Acute Disseminated Encephalomyelitis: A Long-Term Prospective Study and Meta-Analysis

Pavone, Piero; Pettoello-Mantovano, M.; Pira, Alice Le; Giardino, Ida; Pulvirenti, Alfredo; Giugno, Rosalba; Parano, Enrico; Polizzi, Agata; Distefano, Alfio; Ferro, Anabela; Pavone, Lorenzo; Ruggieri, Martino

doi:10.1055/s-0031-1271656

Cited by 81 publications

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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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“…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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“…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

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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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“…Toward the end of his life, he had violent paroxysms of pain that were preceded by a disagreeable smell and accompanied by facial twitching [56,57,62]. In his terminal event, he became delirious [55,62] and was attended to by Wishart. An attempt to resect the lesion was unsuccessful and the patient died from a wound infection [1,97]: I attempted to bleed him from the arm; but, from his restlessness, the blood could not be made to flow.…”

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Early history of neurofibromatosis type 2 and related forms: earliest descriptions of acoustic neuromas, medical curiosities, misconceptions, landmarks and the pioneers behind the eponyms

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“…Some publications suggest a prevalence rate of 0.8 per 100,000 affected patients per year (Leake et al, 2004). Whereas some reports describe no gender predisposition (Dale et al, 2000;Leake et al, 2004), most studies indicate a slight male preponderance in ADEM patients (Pavone et al, 2010). Although the majority of patients with ADEM follow a monophasic disease course, recently recurrent or multiphasic forms have been described with a lower incidence rate (Rust, 2000;Hynson et al, 2001).…”

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Relevance of Autoantibodies for the Classification and Pathogenesis of Neurological Diseases

Mader¹,

Obholzer²,

Reindl³

2011

Autoimmune Disorders - Current Concepts and Advances From Bedside to Mechanistic Insights

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Acute Disseminated Encephalomyelitis: A Long-Term Prospective Study and Meta-Analysis

Abstract: ADEM is generally a benign condition that mosly affects boys more than girls and rarely recurs.

Cited by 81 publications

References 18 publications

Acute disseminated encephalomyelitis

Acute disseminated encephalomyelitis

Early history of neurofibromatosis type 2 and related forms: earliest descriptions of acoustic neuromas, medical curiosities, misconceptions, landmarks and the pioneers behind the eponyms

Relevance of Autoantibodies for the Classification and Pathogenesis of Neurological Diseases

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