Case of the Month
Section Editor: Nicholas Stence, MD
Children's Hospital Colorado, Aurora, CO
November 2020
Next Case of the Month Coming December 8...
Tumefactive Demyelinating Lesion (TDL)
- Background:
- It is a locally aggressive form of demyelination, usually manifesting as a solitary lesion greater than 2 cm that may mimic a neoplasm on imaging.
- Clinical Presentation:
- Patients present with symptoms atypical for multiple sclerosis, such as focal neurologic deficits, seizures, and/or aphasia.
- Patients who present with a solitary tumefactive demyelinating lesion infrequently go on to develop multiple sclerosis.
- Key Diagnostic Features:
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Lesions tend to be large (>2 cm) but with relatively little mass effect or surrounding edema.
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Approximately half of TDLs have pathologic contrast enhancement; the most frequent pattern will be in the form of an open ring, with the incomplete portion of the ring on the gray matter side of the lesion.
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Central traversing vein can be seen within the lesion.
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Peripheral rim showing diffusion restriction
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MRS: marked elevation of the glutamate and glutamine peaks (2.1–2.5 ppm); other findings included elevation of the choline peak (3.2 ppm), elevation of the lactate peak (1.3 ppm), elevation of the lipid peak (0.5–1.5 ppm), and decrease in the NAA peak (2.0 ppm).
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rCBV within TDL is substantially less than that found in high-grade gliomas.
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- Differential Diagnoses
- High-grade glioma: enhancement is usually a complete ring around central necrosis with prominent surrounding vasogenic edema.
- CNS lymphoma: vivid, usually solid enhancement, without central nonenhancement (except sometimes in immunocompromised individuals)
- Cerebral infective process: cerebritis or cerebral abscess - demonstrate restricted diffusion prominent in central liquid component
- Treatment:
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TDLs showed an excellent response to corticosteroid treatment, with a substantial decrease in lesion size or disappearance on follow-up imaging.
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