MRI of the brain in cerebrotendinous xanthomatosis (van Bogaert-Scherer-Epstein disease) |
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| Authors: | F. Vanrietvelde M. Lemmerling M. Mespreuve L. Crevits J. De Reuck M. Kunnen |
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| Affiliation: | (1) Department of Radiology, University Hospital Gent, Gent, Belgium, BE;(2) Department of Radiology, General Hospital St. Joseph Mechelen, Mechelen, Belgium, BE;(3) Department of Neurology, University Hospital Gent, Gent, Belgium, BE |
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| Abstract: | The clinical, biochemical and magnetic resonance imaging findings of two patients with cerebrotendinous xanthomatosis are reported. This is a rare hereditary disease. Early recognition of this entity is important in view of the existing treatment possibilities. Magnetic resonance imaging findings typically include a bilateral and almost symmetrical increase of the signal intensity on the T2-weighted images in the cerebellar and periventricular cerebral white matter, the basal ganglia, the dentate nuclei and the brainstem as well as cerebellar and cerebral atrophy. Received: 18 January 1999; Revised: 3 June 1999; Accepted: 7 June 1999 |
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| Keywords: | : Cerebrotendinous xanthomatosis – MR imaging – Posterior fossa – Choroid plexus |
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