diverticula are associated with CSF leaks. Our patient had both dural ectasia and meningeal diverticula. While no CSF leak was identified, an empiric trial of blood patches directed at the largest diverticulum was undertaken to address potential bleeding from possible dural defects. Dural ectasia repair was not a practical surgical consideration.

Authors/Disclosures
Rishi Sharma, MD PRESENTER	Dr. Sharma has nothing to disclose.
Jeremy K. Cutsforth-Gregory, MD, FAAN (Mayo Clinic)	Dr. Cutsforth-Gregory has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for AAN. Dr. Cutsforth-Gregory has received publishing royalties from a publication relating to health care.
Ajay Madhavan	Ajay Madhavan has nothing to disclose.
Brendan Putko, MD, MSc	Dr. Putko has nothing to disclose.
Heidi Connolly, MD	Dr. Connolly has nothing to disclose.
Marcus Vinicius R. Pinto, MD (Mayo Clinic)	Dr. Pinto has nothing to disclose.

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