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  • Review Article
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Unanticipated demyelinating pathology of the CNS

Nature Reviews Neurology volume 5, pages 591–597 (2009)Cite this article

Abstract

Incidentally identified anomalies within the CNS that are highly suggestive of demyelinating disease have been extensively described in neuropathological series, and are increasingly being detected during premortem investigations. With the exception of studies that focused specifically on the prevalence of this entity, the observed anomalies are unanticipated and unrelated to the intended purpose of the examination. The discovery of MRI technology, and its subsequent widespread adoption in the clinic, has facilitated the identification of such cases. The natural course of individuals with incidentally identified demyelinating anomalies is unknown at present. This Review focuses on the history and nosology of unexpected demyelinating pathology, encompassing both autopsy data and MRI-based antemortem investigations of large cohorts, as well as family members at high risk of developing multiple sclerosis. Longitudinal clinical data acquired from prospectively followed cohorts will also be reviewed. In addition, I discuss the estimated prevalence of demyelinating pathology, the currently proposed criteria for its identification, implications for therapeutic intervention, and predictors of disease progression.

Key Points

  • Incidentally identified demyelinating anomalies within the CNS that are highly suggestive of multiple sclerosis (MS) have been extensively described in neuropathological studies

  • The widespread utilization of MRI technology in medicine is facilitating the identification of individuals possessing premortem anomalies suggestive of MS

  • Longitudinal clinical data acquired from prospectively followed cohorts with suspected demyelinating anomalies affirm the importance of these observed radiological findings

  • The discovery of demyelinating pathology creates intersecting neuroethical, legal, social and practical medical management quandaries and is, therefore, of both immediate and long-term clinical relevance

  • Additional investigations are required to obtain a fuller understanding of the natural history of incidentally identified demyelinating anomalies so that guidelines for surveillance and treatment recommendations can be formulated

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Figure 1: Incidentally identified structural neuroimaging findings highly suggestive of demyelinating disease in two individuals.
Figure 2: The demyelinating disease spectrum.

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Authors and Affiliations

  1. Department of Neurology, UCSF Multiple Sclerosis Center, University of Caifornia, San Francisco, San Francisco, CA, USA

    Darin T. Okuda

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  1. Darin T. Okuda
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Correspondence to Darin T. Okuda.

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The author declares no competing financial interests.

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Okuda, D. Unanticipated demyelinating pathology of the CNS. Nat Rev Neurol 5, 591–597 (2009). https://doi.org/10.1038/nrneurol.2009.157

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