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Neurological Sciences

, Volume 30, Issue 5, pp 421–430 | Cite as

Schilder’s disease: non-invasive diagnosis?

A case report and review
  • Susanna Bacigaluppi
  • Gabriele Polonara
  • Mario L. Zavanone
  • Rolando Campanella
  • Vincenzo Branca
  • Sergio M. Gaini
  • Giovanni Tredici
  • Antonella Costa
Case Report

Abstract

Schilder’s disease, or myelinoclastic diffuse sclerosis, is a rare disorder characterised by an inflammatory white matter plaque of demyelination. Clinical signs and symptoms might be atypical for early multiple sclerosis and at imaging the lesion is easily taken for a brain tumour. Regardless of the use of Poser’s criteria for clinical diagnosis of Schilder’s disease proposed in 1986, diagnostic difficulties are still present, as evidenced by the many reported cases in the English literature revised (Pubmed indexed, period 1998–2008). It clearly emerges that neuroradiological features, observable in additional magnetic resonance sequences are crucial, besides the consideration of Poser’s criteria, in differentiating between demyelinating lesions and brain tumours. A 29-year-old female patient is presented, where a careful evaluation of both the clinical and radiological features, which might have been at a first glance misleadingly suggestive for a brain tumour, allowed non-invasive diagnosis of Schilder’s disease.

Keywords

Schilder’s disease Myelinoclastic diffuse sclerosis Brain tumour Magnetic resonance (MR) MR spectroscopy (MRS) Non-invasive diagnosis 

Notes

Acknowledgments

Thanks to Marco Bacigaluppi M.D. for insightful advice and suggestions during the development of this paper and thanks all colleagues who have contributed to the care of our patient.

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Copyright information

© Springer-Verlag 2009

Authors and Affiliations

  • Susanna Bacigaluppi
    • 1
    • 4
  • Gabriele Polonara
    • 3
  • Mario L. Zavanone
    • 1
  • Rolando Campanella
    • 1
  • Vincenzo Branca
    • 2
  • Sergio M. Gaini
    • 1
  • Giovanni Tredici
    • 4
  • Antonella Costa
    • 2
  1. 1.Department of NeurosurgeryUniversità degli Studi di Milano, Ospedale Policlinico Mangiagalli e Regina Elena, Fondazione I.R.C.C.S.MilanItaly
  2. 2.Department of NeuroradiologyUniversità degli Studi di Milano, Ospedale Policlinico Mangiagalli e Regina Elena, Fondazione I.R.C.C.S.MilanItaly
  3. 3.Department of NeuroradiologyUniversità Politecnica delle MarcheAnconaItaly
  4. 4.Department of Neurosciences and Biomedical TechnologiesUniversità degli Studi di Milano BicoccaMonzaItaly

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