, Volume 49, Issue 5, pp 393–409 | Cite as

Idiopathic inflammatory-demyelinating diseases of the central nervous system

  • A. Rovira CañellasEmail author
  • A. Rovira Gols
  • J. Río Izquierdo
  • M. Tintoré Subirana
  • X. Montalban Gairin


Idiopathic inflammatory-demyelinating diseases (IIDDs) include a broad spectrum of central nervous system disorders that can usually be differentiated on the basis of clinical, imaging, laboratory and pathological findings. However, there can be a considerable overlap between at least some of these disorders, leading to misdiagnoses or diagnostic uncertainty. The relapsing-remitting and secondary progressive forms of multiple sclerosis (MS) are the most common IIDDs. Other MS phenotypes include those with a progressive course from onset (primary progressive and progressive relapsing) or with a benign course continuing for years after onset (benign MS). Uncommon forms of IIDDs can be classified clinically into: (1) fulminant or acute IIDDs, such as the Marburg variant of MS, Baló’s concentric sclerosis, Schilder’s disease, and acute disseminated encephalomyelitis; (2) monosymptomatic IIDDs, such as those involving the spinal cord (transverse myelitis), optic nerve (optic neuritis) or brainstem and cerebellum; and (3) IIDDs with a restricted topographical distribution, including Devic’s neuromyelitis optica, recurrent optic neuritis and relapsing transverse myelitis. Other forms of IIDD, which are classified clinically and radiologically as pseudotumoral, can have different forms of presentation and clinical courses. Although some of these uncommon IIDDs are variants of MS, others probably correspond to different entities. MR imaging of the brain and spine is the imaging technique of choice for diagnosing these disorders, and together with the clinical and laboratory findings can accurately classify them. Precise classification of these disorders may have relevant prognostic and treatment implications, and might be helpful in distinguishing them from tumoral or infectious lesions, avoiding unnecessary aggressive diagnostic or therapeutic procedures.


Multiple sclerosis Magnetic resonance imaging Brain diseases 



The authors thank Celine L. Cavallo for English language support.

Conflict of interest statement

We declare that we have no conflict of interest.


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Copyright information

© Springer-Verlag 2007

Authors and Affiliations

  • A. Rovira Cañellas
    • 1
    Email author
  • A. Rovira Gols
    • 2
  • J. Río Izquierdo
    • 3
  • M. Tintoré Subirana
    • 3
  • X. Montalban Gairin
    • 3
  1. 1.Magnetic Resonance Unit (I.D.I.), Department of RadiologyVall d’Hebron University HospitalBarcelonaSpain
  2. 2.UDIAT, Diagnostic CentreParc Taulí University Institute - UABSabadellSpain
  3. 3.Neuroimmunology Unit, Department of NeurologyVall d’Hebron University HospitalBarcelonaSpain

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