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CREST-associated multiple intracranial aneurysms and bilateral optic neuropathies
J R Ortiz1, N J Newman, D L Barrow
1Department of Ophthalmology, Emory University School of Medicine, Atlanta, Georgia.
Journal of Clinical Neuro-Ophthalmology
|December 1, 1991
Summary
CREST syndrome, a variant of progressive systemic sclerosis, can lead to multiple brain aneurysms and optic nerve damage. The study explores potential ischemic and compressive causes linked to the disease.
Area of Science:
- Neurology
- Rheumatology
- Ophthalmology
Background:
- CREST syndrome, a limited cutaneous variant of progressive systemic sclerosis (PSS), is characterized by calcinosis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangiectasias.
- Systemic sclerosis is associated with various neurological complications, though intracranial vascular abnormalities are less commonly reported.
Observation:
- A patient diagnosed with CREST syndrome presented with multiple intracranial aneurysms.
- The patient subsequently developed bilateral optic neuropathies, indicating a severe complication affecting vision.
Findings:
- The co-occurrence of intracranial aneurysms and optic neuropathies in CREST syndrome suggests a potential link between the systemic vasculopathy of PSS and cerebrovascular events.
- Histopathological examination of the underlying systemic disease is proposed to elucidate the mechanisms, potentially involving ischemic or compressive etiologies.
Implications:
- This case highlights the importance of considering cerebrovascular complications in patients with CREST syndrome.
- Further research into the pathogenesis of neurological complications in systemic sclerosis is warranted to improve patient management and outcomes.
- Ophthalmological and neurological monitoring may be crucial for early detection of severe complications in affected individuals.