Therapeutic Hypothermia With the Use of Intracranial Pressure Monitoring for Acute Disseminated Encephalomyelitis With Brainstem Lesion

Miyamoto, Kenji; Kozu, Seiki; Arakawa, Akiko; Tsuboi, Tatsuo; Hirao, Jun; Ono, Katsushige; Arisaka, Osamu

doi:10.1177/0883073813501874

Cited by 8 publications

(4 citation statements)

References 18 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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“…A seasonal variation of ADEM frequency (with peaks in winter and spring) supports its infectious etiology [10,15]. The most frequent infections involved are viral and related to the upper respiratory tract, such as measles [14,16], mumps [2,[17][18][19], rubella [2], varicella [2,14], influenza [5,20], and infectious mononucleosis [21,22]. Also enterovirus [23], coronavirus [24], human immunodeficiency virus [2], herpes simplex virus [25], cytomegalovirus [22], and hepatitis A virus [26,27] have been associated with ADEM.…”

Section: A Complex Bind Of Infection-triggered Autoimmune Phenomena Amentioning

confidence: 99%

“…Its first expression is characterized by acute onset of different neurological signs and symptoms, accompanied by encephalopathy with a monophasic course, often resolving after treatment within three months since its onset, though relapses might occur in 20-30% of cases [4]. In the case of a prompt diagnosis and adequate therapeutic support, most children Autoimmunity Reviews 14 (2015) [923][924][925][926][927][928][929] with ADEM have a favorable outcome with full recovery, but in the case of diagnostic delays or in the case of inappropriate treatment some children might develop neurological sequelae or persistent deficits and even show a dramatic evolution to multiple sclerosis (MS) [5][6][7]. However, due to the absence of pertinent biological markers, diagnosis of ADEM could be unfocused and, because of uncertainties on etiopathogenesis, an appropriate therapy might be established with delay.…”

Section: Introductionmentioning

confidence: 99%

A spectrum of inflammation and demyelination in acute disseminated encephalomyelitis (ADEM) of children

Esposito

Pietro

Madini

et al. 2015

Autoimmunity Reviews

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Acute disseminated encephalomyelitis (ADEM) is an inflammatory demyelinating disease of the central nervous system that involves multifocal areas of the white matter, rarely the gray matter and spinal cord, mainly affecting children and mostly occurring 1-2weeks after infections or more rarely after vaccinations. Though a specific etiologic agent is not constantly identified, to evaluate carefully patient's clinical history and obtain adequate samples for the search of a potential ADEM causal agent is crucial. In the case of a prompt diagnosis and adequate treatment, most children with ADEM have a favorable outcome with full recovery, but in the case of diagnostic delays or inappropriate treatment some patients might display neurological sequelae and persistent deficits or even show an evolution to multiple sclerosis. The suspicion of ADEM rises on a clinical basis and derives from systemic and neurologic signs combined with magnetic resonance imaging of the central nervous system. Other advanced imaging techniques may help an appropriate differential diagnosis and definition of exact disease extension. Although there is no standardized protocol or management for ADEM, corticosteroids, intravenous immunoglobulin, and plasmapheresis have been successfully used. There is no marker that permits to identify the subset of children with worse prognosis and future studies should try to detect any biological clue for prevision of neurologic damage as well as should optimize treatment strategies using an approach based on the effective risk of negative evolution.

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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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Therapeutic Hypothermia With the Use of Intracranial Pressure Monitoring for Acute Disseminated Encephalomyelitis With Brainstem Lesion

Cited by 8 publications

References 18 publications

Acute disseminated encephalomyelitis

Acute disseminated encephalomyelitis

A spectrum of inflammation and demyelination in acute disseminated encephalomyelitis (ADEM) of children

Inflammatory Manifestations of Systemic Diseases in the Central Nervous System

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