Appendix
← Clinical Reader
Neurology

Superficial Siderosis

Superficial siderosis is a rare neurological disorder characterized by iron deposition in the superficial layers of the brain and spinal cord. This condition typically arises from chronic bleeding into the subarachnoid space, often due to previous head or spinal trauma, tumors, or vascular malformations. As iron accumulates over time, it damages nerve tissues, leading to a spectrum of symptoms ranging from progressive hearing loss and imbalance to cognitive decline and neurological deficits. Des

v1 · Reviewed Nov 8, 2026 by Automated Clinical Reviewer (Strict Verbatim v1)
  • As iron accumulates over time, it damages nerve tissues, leading to a spectrum of symptoms ranging from progressive hearing loss and imbalance to cognitive decline and neurological deficits.
  • Patients with superficial siderosis can be asymptomatic but commonly present with gait ataxia, myelopathy, or hearing impairment in varying combinations.
  • Cerebellar signs are obligatory, whereas signs of cranial nerve dysfunction and myeloradiculopathy are facultative.
  • Cortical superficial siderosis progression was a predictor of incident symptomatic lobar intracerebral hemorrhage.
  • Diagnosis is made when characteristic hemosiderin deposition is seen on iron-specific magnetic resonance imaging (MRI) sequences like gradient-echo (GRE) and susceptibility-weighted imaging (SWI).
  • MRI of the entire neuroaxis is the investigation of choice for the diagnosis and initial workup in patients with superficial siderosis to define the extent of superficial siderosis and to localize a potential bleeding source.
  • The brain MRI should be performed with contrast as the presence of pachymeningeal enhancement may suggest a dural tear and associated CSF leak or intracranial hypotension.
  • Brain MRI should include a sequence sensitive to the magnetic susceptibility property of blood products like GRE or SWI.
  • Hemosiderin deposition on MRI is seen as a T2 hypointensity and preferentially outlines the cerebellum and brainstem surface.
  • SWI is better than GRE-T2 for revealing iron deposits.
  • Constructive interference in steady state (3D-CISS) reveals an increment in caliber, and susceptibility-weighted imaging (SWI) shows characteristic hypointense linear bands.
  • The MRI of the spinal cord may reveal myelomalacia, syringomyelia, “owl-eyes,” or “snake-eyes” signs.
  • A CT is not the imaging modality of choice but can show a hyperdense ring around the brainstem, suggesting superficial siderosis.
  • If the MRI of the neuroaxis shows an epidural fluid collection or is unrevealing, a CT or MR myelogram should be performed to identify potential dural defects.
  • A dural defect is shown on the CT myelogram when intrathecal contrast fills an epidural fluid collection, inferring communication with the subarachnoid space and a dural defect.
  • Dynamic CT myelography and steady-state free precession (SSFP) sequence may help identify the location of a small dural leak with associated epidural fluid collection when an initial CT myelogram is not diagnostic.
  • CISS is also better at revealing dural defects.
  • Digital subtraction angiography should only be pursued when CT or MR angiography suggests a spinal or cranial vascular malformation.
  • A lumbar puncture may provide helpful information as increased RBCs in the CSF suggest active bleeding.
  • Chronic bleeding can also result in an elevation in CSF protein, iron, and ferritin.
  • Neurological findings include bilateral increment in P1 and N1 latencies in sacculocollic reflex, desynchronization in brainstem auditory evoked potentials (BAEP), and partial or complete loss of the visual suppression of vestibular-ocular reflex (vermian lesion).
  • Treatment depends on the etiology of superficial siderosis.
  • If a dural defect is identified, regardless of whether the patient is asymptomatic, it is recommended to pursue treatment given the slow progression and potential for neurological injury.
  • Dural defects are often identified using CT myelography and repaired with sutures, patches, muscle/fat grafts, or fibrin glue.
  • Blood patches and minimally invasive repair of dural defects are other therapeutic options.
  • Treatment for other underlying causes of superficial siderosis, including vascular malformations, cerebral amyloid angiopathy, reversible cerebral vasoconstriction syndromes, cerebral cortical venous thrombosis, and central nervous system tumors, focuses on treating each specific disease to reduce bleeding in the subarachnoid space.
  • Management with supportive care can help patients acclimate and prevent falls and further injuries.
  • Physical and occupational therapy can be helpful for individuals with gait ataxia, pyramidal symptoms, and balance issues and should be offered to every patient with superficial siderosis.
  • Cochlear implantation has shown some benefits, but the results of sustained benefits are mixed.
  • Given the possibility of improvement, it is reasonable to refer patients to an otologist for cochlear implantation, but further deterioration due to superficial siderosis is a potential factor in decision-making.
  • When a referral is made for cochlear implantation, it is important to counsel the patient on the limited benefit of the surgery and the eventual decline of hearing due to the progressive course of superficial siderosis.
  • Chelation therapy has been studied in superficial siderosis due to the pathophysiology of hemosiderin deposition as the cause of superficial siderosis.
  • Iron chelators such as deferiprone reduce the amount of hemosiderin and iron deposition on brain MRI.
  • Currently, chelating agents are not recommended in patients with superficial siderosis as they have not been shown to provide a clinically meaningful benefit.
  • After the dural defect is repaired, markers of successful closure include the resolution of CSF xanthochromia and epidural fluid collection.
  • Clinical progression of superficial siderosis can be halted, although long-term follow-up is still required.
  • The most frequent complications with superficial siderosis include hearing and peripheral vestibular loss and falls secondary to gait impairment.
  • Other complications include anosmia, amyloid spells (transient neurological episodes that can mimic transient ischemic attacks), dementia, sensory deficits, bladder incontinence, and cranial nerve dysfunction.
  • Cortical superficial siderosis progression was a predictor of incident symptomatic lobar intracerebral hemorrhage.
  • The most concerning complication of deferiprone chelating therapy is agranulocytosis.
  • Regular long-term monitoring of patients with superficial siderosis should be implemented to assess disease progression, monitor for new symptoms, and adjust treatment strategies as needed.
  • Although the evidence for cochlear implantation is mixed, tracking hearing over time can help patients stay informed and adjust to changes.
  • review
    Superficial Siderosis. StatPearls [Internet] — Justin Hoskin, Kyle Kaneko, Sunil Munakomi, Terry Fife — StatPearls Publishing, 2024

Decision support only. Verify against local protocols and current labeling. Source: www.ncbi.nlm.nih.gov