Tumefactive demyelination: clinical, imaging and follow-up observations in thirty-nine patients
Article first published online: 31 DEC 2012
© 2012 John Wiley & Sons A/S
Acta Neurologica Scandinavica
Volume 128, Issue 1, pages 39–47, July 2013
How to Cite
Tumefactive demyelination: clinical, imaging and follow-up observations in 39 patients. Acta Neurol Scand 2013: 128: 39–47. © 2012 John Wiley & Sons A/S, , , , , , , , .
- Issue published online: 13 JUN 2013
- Article first published online: 31 DEC 2012
- Manuscript Accepted: 23 NOV 2012
- magnetic resonance imaging;
- tumefactive demyelination
We describe the clinical, neuroimaging and pathological features and therapeutic outcome in a large cohort of 39 patients with tumefactive demyelination.
Materials and Methods
A retrospective audit of 39 patients with ‘tumefactive demyelination’ was performed. The demographic, clinical, MR imaging and pathological details were reviewed.
The clinical course was monophasic (n = 22) or relapsing–remitting (n = 17). Common neurological manifestations at presentation included hemiparesis - 27; ataxia - 11; vomiting - 10; headache -9; ophthalmoplegia - 7; seizure - 5; impaired vision - 4; aphasia - 4; visual field defects - 3; papilloedema - 5; extrapyramidal - 5; intellectual decline - 5; behavioural disturbances - 3; altered sensorium - 5. MRI revealed fronto-parietal lesions, which were isolated in 14 (36%) patients. Moderate perilesional oedema and/or mass effect was noted in 12 (30.8%) patients. Post-contrast MR sequences revealed partial ring enhancement in 15, complete ring in seven, patchy enhancement in six, uniform enhancement in two and lack of enhancement in nine cases. Clinical and MR characteristics did not help distinguish between monophasic and relapsing-remitting subgroups. In the monophasic group, 53.8% had complete recovery, while 38.5% had partial improvement (follow-up duration, 8.31 ± 9.3 months). In the relapsing-remitting subgroup, the median time to relapse was 4 months (n = 12, follow-up, 37.8 ± 39.4 months). Patients with monophasic course or single relapse received steroids. Patients with more than one relapse received cyclophosphamide (2), mycophenolate (1), azathioprine (1) or methotrexate (1).
A high proportion of cases of tumefactive demyelination follow a relapsing course, thus necessitating a long-term follow-up. MRI, although helpful in diagnosis, does not predict monophasic or relapsing-remitting course. Guidelines for the management of acute episodes and prevention of relapses are required.