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A case of malignant monophasic multiple sclerosis (Marburg's disease type) successfully treated with decompressive hemicraniectomy
  1. Jose J González Sánchez1,
  2. Joaquim Enseñat Nora2,
  3. Matteo de Notaris1,
  4. Jordi Rumià Arboix1,
  5. Carlos García-Amorena García1,
  6. Enrique Ferrer Rodríguez1
  1. 1Division of Neurosurgery, Clinic Institute of Neurological Diseases, Hospital Clinic of Barcelona, Barcelona, Spain
  2. 2Department of Neurological Sciences, Division of Neurosurgery, Universita degli Studi di Napoli Federico II, Naples, Italy
  1. Correspondence to Dr Jose J González Sánchez, Division of Neurosurgery, Hospital Clinic of Barcelona, Stair 4th, 5th Floor, Casanovas Street, Barcelona, Spain; jjgcmb{at}hotmail.com

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Among multiple sclerosis (MS) variants, Otto Marburg1 described in 1906 three cases of monophasic acute demyelination disease with progressive patient deterioration and death. Marburg's disease has an hyperacute onset of multifocal neurological disturbances and altered consciousness status in different degrees, causing death in almost all cases with little or no response to intensive medical treatment. We report a case of acute tumour-like demyelination encephalitis diagnosed as an acute variant of MS in which left decompressive hemicraniectomy (DHC) reversed the fulminant course of disease.

Case report

A 31-year-old right-handed woman, without any relevant pathological conditions, experienced an acute onset of vomiting, consciousness deterioration (6 points on the Glasgow Coma Scale) and right hemiparesis. Sedation and mechanical ventilation were carried out.

A CT scan revealed diffuse left frontotemporal mass effect hypodensity (figure 1A). Subsequent MRI showed left frontotemporal, middle corpus callosum and brainstem hypointensity on T1-weighted images. T2-weighted and fluid-attenuated inversion recovery sequences demonstrated a diffuse white-matter hyperintensity. Mild gadolinium contrast enhancement was observed (figure 1B). Both CT scan and MRI images did not show severe demyelination disease or involvement of brainstem structures. From the spectroscopy, the lactic acid peak explained tissue necrosis. Common biochemical laboratory tests were normal. HIV, BHV, CHV and Treponema pallidum serologies were negative. Autoimmunity tests (lupic anticoagulant, complement, reumatoid factor, ANAs, URF, AntiDNA, ANCAs) were …

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