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Corticosteroid sensitivity in gliomatosis cerebri delays diagnosis
  1. Jan Bittar1,
  2. Carlos Kamiya-Matsuoka1,
  3. Pedro C Barata1,
  4. Soo-Hyun Lee-Kim1,
  5. Adriana Olar2,
  6. Ivo W Tremont-Lukats1
  1. 1Department of Neuro-Oncology, The University of Texas-M.D. Anderson Cancer Center, Houston, Texas, USA
  2. 2Department of Pathology, The University of Texas-M.D. Anderson Cancer Center, Houston, Texas, USA
  1. Correspondence to Dr Carlos Kamiya-Matsuoka,1400 Holcombe Blvd, Room FC7.3000, Unit 431, Houston, Texas 77030, USA; ckamiya{at}mdanderson.org

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Introduction

Gliomatosis cerebri is a rare malignant tumour originating from cerebral glial cells. Most experts currently view gliomatosis cerebri as a pattern of extensive glioma infiltration encompassing at least three cerebral lobes.1 It occurs at any age, with peak incidence between 40 years and 50 years and it is slightly more common in men.2 On MRI, gliomatosis cerebri shows no, or minimal, contrast enhancement; however, it has a diffuse hyperintense infiltrative process on T2-weighted fluid-attenuated inversion recovery (FLAIR) sequence. It is usually possible to diagnose gliomatosis cerebri using an open biopsy or a stereotactic biopsy. Despite treatment, its median survival varies from 6 months to 39 months.3 We report an unusual presentation of gliomatosis cerebri, which underscores the difficulty in pinpointing the correct diagnosis after corticosteroids have modified the disease course.

Case report

A 78-year-old woman had an episode of acute onset slurred speech and confusion. Her initial CT scan of head showed diffuse left hemispherical white matter hypodensity with sulcal effacement and minimal midline shift to the right. Cerebrospinal fluid (CSF) examination suggested viral encephalitis for which she was empirically given intravenous dexamethasone and acyclovir, with oral levetiracetam 500 mg twice daily. After initially improving, she rapidly worsened with corticosteroid tapering. A repeat CT scan of head showed no change. She took dexamethasone 4 mg twice daily before undergoing a brain biopsy, which showed mild hypercellularity suspicious but not definitive for glioma.

Two weeks later, we saw her for a second opinion. The MRI of …

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    Phil Smith Geraint N Fuller