Clinically isolated syndrome (CIS) patients present with a single attack of inflammatory demyelination of the central nervous system. Recent advances in multiple sclerosis (MS) diagnostic criteria have expanded the number of CIS patients eligible for a diagnosis of MS at the onset of the disease, shrinking the prevalence of CIS. MS treatment options are rapidly expanding, which is driving the need to recognise MS at its earliest stages. In CIS patients, finding typical MS white matter lesions on the patient’s MRI scan remains the most influential prognostic investigation for predicting subsequent diagnosis with MS. Additional imaging, cerebrospinal fluid and serum testing, information from the clinical history and genetic testing also contribute. For those subsequently diagnosed with MS, there is a wide spectrum of long-term clinical outcomes. Detailed assessment at the point of presentation with CIS provides fewer clues to calculate a personalised risk of long-term severe disability.
Clinicians should select suitable CIS cases for steroid treatment to speed neurological recovery. Unfortunately, there are still no neuroprotection or remyelination strategies available. The use of MS disease modifying therapy for CIS varies among clinicians and national guidelines, suggesting a lack of robust evidence to guide practice. Clinicians should focus on confirming MS speedily and accurately with appropriate investigations. Diagnosis with CIS provides an opportune moment to promote a healthy lifestyle, in particular smoking cessation. Patients also need to understand the link between CIS and MS. This review provides clinicians an update on the contemporary evidence guiding prognostication and management of CIS.