Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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August 29, 2011
Transthyretin-Associated Amyloidomas
- Amyloidoses are a group of diseases characterised by deposition of insoluble fibrillar proteins in tissues. The most common CNS finding is of cerebral amyloid angiopathy, which can lead to hemorrhage.
- Localised masses of amyloid, or amyloidomas, is the rarest form of cerebral amyloidosis. Such masses are usually located supratentorially within the subocortical white matter, with medial extension to the lateral ventricle ependyma.
- Clinical progression is similar to that of a slow-growing neoplasm, and patients may present with seizures, headache, focal motor signs, and cognitive decline.
- Key Diagnostic Features: On CT amyloidomas typically appear hyperdense with little or no mass effect. On MR imaging amyloidomas can appear hypo/iso/hyper-intense on T1 images, return mixed signal on T2 images, and demonstrate variable contrast enhancement.
- DDx: PML, Tumefactive MS
- Rx: Surgery—however, lesions can recur.
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