Life-saving decompressive craniectomy for acute disseminated encephalomyelitis in a child: a case report

Granget, E; Milh, Mathieu; Pech-Gourg, G.; Paut, O.; Girard, Nadine; Léna, G.; Scavarda, Didier

doi:10.1007/s00381-012-1733-9

Cited by 21 publications

(17 citation statements)

References 23 publications

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“…47 In single case reports, patients with fulminant ADEM and cerebral edema have been treated with hypothermia or decompressive craniotomy. 52,53 OUTCOME The majority of children with ADEM are reported to have full recovery. Typically, neurologic improvement is seen within days following initiation of treatment, and recovery to baseline will occur within weeks rather than months.…”

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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“…Several other case reports suggest that craniectomy can be life-saving in severe, fulminant ADEM and provide good long-term recovery. 4–7…”

Section: Discussionmentioning

confidence: 99%

Craniectomy for acute disseminated encephalomyelitis

Bourke

Woon

2020

Pract Neurol

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“…Particularly severe cases, e.g., AHLE or refractory ADEM, should also be treated with early plasma exchange, 5 to 7 treatment courses [ 79 ]. Therapeutic hypothermia and decompressive craniectomy have been reported in the literature as techniques to manage severe edema [ 80 , 81 ].…”

Section: Postviral Central Nervous System Inflammatory Diseasementioning

confidence: 99%

Inflammatory Manifestations of Systemic Diseases in the Central Nervous System

Lapides

McDonald

2020

Curr Treat Options Neurol

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Purpose of Review This review presents the current recommended therapeutic interventions for inflammatory disease in the central nervous system (CNS) secondary to systemic diseases of immune dysregulation. Treatment recommendations for CNS inflammation associated with rheumatologic conditions, immune-related adverse effects from immune checkpoint inhibitors (ICIs), and demyelinating disease from tumor necrosis factor-α (anti-TNFs) are explored. Additional therapeutic options for inflammation related to postviral syndromes and genetic immunodeficiencies are also discussed. Recent Findings In addition to treatment of mild, moderate, and severe CNS rheumatologic disease as guided by the European League Against Rheumatism (EULAR), early consideration of rituximab for severe IgG4-related disease and induction with anti-TNF therapy for severe neurosarcoidosis should be considered. Although often not first line, treatment options for CNS inflammatory diseases based on disease mechanism are emerging, including tocilizumab for Behcet's disease, natalizumab for ICI associated autoimmune encephalitis, and abatacept for treatment of infiltrative disease secondary to CTLA-4 deficiency. Hematopoietic stem cell treatments represent highly efficacious but risky options for autoimmunity related to genetic immunodeficiency. Summary While early high dose steroids remains first line therapy for most CNS inflammatory conditions, a rapidly expanding arsenal of immune targeted therapies offers clinicians tailored disease specific options for treatment.

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Life-saving decompressive craniectomy for acute disseminated encephalomyelitis in a child: a case report

Cited by 21 publications

References 23 publications

Acute disseminated encephalomyelitis

Acute disseminated encephalomyelitis

Craniectomy for acute disseminated encephalomyelitis

Inflammatory Manifestations of Systemic Diseases in the Central Nervous System

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