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Clinically isolated syndromes and the relationship to multiple sclerosis

Wallace J. Brownlee, David H. Miller

Journal of Clinical Neuroscience, Available online 11 July 2014

The most common presentation of multiple sclerosis (MS) is with a clinically isolated syndrome (CIS) affecting the optic nerves, brainstem or spinal cord. Two thirds of patients with CIS will have further episodes of neurological dysfunction and convert to relapsing-remitting MS, while the remaining patients have a monophasic illness, at least clinically. Abnormalities on a baseline MRI scan predict the subsequent development of MS in patients with CIS. In the long term, about 80% of patients with an abnormal MRI convert to MS compared with 20% with a normal MRI. For patients who develop MS the long term prognosis is varied. After 20 years, almost half will have developed secondary progressive MS, while around one third have a benign disease course with little physical disability. Disease-modifying treatments delay conversion to MS in selected CIS patients with abnormal MRI but an effect on long term disability has not been demonstrated. In this review we discuss recent advances in the diagnosis, management and prognostication of patients with CIS.

From Dr. Claire Riley: The shrinking diagnostic category of clinically isolated syndrome is reviewed in detail by Professors Miller and Brownlee, contextualizing the shift in diagnostic criteria in 2010.  The differences in prescribing practices in the UK and US are highlighted.  The authors describe the need for accurate biomarkers in order to distinguish among CIS patients, and identify which patients would most benefit from treatment with disease modifying therapy.  As pressure on health care spending increases, these biomarkers may become critical in determining which patients merit treatment.  MRI metrics such as deep grey matter atrophy or cortical lesions may prove valuable in identifying those CIS patients likely to go on to develop disability from the disease.

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About the Editors

  • Prof Timothy Vartanian

    Timothy Vartanian, Professor at the Brain and Mind Research Institute and the Department of Neurology, Weill Cornell Medical College, Cornell...
  • Dr Claire S. Riley

    Claire S. Riley, MD is an assistant attending neurologist and assistant professor of neurology in the Neurological Institute, Columbia University,...
  • Dr Rebecca Farber

    Rebecca Farber, MD is an attending neurologist and assistant professor of neurology at the Neurological Institute, Columbia University, in New...

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