Superficial Siderosis of the Central Nervous System: A Case Report of the Sparsely Occurring Neurological Disorder Accompanied by Review of Literature

Mishra BK, Pradhan RK, Dash S, Mishra SV and Mathur D

Department of Neurology, Care Hospitals, Bhubaneswar, Odisha, India
Department of Neurology, Apollo Hospitals, Bhubaneswar, Odisha, India
IMS and SUM Hospital, Bhubaneswar, Odisha, India

^*Correspondance to: Mathur D 

 PDF  Full Text Case Report | Open Access

Abstract:

Background: Superficial Siderosis (SS) of the Central Nervous System (CNS) is a neurological dysfunction that occurs sparsely and is attributable to chronic and repetitive outpouring of blood into the cerebrospinal fluid. This results in accumulation of iron on the surface of the brain and spinal cord, which damages nerve tissues over time. Generally, these patients present with cerebellar ataxia, progressive and irreversible Sensorineural Hearing Loss (SNHL), and bilateral pyramidal impairment, but very few manifest all three symptoms simultaneously, making diagnosis and treatment arduous. Case Presentation: We report the case of a man in his late fifties who came to the hospital with complaints of unsteadiness during walking, headache, decreased hearing, and tingling and numbness in his bilateral lower limbs for the last 3–5 years. He had a medical history of hypertension and type II diabetes mellitus. Results: A physical examination revealed cerebellar ataxia, gaze-evoked nystagmus, broken pursuit and slow saccades, progressive bilateral SNHL, corticospinal and dorsal column involvement. Bilateral intention tremor was present. Bilateral finger–nose test, heel–knee–shin test, and tandem walking were impaired, indicating cerebellar dysfunction. Cranial nerve VIII was impaired, Weber test lateralized to the right side, and Romberg sign was positive. Conclusion: This case indicates that patients presenting with progressive cerebellar ataxia, hearing loss, headache, and bilateral pyramidal and dorsal column signs may have the possibility of developing superficial siderosis, and the diagnosis should be further corroborated by Magnetic Resonance Imaging (MRI) along with angiography and venography.

Keywords:

Superficial siderosis of the central nervous system; Cerebellar ataxia; Sensorineural deafness; Case report; Iron deposition.

Cite the Article:

Mishra BK, Pradhan RK, Dash S, Mishra SV, Mathur D. Superficial Siderosis of the Central Nervous System: A Case Report of the Sparsely Occurring Neurological Disorder Accompanied by Review of Literature. Clin Case Rep Int. 2025; 9: 1738..