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Neurological Rehabilitation

Ataxia in a Man With Superficial Siderosis: A Case Report

(Kiosk 3) Ataxia in a Man with Superficial Siderosis: A Case Report

Friday, November 17, 2023
8:00 AM – 9:15 AM CT

    Primary Author(s)

  • Lena Sheorey, MD (she/her/hers)

    Resident Physician
    Rutgers New Jersey Medical School PM&R Program
    Jersey City, New Jersey

    • Co-Author(s)

  • AA

    Allison Averill, MD

    Director Neurorehabilitation
    Kessler Institute for Rehabilitation - West Orange
    River Vale, New Jersey

Case Diagnosis: A 63-year-old man presenting with hearing loss, ataxia, and diplopia who was diagnosed with superficial siderosis.

Case Description or Program Description: A 63-year-old man with Lupus Anticoagulant Syndrome on Warfarin presented with hearing loss, ataxia, and diplopia. His exam showed leftward tongue deviation, scissored gait, mild left shoulder weakness, and dysmetria on left finger-to-nose testing. Non-contrast CT head scan showed no acute abnormalities. However, MRI of the brain and cervical spine revealed superficial siderosis involving the intramedullary area and the proximal to mid-cervical spinal cord. He was admitted to acute rehabilitation where he underwent interdisciplinary therapy.

Setting: Acute Rehabilitation Hospital

Assessment/Results: While in acute rehabilitation, the patient improved with exercises for oculomotor strengthening, vestibular adaptation, and balance.

Discussion (relevance): Superficial Siderosis of the Central Nervous System (SSCNS) is a rare neurodegenerative disease in which hemosiderin is deposited on the pial surface of the brain or spinal cord.  It typically results from chronic low-grade blood extravasation into the subarachnoid space due to an occult source, CNS tumor, arteriovenous malformation, trauma, cerebral amyloid angiopathy, or an idiopathic cause. The 2 most common symptoms of SSCNS are hearing loss (81%) and ataxia (81%). These symptoms frequently overlap with those of multiple other conditions, including stroke, vertigo, cerebellar ataxia, and vestibular ataxia.  However, accurate and early diagnosis of SSCNS is crucial, as it allows for potential medical treatment using iron-chelators. Furthermore, while this patient experienced the 2 most common symptoms of SSCNS, he also presented with diplopia which is an atypical symptom that is documented in only 4% of SSCNS cases.

Conclusions: Superficial siderosis is a rare clinical syndrome whose non-specific symptoms overlap with many other disease processes, causing it to be either misdiagnosed or missed entirely. Therefore, comprehensive knowledge of both typical and atypical SSCNS presentations is vital in generating a broad differential for vestibular symptoms.