This case report describes a male diagnosed with Superficial Siderosis of the central nervous system with associated autonomic dysfunction. 

Superficial Siderosis is a disorder in which hemosiderin deposits on the pial surface of the brain and/or spinal cord and is generally associated with the clinical triad of hearing loss, ataxia, and myelopathy. Autonomic impairment is not widely reported.

Case Presentation: A 56-year-old man with gait impairment and imbalance was referred for 2 cervical spine surgeries for presumed compressive myelopathy. By age 71 he had developed cerebellar ataxia, bilateral sensorineural hearing loss, spastic paraparesis, sensory ataxia, peripheral sensory loss, urinary retention, early cognitive changes, and depression. By age 76 he developed orthostatic hypotension, orthostatic headaches, and coat hanger pain syndrome.

Magnetic resonance imaging of the brain and spine revealed evidence of diffuse superficial siderosis. Computed tomography myelogram was suggestive of a dural tear at C4-5. Laboratory tests to screen for common causes of sensory loss and peripheral neuropathy were unremarkable. Nerve conduction studies and electromyography were consistent with mild distal sensory/motor polyneuropathy versus age-related changes, which did not explain the severity of his symptoms. Autonomic reflex testing revealed evidence for distal-predominant postganglionic sympathetic sudomotor dysfunction with cardiovascular adrenergic failure accompanied by orthostatic hypotension. 

This case is a patient with superficial siderosis of the brain and spinal cord who developed autonomic failure manifested as orthostatic hypotension. Prior case reports have suggested that polyradiculopathy and axonal polyneuropathy may also be associated with superficial siderosis. We hypothesize that the peripheral autonomic fibers may be affected by the same unknown mechanism. We also hypothesize that there may be a central component of autonomic failure in this case, given the brainstem involvement. This case provides support for the likelihood that superficial siderosis may cause autonomic dysfunction with both peripheral and central manifestations.