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Updated: Jan 26, 2026

Comprehensive Autopsy Program for Individuals with Multiple Sclerosis
Published on: July 19, 2019
Tumefactive multiple sclerosis which initially presented with brainstem encephalitis with a long-term follow-up
Akihiko Mitsutake1, Tatsuya Sato2, Junko Katsumata2
1Department of Neurology, Tokyo Teishin Hospital, 2-14-23 Fujimi, Chiyoda-ku, Tokyo 102-8798, Japan; Department of Neurology, The University of Tokyo Hospital, 7-3-1 Hongo, Bunkyo-ku, Tokyo 113-8655, Japan.
Abstract:
Tumefactive demyelinating lesions (TDLs) are rare in multiple sclerosis (MS). We herein report a case of tumefactive MS which initially presented with brainstem encephalitis with a long-term follow-up. The patient had experienced relapse mostly in the brainstem in the first twenty years, and then in the periventricular white matter afterwards. The patient responded well to steroid treatment recovered without sequalae. However, immunodeficiency due to the long-term use of oral prednisolone made aggressive therapy during the relapse impossible, so recovery after steroid therapy is incomplete. Our case is different from classical MS in clinical course and response to treatment. Our report offers rare information on long-term outcome of tumefactive MS.
Insights
This case study details a rare instance of tumefactive multiple sclerosis (MS) presenting as brainstem encephalitis. Long-term steroid use complicated treatment, leading to incomplete recovery and highlighting atypical MS progression.
Area of Science:
- Neurology
- Neuroimmunology
- Demyelinating Diseases
Background:
- Tumefactive demyelinating lesions (TDLs) are uncommon manifestations of multiple sclerosis (MS).
- This report focuses on a unique case of tumefactive MS with an initial presentation of brainstem encephalitis.
Observation:
- The patient experienced relapses predominantly in the brainstem for two decades, followed by periventricular white matter involvement.
- Initial response to steroid treatment was favorable, with recovery.
- Long-term oral steroid use led to immunodeficiency, precluding aggressive therapies and resulting in incomplete recovery during relapses.
Findings:
- The clinical course and treatment response deviated from classical multiple sclerosis presentations.
- This case provides rare insights into the long-term outcomes of tumefactive MS.
Implications:
- Understanding atypical presentations of tumefactive MS is crucial for accurate diagnosis and management.
- The long-term effects of immunosuppression in MS require careful consideration.
- This case underscores the heterogeneity of MS and the importance of individualized treatment strategies.
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