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Evidence for preganglionic pupillary involvement in superficial siderosis
V S Pelak1, S L Galetta, R I Grossman
1Department of Neurology, Hospital of the University of Pennsylvania, University of Pennsylvania School of Medicine, Philadelphia 19104, USA.
Abstract:
A 36-year-old man presented with spinal myoclonus, ataxia, hearing loss, and unilateral pupillary dilation. MRI demonstrated hemosiderin deposition along the superficial surfaces of the brain, brainstem, cerebellum, and spine. The pupillary changes were localized to the preganglionic oculomotor nerve. In contrast to vasculopathic oculomotor nerve palsies, superficial siderosis may cause selective involvement of the superficially located pupillary fibers.
Insights
Superficial siderosis, a condition causing hemosiderin deposition, can lead to spinal myoclonus, ataxia, and hearing loss. This case highlights its potential to selectively affect pupillary fibers of the oculomotor nerve.
Area of Science:
- Neurology
- Neuroimaging
- Neuro-ophthalmology
Background:
- Superficial siderosis is characterized by iron deposition in the brain's superficial layers.
- It can manifest with diverse neurological symptoms.
Observation:
- A 36-year-old male presented with spinal myoclonus, ataxia, hearing loss, and unilateral pupillary dilation.
- Magnetic Resonance Imaging (MRI) revealed hemosiderin deposition throughout the central nervous system.
Findings:
- The pupillary abnormalities were specifically linked to the preganglionic oculomotor nerve.
- This suggests superficial siderosis can selectively impact superficial pupillary fibers, differing from vasculopathic causes.
Implications:
- This case expands the understanding of superficial siderosis's clinical manifestations.
- It emphasizes the importance of considering superficial siderosis in the differential diagnosis of oculomotor nerve palsies with pupillary involvement.