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Superficial siderosis of central nervous system — Clinical Guidelines

Overview

Superficial siderosis of the central nervous system (CNS) is a rare disorder characterized by chronic or recurrent subarachnoid hemorrhages leading to hemosiderin deposition in the subpial layers of the brain and spinal cord, causing progressive neurological deficits such as sensorineural hearing loss, cerebellar ataxia, and pyramidal signs 1 8.

Diagnosis

MRI Findings: Diffuse hemosiderin staining on gradient-echo sequences, particularly in cerebellar convexities and cerebral regions 3.

CSF Analysis: Xanthochromia and elevated iron/ferritin levels can support diagnosis 9.

Spinal Imaging: Gadolinium-enhanced MRI and spinal angiography to identify dural defects, spinal cord herniation, or vascular malformations 2 7.

Clinical Presentation: Symmetric infratentorial siderosis pattern often suggests spinal dural leaks; evaluate for spinal pathologies in such cases 1.

Neurological Examination: Focus on sensorineural hearing loss, cerebellar dysfunction, and pyramidal tract involvement 8.

History of Bleeding Events: Assess for history of subarachnoid hemorrhage or other bleeding episodes 1 8.

Management

Iron Chelation Therapy: Trientine dihydrochloride may improve clinical symptoms and reduce CSF iron levels 9.

Surgical Intervention: Consider surgical repair of dural defects or resection of vascular malformations when identified 2 7.

Symptomatic Treatment: Manage seizures with anticonvulsants like carbamazepine; use analgesics for headache management 5.

Supportive Care: Address ataxia, hearing loss, and other neurological deficits with physical therapy and assistive devices as needed 8.

Special Populations

Elderly: Commonly affected; management focuses on symptomatic relief and supportive care 8.

Comorbidities: Presence of conditions like amyloid angiopathy or cavernous malformations may influence underlying bleeding sources 6.

Key Recommendations

Perform gadolinium-enhanced spinal MRI and spinal angiography in patients with superficial siderosis and no identifiable intracranial bleeding source to detect spinal dural defects or vascular malformations (Evidence: Moderate 2 7).

Consider iron chelation therapy with trientine dihydrochloride for patients with elevated CSF iron levels to potentially improve clinical outcomes (Evidence: Weak 9).

Evaluate for and address underlying causes such as spinal cord herniation or arteriovenous malformations surgically when identified, as this can lead to symptom resolution (Evidence: Expert opinion 2 7).

References

1 Friedauer L, Rezny-Kasprzak B, Steinmetz H, du Mesnil de Rochemont R, Foerch C. Spinal dural leaks in patients with infratentorial superficial siderosis of the central nervous system-Refinement of a diagnostic algorithm. European journal of neurology 2022. link 2 Boncoraglio GB, Ballabio E, Erbetta A, Prada F, Savoiardo M, Parati EA. Superficial siderosis due to dural defect with thoracic spinal cord herniation. Journal of the neurological sciences 2012. link 3 Gonella MC, Fischbein NJ, Lane B, Shuer LM, Greicius MD. Episodic encephalopathy due to an occult spinal vascular malformation complicated by superficial siderosis. Clinical neurology and neurosurgery 2010. link 4 Wang K, Xu Z, Xiong G, Benyan L. Superficial siderosis of the central nervous system manifested with seizures. Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia 2010. link 5 Iannaccone S, Golzi V, Sferrazza B, de Rino F, Smirne S, Ferini-Strambi L. Central nervous system superficial siderosis, headache, and epilepsy. Headache 1999. link 6 Hsu WC, Loevner LA, Forman MS, Thaler ER. Superficial siderosis of the CNS associated with multiple cavernous malformations. AJNR. American journal of neuroradiology 1999. link 7 Schievink WI, Apostolides PJ, Spetzler RF. Surgical treatment of superficial siderosis associated with a spinal arteriovenous malformation. Case report. Journal of neurosurgery 1998. link 8 Fearnley JM, Stevens JM, Rudge P. Superficial siderosis of the central nervous system. Brain : a journal of neurology 1995. link 9 River Y, Honigman S, Gomori JM, Reches A. Superficial hemosiderosis of the central nervous system. Movement disorders : official journal of the Movement Disorder Society 1994. link

Superficial siderosis of central nervous system