Intravenous immunoglobulin therapy in acute disseminated encephalomyelitis

Pradhan, Sunil; Gupta, Rameshwar P.; Shashank, Sneh; Pandey, Nirmal

doi:10.1016/s0022-510x(99)00072-6

Cited by 139 publications

(65 citation statements)

References 20 publications

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“…49 IV immunoglobulin treatment has been described in case reports and small case series, mostly in combination with corticosteroids or as a second-line treatment in steroid-unresponsive ADEM. 50,51 The usual total dose is 2 g/kg, administered over 2-5 days. 47 Plasma exchange is recommended for therapy-refractory patients with fulminant disease, e.g., using 7 exchanges every other day.…”

Section: Differential Diagnosis Investigationsmentioning

confidence: 99%

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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Section: Differential Diagnosis Investigationsmentioning

confidence: 99%

Acute disseminated encephalomyelitis

et al. 2016

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“…IV immunoglobulins may block the Fc-receptors on macrophages rendering them less auto-aggressive for cell-mediated immune response [16,17], and, furthermore, they may neutralize activated complements preventing immune complexes from damaging oligodendrocytes and myelin [18,19]. Appreciable clinical improvement in patients with ADEM treated with IV immunoglobulins was noted after 2-4 days of therapy [20].…”

Section: Discussionmentioning

confidence: 99%

Combination regimen of methylprednisolone, IV immunoglobulin, and plasmapheresis early in the treatment of acute disseminated encephalomyelitis

Keilson

2006

J. Clin. Apheresis

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Acute disseminated encephalomyelitis (ADEM) is a demyelinating disease of the central nervous system that is associated with significant morbidity and mortality. Early recognition of the disease is of paramount importance; however, treatment options are limited because only case reports and small series are available in the literature. We report a case of a 42 year-old previously healthy man, whom we treated successfully with a combination regimen of methylprednisolone, IV immunoglobulin, and plasmapheresis early in the course of the disease.

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“…64 Another observational study on 4 pediatric patients affected by corticosteroidresistant ADEM (with no improvement after receiving a 3-5 day course of high dose intravenous methylprednisolone) showed rapid improvement after administration of IVIG. 65 On a review of 20 pediatric cases of monophasic ADEM, 70% have shown complete recovery after IVIG or IVIG plus steroids. In the 5 case reports with recurrent ADEM, 2 were completely recovered after IVIG.…”

Section: Immunotherapeutic Approaches Of Chorea As Manifestation Of Omentioning

confidence: 99%