SUPERFICIAL SIDEROSIS: SEALING THE DEFECT

Abstract

Superficial siderosis (SS) of the CNS results from chronic hemorrhage into the subarachnoid space with hemosiderin deposition in the subpial layers.1,2 The clinical presentation includes progressive ataxia and deafness. Some patients have a history of trauma or intradural surgery. Despite extensive investigations, a cause of bleeding is frequently elusive. An extra-arachnoid, intraspinal, or intracranial CSF collection, often longitudinally extensive, is sometimes identified in spinal neuroimaging in SS.1–6 A dynamic CT myelogram can identify the dural defect connecting the intrathecal space with the fluid-filled collection.4 The precise mechanism of bleeding is unknown. Rarely CSF hypovolemia accompanies SS.6 Increased CSF RBC count may be seen in CSF hypovolemia.7 These observations have led to the suggestion that repairing the dural defect may halt bleeding and prevent deficit progression.6 Clinical confirmation of this hypothesis is lacking. We describe a patient with SS and CSF hypovolemia due to a CSF leak. Repair of the leak was accompanied by clinical improvement and resolution of neuroimaging and CSF abnormalities. ### Case report. A 64-year-old man was evaluated for a 3-year history of progressive imbalance and 10-year history of decreased hearing. His history was remarkable for childhood poliomyelitis. Over the years he had multiple horse riding-related falls. Twenty years earlier he had a C4-C7 laminectomy for right upper limb weakness. On examination, he had mild …