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Sneddon's disease presenting with visual loss and dementia.
W B Wilson1, D B Smith, R R Wright
1Department of Ophthalmology, University of Colorado School of Medicine, Denver.
Journal of Clinical Neuro-Ophthalmology
|December 1, 1988
Summary
Sneddon's disease, a rare condition, can cause stroke-like symptoms and vision loss. This case highlights its potential link to immune system dysfunction and vasculopathy.
Area of Science:
- Neurology
- Ophthalmology
- Rheumatology
Background:
- Sneddon's disease is a rare, non-inflammatory occlusive hydrocephalus characterized by generalized livedo reticularis and recurrent neurological events.
- The exact etiology of Sneddon's disease remains unclear, but it is increasingly suspected to involve an autoimmune component.
Observation:
- A 51-year-old woman with Sneddon's disease presented with transient visual field loss and hemiparesis, alongside a decade of cognitive decline.
- The patient exhibited characteristic skin changes of generalized livedo reticularis, but lacked Raynaud's phenomenon or digital ulcers.
Findings:
- The patient's neurological symptoms, including transient hemifield vision loss and hemiparesis, were attributed to Sneddon's disease after association with her cutaneous manifestations.
- Review of literature and this case suggest Sneddon's disease may be an immunologically mediated vasculopathy.
Implications:
- This case underscores the importance of recognizing the neuro-ophthalmic manifestations of Sneddon's disease.
- Further research into the immunological aspects of Sneddon's disease is warranted to explore potential therapeutic targets.