Tumefactive demyelination: clinical, imaging and follow-up observations in thirty-nine patients
Purpose 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 i...
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Published in | Acta neurologica Scandinavica Vol. 128; no. 1; pp. 39 - 47 |
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Main Authors | , , , , , , , , |
Format | Journal Article |
Language | English |
Published |
Denmark
Blackwell Publishing Ltd
01.07.2013
Hindawi Limited |
Subjects | |
Online Access | Get full text |
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Summary: | Purpose
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.
Results
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).
Conclusions
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. |
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Bibliography: | ark:/67375/WNG-V3V1M0LS-9 istex:AAE40EE69CA797E62E613043A63DF47E478CD9FB Table S1. Clinical, Imaging and Follow-up observation in tumefactive demyelination - Monophasic group. Table S2. Clinical, Imaging, and Follow-up observation in Tumefactive Demyelination - Relapsing - remitting group. ArticleID:ANE12071 ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 content type line 23 ObjectType-Article-2 ObjectType-Feature-1 |
ISSN: | 0001-6314 1600-0404 |
DOI: | 10.1111/ane.12071 |