Acute Disseminated Encephalomyelitis in Children and Adolescents: A Single Center Experience

Erol, İlknur; Özkale, Yasemin; Alkan, Özlem; Alehan, Füsun

doi:10.1016/j.pediatrneurol.2013.03.021

Cited by 52 publications

(45 citation statements)

References 33 publications

(55 reference statements)

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“…44,45 CSF protein is increased (up to 1.1 g/L) in 23%-62% of pediatric patients with ADEM. 16,20,[42][43][44] An elevated CSF IgG index has been reported in 2/54 and 3/13 children in 2 pediatric ADEM cohorts. Of note, none of these patients with elevated IgG indices had CSF oligoclonal bands (OCBs).…”

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confidence: 98%

“…CSF leukocyte count has been described to be normal in 42%-72% of children with ADEM. 16,20,[42][43][44] Pleocytosis is typically mild, with a high percentage of lymphocytes and monocytes. 44,45 CSF protein is increased (up to 1.1 g/L) in 23%-62% of pediatric patients with ADEM.…”

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confidence: 99%

“…Out of Table 3 Red flags for a diagnosis of ADEM and possible differential diagnoses 53 patients described in 3 more recent case series, OCBs were only detected in one patient (1.9%). 34,42,43 In cases where CSF OCBs are detected in ADEM, they tend to manifest as mirror bands present in both serum and CSF, and would therefore not be considered as true OCBs per definition, but suggest an antibody production that is not intrathecally restricted. 46 Infectious studies of the CSF are, by definition, negative in ADEM with the caveat that the breadth and sensitivity of current infectious assays remain limited.…”

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Acute disseminated encephalomyelitis

et al. 2016

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Acute disseminated encephalomyelitis (ADEM) is an immune-mediated demyelinating CNS disorder with predilection to early childhood. ADEM is generally considered a monophasic disease. However, recurrent ADEM has been described and defined as multiphasic disseminated encephalomyelitis. ADEM often occurs postinfectiously, although a causal relationship has never been established. ADEM and multiple sclerosis are currently viewed as distinct entities, generally distinguishable even at disease onset. However, pathologic studies have demonstrated transitional cases of yet unclear significance. ADEM is clinically defined by acute polyfocal neurologic deficits including encephalopathy. MRI typically demonstrates reversible, ill-defined white matter lesions of the brain and often also the spinal cord, along with frequent involvement of thalami and basal ganglia. CSF analysis may reveal a mild pleocytosis and elevated protein, but is generally negative for intrathecal oligoclonal immunoglobulin G synthesis. In the absence of a specific diagnostic test, ADEM is considered a diagnosis of exclusion, and ADEM mimics, especially those requiring a different treatment approach, have to be carefully ruled out. The role of biomarkers, including autoantibodies like antimyelin oligodendrocyte glycoprotein, in the pathogenesis and diagnosis of ADEM is currently under debate. Based on the presumed autoimmune etiology of ADEM, the current treatment approach consists of early immunotherapy. Outcome of ADEM in pediatric patients is generally favorable, but cognitive deficits have been reported even in the absence of other neurologic sequelae. This review summarizes the current knowledge on epidemiology, pathology, clinical presentation, neuroimaging features, CSF findings, differential diagnosis, therapy, and outcome, with a focus on recent advances and controversies. Neurology ® 2016;87 (Suppl 2):S38-S45 GLOSSARY ADEM 5 acute disseminated encephalomyelitis; ADEM-ON 5 acute disseminated encephalomyelitis followed by optic neuritis; AHL 5 acute hemorrhagic leukoencephalopathy; IgG 5 immunoglobulin G; IPMSSG 5 International Pediatric Multiple Sclerosis Study Group; MDEM 5 multiphasic disseminated encephalomyelitis; MOG 5 myelin oligodendrocyte glycoprotein; MS 5 multiple sclerosis; NMOSD 5 neuromyelitis optica spectrum disorders; OCB 5 oligoclonal band.Acute disseminated encephalomyelitis (ADEM) is an immune-mediated demyelinating CNS disorder, characterized clinically by new-onset polyfocal neurologic symptoms including encephalopathy, coupled with neuroimaging evidence of multifocal demyelination. ADEM is classically considered a monophasic illness, with highest incidence in early childhood. The first descriptions of an ADEM-like disorder with recognition of a temporal relationship to infections (especially smallpox and measles) date back to the 18th century.1 Over a century later, an association of ADEM with vaccines, notably rabies, was reported. Mortality rates were high (up to 30% for ADEM following measles infection), and neur...

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Acute disseminated encephalomyelitis

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“…Its use on relapse patients or patients with no improvement after a course of steroids has been described. 12,13 In this patient, the resolution of the infection/condition was complete with the use of steroids. Studies on children with ADEM show that two-thirds recover completely over the course of several weeks.…”

Section: Discussionmentioning

confidence: 75%

Madariaga Virus Infection Associated with a Case of Acute Disseminated Encephalomyelitis

Luciani¹,

Abadía²,

Martínez-Torres³

et al. 2015

The American Society of Tropical Medicine and Hygiene

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Abstract. We present the first report of a pediatric case of acute disseminated encephalomyelitis (ADEM) associated with Madariaga virus infection (MADV, Alphavirus, Togaviridae; formerly known as South American variants of eastern equine encephalitis virus [EEEV]) in a patient of the 2010 alphaviral epidemic reported in Panama. The patient was admitted to the Hospital del Niñ o in Panama City with suspected meningitis, exhibited with decreased alertness and disorientation in space and time, hemiparesis, and left Babinski sign. The patient was transferred to the intensive care unit and treated with aciclovir and methylprednisolone. The magnetic resonance imaging (MRI) of the brain revealed multiple hyperintense lesions at T 2 -weighted images (T2) and fluid-attenuated inversion recovery (FLAIR) on the cortical-subcortical level. Sera samples obtained on days 6 and 12 were immunoglobulin M (IgM) positive for MADV. The findings on the clinical and cerebrospinal analyses, rapid symptom progression as well as neuroimaging, and serologic studies support our diagnosis. Our results suggest that MADV should be included in the etiologic differential diagnosis of ADEM in endemic countries.

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“…The efficacy of IVIG in 30 paediatric patients with ADEM was described in seven series (Table SIII) . Outcome measures were recovery and relapse rates.…”

Section: Resultsmentioning

confidence: 99%