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Paediatric clinically isolated syndromes: report of seven cases, differential diagnosis and literature review

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Abstract

Purpose

Few paediatric cases of clinically isolated syndrome (CIS) have been described in literature, even though it has been increasingly recognized also in this age group. Our study retrospectively enrolled seven Italian patients (four males and three females) who met the International Paediatric Multiple Sclerosis Study Group (IPMSSG) 2012 criteria for clinically isolated syndrome over the period 2010–2014; their clinical, laboratory and imaging findings were compared with current literature and with those seen in five patients (three males and two females) with acute disseminated encephalomyelitis, who were followed in our department over the same years (mean follow-up time 2.84 ± 1.8 years).

Results

In our CIS sample, male sex was prevalent, 42.8 % of patients had a multifocal presentation, MRI lesions mostly appeared confluent and with poorly defined margins, and CSF oligoclonal bands (OCBs) were identified in 28.6 %. All acute disseminated encephalomyelitis (ADEM) patients had polyfocal presentation and encephalopathy; large MRI subcortical lesions and polyclonal IgG distribution were identified. During the subsequent follow-up assessments, MRI scan revealed new lesions in three CIS patients, while in ADEM children it appeared normal.

Conclusions

Paediatric CIS patients often show peculiar epidemiological, clinical and radiological features, which significantly differ from adult ones. The presence of encephalopathy and of extended MRI lesions leads to a diagnosis of ADEM, instead. In CIS patients the presence of multiple asymptomatic MRI lesions and of OCBs revealed to be the most predictive risk factors for progression to clinically definite multiple sclerosis (CDMS), so a regular long-term follow-up is recommended; in ADEM, no suitable risk factors for a relapse could be identified.

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Acknowledgments

We are grateful to Professor Gian Luigi Marseglia, MD, Chair of the Department of Paediatrics of IRCCS “San Matteo” Hospital, Pavia, Chair of Paediatrics - University of Pavia, Medicine and Surgery Faculty, who gave formal approval for our study.

Authors’ contributions

No sponsors were involved in the study design, in the collection, analysis, and interpretation of data, in the writing of the report and in the decision to submit the paper for publication. The authors were not given any form of payment to produce the manuscript. All authors read and approved the final manuscript.

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Authors and Affiliations

  1. Department of Paediatrics, University of Pavia, IRCCS Policlinico San Matteo, viale Camillo Golgi, 19, 27100, Pavia, Italy

    Chiara Trabatti, Thomas Foiadelli, Maria Valentina Spartà, Chiara Gagliardone & Salvatore Savasta

  2. Department of Molecular Medicine, University of Pavia, Pavia, Italy

    Berardo Rinaldi

  3. Department of Radiology, Radio diagnostic service, IRCCS Policlinico San Matteo, Pavia, Italy

    Maria Delmonte

  4. Clinical Neurophysiology Service, IRCCS C. Mondino National Neurological Institute, Pavia, Italy

    Alessandro Lozza

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  1. Chiara Trabatti
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Correspondence to Chiara Trabatti.

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Trabatti, C., Foiadelli, T., Spartà, M.V. et al. Paediatric clinically isolated syndromes: report of seven cases, differential diagnosis and literature review. Childs Nerv Syst 32, 69–77 (2016). https://doi.org/10.1007/s00381-015-2959-0

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