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A rare presentation of atypical demyelination: tumefactive multiple sclerosis causing Gerstmann's syndrome

Sharmilee Gnanapavan, Zane Jaunmuktane, Kelly Pegoretti Baruteau

  • 1Blizard Institute, Barts and The London School of Medicine & Dentistry, Queen Mary, University of London, London, UK. k.schmierer@qmul.ac.uk.

BMC Neurology
|April 4, 2014
PubMed
Abstract

Insights

Tumefactive multiple sclerosis (MS) can present unusually. Early biopsy and natalizumab treatment aided a patient with a complex cognitive syndrome and a large demyelinating lesion.

Area of Science:

  • Neurology
  • Neuroimmunology

Background:

  • Tumefactive demyelinating lesions are rare in multiple sclerosis (MS), often posing diagnostic challenges.
  • Complex cognitive syndromes are uncommon in tumefactive MS, complicating differential diagnoses.

Observation:

  • A 30-year-old woman presented with Gerstmann's syndrome and a large, heterogeneous, contrast-enhancing lesion in the left cerebral hemisphere on MRI.
  • Initial treatment with intravenous corticosteroids was ineffective, and a suspected tumor or lymphoma prompted brain biopsy.
  • Brain biopsy confirmed inflammatory demyelination, leading to a diagnosis of tumefactive MS.

Findings:

  • The patient's tumefactive MS lesion presented with an unusual complex cognitive syndrome (Gerstmann's syndrome).
  • Natalizumab treatment effectively suppressed disease activity following the diagnosis of tumefactive MS.

Implications:

  • This case underscores the importance of recognizing the diverse presentations of tumefactive MS.
  • Timely diagnosis through brain biopsy is crucial for appropriate management.
  • Natalizumab may be a beneficial treatment for tumefactive demyelination in relapsing MS.

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