Consensus definitions proposed for pediatric multiple sclerosis and related disorders

Abstract

CNS inflammatory demyelinating disorders presenting in children and adolescents can be defined and distinguished. However, prospective research is necessary to determine the validity and utility of the proposed diagnostic categories.

Diagnosing paediatric multiple sclerosis versus acute disseminated encephalomyelitis

“…Only very recently, an international panel has outlined diagnostic criteria for paediatric MS, ADEM, and related disorders. 3 In these definitions, ADEM is characterized as a transitory and self-limiting, (sub)acute multifocal inflammatory demyelinating process accompanied by encephalopathy as a clinical conditio sine qua non. The diagnostic criteria for paediatric MS are similar to those applied for adult MS patients, 2 with the important addendum that in a child whose initial attack was diagnosed as ADEM, a second non-ADEM demyelinating event alone is not sufficient for the diagnosis of MS.…”

Acute disseminated encephalomyelitis following allo-SCT: central nervous system manifestation of GVHD

“…Diagnosis is on the basis of a constellation of clinical and radiological features ( Table 1). 1 The principal differential diagnoses include multiple sclerosis, characterized by recurrent episodes of CNS demyelination, optic neuritis, transverse myelitis and neuromyelitis optica. ADEM is often preceded by infection (occasionally immunisation), typically 1 month before neurological dysfunction.…”

Acute disseminated encephalomyelitis: clinical features, HLA DRB1*1501, HLA DRB1*1503, HLA DQA1*0102, HLA DQB1*0602, and HLA DPA1*0301 allelic association study

“…ADEM can progress with recurrent manifestation of multiple functional neurological systems, similar to multiple sclerosis (MS), and, in these cases, it is defined as multiphasic encephalomyelitis (MDEM) 5,9 . When the same functional neurological systems are involved, characterizing a stereotypic recurrent neurological deficit identical of the first demyelinating event, it is denominated recurrent encephalomyelitis (RDEM) 5,[9][10][11][12] .Post-infectious forms of encephalomyelitis, also termed ADEM compose one of the several categories of inflammatory demyelinating disorders (IDD) of the CNS 13,14 . Recently, new criteria for pediatric population were proposed 10 .…”

“…When the same functional neurological systems are involved, characterizing a stereotypic recurrent neurological deficit identical of the first demyelinating event, it is denominated recurrent encephalomyelitis (RDEM) 5,[9][10][11][12] .Post-infectious forms of encephalomyelitis, also termed ADEM compose one of the several categories of inflammatory demyelinating disorders (IDD) of the CNS 13,14 . Recently, new criteria for pediatric population were proposed 10 . Pediatric monophasic ADEM includes obligatorily encephalopathy associated to multifocal areas of CNS demyelinating or inflammatory origin, after exclusion of any other cause [10][11][12] and neuroimaging showing focal or multifocal lesions predominantly involving white matter, without evidence of previous lesions 10 .…”

“…Recently, new criteria for pediatric population were proposed 10 . Pediatric monophasic ADEM includes obligatorily encephalopathy associated to multifocal areas of CNS demyelinating or inflammatory origin, after exclusion of any other cause [10][11][12] and neuroimaging showing focal or multifocal lesions predominantly involving white matter, without evidence of previous lesions 10 . The pediatric RDEM is defined as a new event of ADEM, with recurrence of the same initial signs and symptoms, always associated to encephalopathy; magnetic resonance image (MRI) shows no new lesion and the original ones can have enlarged, during a interval time of three months, or more, of the remission, and at least one month after the last treatment with corticosteroids [10][11][12] .…”