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.