Superficial siderosis in Marfan syndrome

Abstract

A 49-year-old woman presented with a dorsal column-predominant sensory myelopathy secondary to superficial siderosis. She had Marfan syndrome and was found to have dural ectasia. Superficial siderosis results from subpial haemosiderin deposition due to chronic subarachnoid space bleeding, often from a dural pathology, such as a tear. Dural ectasia is common in Marfan syndrome, but superficial siderosis is not. Gait ataxia, often with hearing impairment, is a common clinical presentation of superficial siderosis. Patients may develop myelopathy, here being a pure sensory myelopathy with predominant dorsal column deficit. Meningeal diverticulae and superficial siderosis are both associated with cerebrospinal fluid (CSF) leaks. This patient had dural ectasia and meningeal diverticulae, but the CT myelogram found no CSF leak. Superficial siderosis should be considered in patients with sensory myelopathy. Lumbosacral dural ectasia, or less likely CSF leak, can cause superficial siderosis in people with Marfan syndrome.

Keywords: CSF; CSF DYNAMICS; EVOKED POTENTIALS, SOMATOSENSORY; MYELOPATHY; SUPERFICIAL SIDEROSIS.