Objective: To highlight the phenomenology in a complex case of superficial siderosis (SS) presenting with hearing loss, ataxia, and parkinsonism

Background: SS is a rare condition that is characterized by hemosiderin deposition within the surfaces of the brain and spinal cord. It is organized into two subtypes – cortical (cSS) and infratentorial SS (iSS) based on the locations that are affected. Infratentorial is associated with trauma while cortical has a close association with cerebral amyloid angiopathy (CAA). Infratentorial presents with the classic triad of symptoms of hearing loss, progressive ataxia, and myelopathy.

Method: A 68-year-old woman initially presented with postural instability and multiple falls. Over the course of a year, she also developed bilateral hearing loss and coordination problems. Exam was notable for scanning speech, a low amplitude resting tremor in the right hand, bradykinesia with finger taps L>R, ataxia on finger to nose and mirror trace L>R, and slow volitional eye movements with impaired saccadic initiation R>L. She required a walker to ambulate and had a wide-base, slow pace, and was unable to complete tandem gait. Strength was normal and reflexes were brisk.

Results: Axial T2-weighted brain MRI revealed significant SS of the supratentorial gyri, brainstem, and cerebellum. MRI of the C-spine, T-spine, and LS-spine demonstrated hemosiderin deposition along the entirety of the spine. Additional workup did not reveal a clear source of bleeding, vascular anomaly, or evidence of CAA, and therefore this patient’s SS was considered to be idiopathic. Due to her ataxia and parkinsonism, amantadine was initiated, which along with intense physical therapy led to significant improvement in her balance and motor coordination to the degree that she could use a cane instead of a walker and no longer had falls.

Conclusion: This case highlights parkinsonism as a manifestation of SS, in addition to the more commonly reported symptoms of ataxia and hearing loss. Addressing the underlying cause of the bleeding is the first step, but SS remains idiopathic in up to 40% of cases. Iron-chelating treatments such as deferiprone have not demonstrated clinical benefit, and therefore addressing the symptoms that are most distressing for the patient becomes the main goal of care. In this case, intensive physical therapy along with amantadine improved the patient’s parkinsonism and ataxia.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/beyond-the-classic-triad-an-unusual-phenomenology-of-parkinsonism-in-a-patient-with-superficial-siderosis-ss/