Role of MRI in the differentiation of ADEM from MS in children

Callen, David; Shroff, Manohar; Branson, Helen M.; Li, D. K.; Lotze, Tim; Stephens, DH; Banwell, Brenda

doi:10.1212/01.wnl.0000338630.20412.45

Cited by 220 publications

(138 citation statements)

References 14 publications

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“…Imaging abnormalities frequently resolve as the patient sustains clinical recovery [8,15,17,18]. Monophasic ADEM, at times, may be difficult to distinguish from the first attack of MS; however, MRI findings of diffuse, bilateral T2-hyperintense lesions in addition to absence of T1-hypointense Bblack holes^and lack of periventricular lesions weigh heavily in favor of a diagnosis of ADEM [23,24].…”

Section: Ademmentioning

confidence: 99%

Therapeutic Approach to the Management of Pediatric Demyelinating Disease: Multiple Sclerosis and Acute Disseminated Encephalomyelitis

Brenton

Banwell

2016

Neurotherapeutics

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Acquired pediatric demyelinating diseases manifest acutely with optic neuritis, transverse myelitis, acute disseminated encephalomyelitis, or with various other acute deficits in focal or polyfocal areas of the central nervous system. Patients may experience a monophasic illness (as in the case of acute disseminated encephalomyelitis) or one that may manifest as a chronic, relapsing disease [e.g., multiple sclerosis (MS)]. The diagnosis of pediatric MS and other demyelinating disorders of childhood has been facilitated by consensus statements regarding diagnostic definitions. Treatment of pediatric MS has been modeled after data obtained from clinical trials in adult-onset MS. There are now an increasing number of new therapeutic agents for MS, and many will be formally studied for use in pediatric patients. There are important efficacy and safety concerns regarding the use of these therapies in children and young adults. This review will discuss acute management as well as chronic immunotherapies in acquired pediatric demyelination.

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Section: Ademmentioning

confidence: 99%

Therapeutic Approach to the Management of Pediatric Demyelinating Disease: Multiple Sclerosis and Acute Disseminated Encephalomyelitis

Brenton

Banwell

2016

Neurotherapeutics

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“…Two of these 3 criteria were described to be sensitive for distinguishing MS from ADEM. 1 Subsequently, a large population-based study revealed that the highest odds ratio for subsequent MS diagnosis was the presence of both 1 periventricular T2-hyperintense lesion and at least 1 T1-hypointense lesion.…”

Section: Role Of Mri In Ms Diagnosismentioning

confidence: 99%

MRI in the evaluation of pediatric multiple sclerosis

et al. 2016

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MRI plays a pivotal role in the diagnosis of multiple sclerosis (MS) in children, as it does in adults. The presence of multiple lesions in CNS locations commonly affected by MS, along with the presence of both enhancing and nonenhancing lesions, can facilitate a diagnosis of MS at the time of a first attack, whereas the accrual of serial lesions or new clinical attacks over time confirms the diagnosis in patients not meeting such criteria at onset. T2 and enhancing lesion accrual could serve as a primary outcome metric for pediatric MS clinical trials of selected therapies with anti-inflammatory activity in order to facilitate feasible trial size numbers. More-advanced MRI techniques reveal the impact of MS on tissue integrity within both T2-bright and T1-hypointense lesions and regions of normal-appearing tissue. Volumetric MRI analyses quantify the impact of MS on age-expected brain growth, and fMRI reveals activation and resting-state functional connectivity patterns in patients with pediatric MS that differ from those seen in healthy age-matched youth. Such studies are of critical importance because MS onset during childhood may profoundly influence maturing and actively myelinating neural networks. Highfield MRI visualizes MS pathology at a near-microscopic level and has the potential to more fully explain mechanisms for cognitive impairment, fatigue, and disability in patients with pediatric MS. Multiple sclerosis (MS) in childhood and adolescence is characterized by both clinical and MRI evidence of highly active inflammation. Although many aspects of relapsing-remitting MS (RRMS) are similar in both pediatric-and adult-onset disease, very young pediatric patients have certain unique features. Disorders to consider in the differential diagnosis of MS include non-MS inflammatory diseases, CNS involvement in systemic inflammatory disorders, and genetic and metabolic disorders of white matter, some of which have an associated inflammatory response. MRI forms a key component in evaluation. Pediatric-onset MS also provides a window into understanding the earliest biological aspects of MS. The disease has a great impact on brain integrity, as evidenced by MRI metrics that quantify disruption of tissue microstructure as well as loss of age-expected brain volume. The present article focuses on recent advances in neuroimaging in pediatric MS, with practical considerations for the use of MRI in diagnosis, monitoring of clinical course, and response to therapy. Potential advanced imaging techniques to further understand the impact of the disease on the ongoing maturing CNS are discussed, as is the utility of MRI as an outcome measure for clinical trials in pediatric MS. ROLE OF MRI IN MS DIAGNOSISConsiderable recent effort has focused on the contributions of MRI for distinguishing children with MS from children with monophasic demyelination. Other articles in this supplement provide details regarding the clinical features of incident demyelination, including acute disseminated From The Children's

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“…The French KIDMUS study showed that the presence of lesions perpendicular to the long axis of the corpus callosum and the sole presence of well-defined lesions had a sensitivity of 21%, specificity of 100%, positive predictive value (PPV) of 100%, and negative predictive value (NPV) of 61% for identification of MS. 17 The Callen et al 18 MS vs ADEM criteria demonstrated that the presence of 2 out of 3 of (1) absence of diffuse bilateral lesion pattern, (2) presence of black holes, or (3) presence of 2 or more lesions with periventricular lesions yielded a sensitivity of 81%, specificity of 95%, PPV of 95%, and NPV of 79% of identifying a child as having MS rather than ADEM. Verhey et al 19 demonstrated that the presence of $1 periventricular T2 bright lesion and $1 T1-hypointense lesion had a sensitivity of 84%, specificity of 93%, PPV of 76%, and NPV of 96% of identifying children with MS as compared to children with monophasic ADS.…”

Section: Mri Abnormalities Associated With Msmentioning

confidence: 99%

Pediatric acquired CNS demyelinating syndromes

et al. 2016

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Approximately one-third of children with an acquired demyelinating syndrome (ADS) will be diagnosed with multiple sclerosis (MS), either at onset according to the 2010 McDonald criteria, or on the basis of clinical or MRI evidence of relapsing disease, in the majority of patients within 2-4 years. ADS in adolescents, female patients, and patients with polyfocal deficits is associated with the highest likelihood of MS, while children with acute disseminated encephalomyelitis, those with documented preceding infection, and ADS presentation in young children more commonly portends a monophasic outcome. While pediatric MS associates with similar genetic risk alleles as have been documented in adult-onset MS, such associations are not diagnostically valuable at the individual level. The presence of antibodies directed against aquaporin-4 strongly supports a diagnosis of neuromyelitis optica, and should be assayed in children manifesting with severe optic neuritis, longitudinally extensive myelitis, or brainstem/hypothalamic syndromes. Further research will determine whether other antibody signatures are indicative of relapsing demyelination distinct from MS. Neurology ® 2016;87 (Suppl 2):S67-S73 GLOSSARY ADEM 5 acute disseminated encephalomyelitis; ADS 5 acquired demyelinating syndromes; CI 5 confidence interval; CIS 5 clinically isolated syndrome; HLA 5 human leukocyte antigen; HR 5 hazard ratio; MOG 5 myelin oligodendrocyte glycoprotein; MS 5 multiple sclerosis; NMO 5 neuromyelitis optica; NPV 5 negative predictive value; OCB 5 oligoclonal band; ON 5 optic neuritis; OR 5 odds ratio; PPV 5 positive predictive value; SNP 5 single nucleotide polymorphism; TM 5 transverse myelitis.

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Role of MRI in the differentiation of ADEM from MS in children

Abstract: MRI diagnostic criteria are proposed that may be useful in differentiating children experiencing the first attack of multiple sclerosis from those with monophasic acute disseminated encephalomyelitis.

Cited by 220 publications

References 14 publications

Therapeutic Approach to the Management of Pediatric Demyelinating Disease: Multiple Sclerosis and Acute Disseminated Encephalomyelitis

Therapeutic Approach to the Management of Pediatric Demyelinating Disease: Multiple Sclerosis and Acute Disseminated Encephalomyelitis

MRI in the evaluation of pediatric multiple sclerosis

Pediatric acquired CNS demyelinating syndromes

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