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Updated: Feb 17, 2026

Longitudinal In Vivo Imaging of the Cerebrovasculature: Relevance to CNS Diseases
Published on: December 6, 2016
The evolution of cerebrovascular changes in Köhlmeier-Degos disease: An 11-year follow-up case report
Louie Ye1, Ernest Lekgabe1, Alpha Tsui2
1Department of Radiology, the Royal Melbourne Hospital, Parkville, VIC, Australia.
Insights
Köhlmeier-Degos disease, a rare vasculopathy, often involves the central nervous system. This case study details 11-year neuroimaging findings, offering new insights into its progression.
Area of Science:
- Neurology
- Vascular Biology
- Rare Diseases
Background:
- Köhlmeier-Degos disease is a rare idiopathic vasculopathy with unclear pathogenesis.
- Central nervous system (CNS) involvement in Köhlmeier-Degos disease is uncommon and poorly understood.
- Longitudinal studies detailing the evolution of neurovascular and neuropathological changes are lacking.
Abstract:
Köhlmeier-Degos disease is rare idiopathic vasculopathy, the exact pathogenesis of which remains unclear. Here, we review pertinent literatutre and present a case of a Köhlmeier-Degos disease with central nervous system involvement followed-up over 11 years with various neuroimaging modalities. Evolution of neurovascular and neuropathological changes over an extended time period has not been previously described.
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