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

Chiara Trabatti, Thomas Foiadelli, Maria Valentina Spartà, Chiara Gagliardone, Berardo Rinaldi, Maria Delmonte, Alessandro Lozza, Salvatore Savasta

Research output: Contribution to journalArticle

1 Citation (Scopus)

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.

Original languageEnglish
Pages (from-to)69-77
Number of pages9
JournalChild's Nervous System
Volume32
Issue number1
DOIs
Publication statusPublished - Jan 1 2016

Fingerprint

Acute Disseminated Encephalomyelitis
Differential Diagnosis
Pediatrics
Oligoclonal Bands
Brain Diseases
Multiple Sclerosis
Age Groups
Immunoglobulin G
Magnetic Resonance Imaging
Recurrence

Keywords

  • ADEM
  • Children
  • CIS
  • Clinically definite multiple sclerosis
  • Demyelinating diseases
  • Differential diagnosis

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Clinical Neurology

Cite this

Paediatric clinically isolated syndromes : report of seven cases, differential diagnosis and literature review. / Trabatti, Chiara; Foiadelli, Thomas; Spartà, Maria Valentina; Gagliardone, Chiara; Rinaldi, Berardo; Delmonte, Maria; Lozza, Alessandro; Savasta, Salvatore.

In: Child's Nervous System, Vol. 32, No. 1, 01.01.2016, p. 69-77.

Research output: Contribution to journalArticle

Trabatti, Chiara ; Foiadelli, Thomas ; Spartà, Maria Valentina ; Gagliardone, Chiara ; Rinaldi, Berardo ; Delmonte, Maria ; Lozza, Alessandro ; Savasta, Salvatore. / Paediatric clinically isolated syndromes : report of seven cases, differential diagnosis and literature review. In: Child's Nervous System. 2016 ; Vol. 32, No. 1. pp. 69-77.
@article{3c7f9f1a7a8c4d95bb8b134e19d04eaa,
title = "Paediatric clinically isolated syndromes: report of seven cases, differential diagnosis and literature review",
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.",
keywords = "ADEM, Children, CIS, Clinically definite multiple sclerosis, Demyelinating diseases, Differential diagnosis",
author = "Chiara Trabatti and Thomas Foiadelli and Spart{\`a}, {Maria Valentina} and Chiara Gagliardone and Berardo Rinaldi and Maria Delmonte and Alessandro Lozza and Salvatore Savasta",
year = "2016",
month = "1",
day = "1",
doi = "10.1007/s00381-015-2959-0",
language = "English",
volume = "32",
pages = "69--77",
journal = "Child's Nervous System",
issn = "0256-7040",
publisher = "Springer Verlag",
number = "1",

}

TY - JOUR

T1 - Paediatric clinically isolated syndromes

T2 - report of seven cases, differential diagnosis and literature review

AU - Trabatti, Chiara

AU - Foiadelli, Thomas

AU - Spartà, Maria Valentina

AU - Gagliardone, Chiara

AU - Rinaldi, Berardo

AU - Delmonte, Maria

AU - Lozza, Alessandro

AU - Savasta, Salvatore

PY - 2016/1/1

Y1 - 2016/1/1

N2 - 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.

AB - 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.

KW - ADEM

KW - Children

KW - CIS

KW - Clinically definite multiple sclerosis

KW - Demyelinating diseases

KW - Differential diagnosis

UR - http://www.scopus.com/inward/record.url?scp=84956930006&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=84956930006&partnerID=8YFLogxK

U2 - 10.1007/s00381-015-2959-0

DO - 10.1007/s00381-015-2959-0

M3 - Article

AN - SCOPUS:84956930006

VL - 32

SP - 69

EP - 77

JO - Child's Nervous System

JF - Child's Nervous System

SN - 0256-7040

IS - 1

ER -