Tumefactive demyelination: an approach to diagnosis and management

This review article looks at tumefactive demyelination and proposes an approach to diagnosis and management. Tumefactive demyelinating (TD) lesions are larger than those seen in typical MS (>2cm) and may occur in individuals either with or without established MS. The presence of radiological features can spare patients the need for brain biopsy.

On MRI, most pathologically proven tumefactive lesions have a closed ring appearance. Recent observations show a non-contrast CT brain scan in addition to MRI imaging can improve diagnostic accuracy of TD lesions compared with MRI alone. Specifically CT hypoattenuation of MRI enhanced regions appear to be more predictive of TD than tumour. In relation to the pathology, there are no histological features that distinguish isolated TD from the demyelination seen in more typical MS lesions. It is not clear if TD is immunopathologically distinct from MS.

As regards treatment, acute lesions are treated with corticosteroids but the timing of longer term disease modifying therapy (DMT) is less clear. When a diagnosis of MS is fulfilled, treatment should be with conventional first line MS DMT agents and escalated to second line DMTs if those agents fail to control further events.

Authors: Hardy TA, Chataway J.

Source: J Neurol Neurosurg Psychiatry. 2013 Jan 19. [Epub ahead of print]

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