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Updated: Oct 2, 2026

Reliable Isolation of Central Nervous System Microvessels Across Five Vertebrate Groups
Published on: January 12, 2020
[Two patients with isolated vasculitis of the central nervous system]
J J B Klück1, J W M ter Berg, R T van der Laan
1Afd. Neurologie, Maaslandziekenhuis, Postbus 5500, 6130 MB Sittard.
Abstract:
Over the course of time, a 43-year-old woman and a 39-year-old man developed visual, sensory, motor, cerebellar and cognitive disturbances. Initially these occurred episodically, but later became continuous. Isolated vasculitis of the central nervous system was only diagnosed after brain biopsies were taken, upon which treatment with prednisone and cyclophosphamide was instituted. In both patients the symptoms persisted for 23 and 19 months, respectively, after treatment was initiated, but no new symptoms developed. The woman did, however, develop pancytopenia as a result of the cyclophosphamide treatment. Non-infectious vasculitis of the small veins and arterioles of the brain is lethal if left untreated. Leptomeningeal and cortical biopsy is essential in establishing the diagnosis in order to rule out other causative diseases. Combination therapy consisting of prednisone and cyclophosphamide for at least one year is recommended. The efficacy of this treatment is unknown due to the rarity of this disease.
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