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.

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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