Atypical Inflammatory Demyelinating Syndromes of the Central Nervous System

  • Todd A. HardyEmail author
Part of the Contemporary Clinical Neuroscience book series (CCNE)


The atypical demyelinating syndromes are a group of conditions, characterised pathologically by demyelination, that form part of the differential diagnosis of multiple sclerosis (MS) but differ from it due to variations in clinical presentation, MRI appearance, pathology, and response to treatment. The potential for some of these syndromes to overlap with conventional MS means that diagnostic uncertainties are common and therapeutic decision-making often focuses on whether to commence MS disease-modifying therapies (DMTs) or other immunosuppression. In this chapter, the spectrum of atypical demyelinating diseases is reviewed. I discuss the difficulties in diagnosing and distinguishing between conditions such as acute disseminated encephalomyelitis (ADEM), tumefactive demyelination, Baló’s concentric sclerosis, Marburg’s multiple sclerosis, and Schilder’s diffuse myelinoclastic sclerosis and contrast these conditions with conventional MS. Advances in magnetic resonance imaging (MRI) and immunobiology may prove useful in our future understanding of these conditions.


Neuroinflammation AHL Hurst’s Myelin oligodendrocyte glycoprotein MOG Haemorrhagic leukoencephalitis CNS 


Acknowledgements, Competing Interests, and Funding

TAH: Has received honoraria or travel sponsorship from Bayer-Schering, Novartis, Biogen Idec, Merck-Serono, Teva, Roche, Alexion and Genzyme.

This work received no specific grant from any funding agency in the public, commercial or not-for-profit sectors.


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Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Department of Neurology, Concord HospitalUniversity of SydneyConcordAustralia
  2. 2.Neuroimmunology Clinic, Concord HospitalUniversity of SydneySydneyAustralia
  3. 3.Brain & Mind CentreUniversity of SydneySydneyAustralia

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