Acute Disseminated Encephalomyelitis: A Gray Distinction

Libdeh, Amal Abu; Goodkin, Howard P.; Ramirez-Montealegre, Denia; Brenton, J. Nicholas

doi:10.1016/j.pediatrneurol.2016.12.006

Cited by 8 publications

(6 citation statements)

References 14 publications

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“…Gadolinium enhancement is variable, reported in one-third of cases 24 . Neuroimaging can be normal early in the course of ADEM; therefore, if clinical suspicion remains high, repeat imaging should be obtained 27 . Following treatment and clinical recovery, the majority of children exhibit complete or partial resolution of previously noted MRI abnormalities 28 …”

Section: Acquired Demyelinating Syndromesmentioning

confidence: 99%

Pediatric Acquired Demyelinating Disorders

Brenton¹

2022

CONTINUUM: Lifelong Learning in Neurology

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PURPOSE OF REVIEW: This article reviews the clinical presentation, diagnostic evaluation, treatment, and prognosis of the most common monophasic and relapsing acquired demyelinating disorders presenting in childhood.RECENT FINDINGS: Our understanding of neuroimmune disorders of the central nervous system is rapidly expanding. Several clinical and paraclinical factors help to inform the diagnosis and ultimately the suspicion for a monophasic versus relapsing course, including the age of the patient (prepubertal versus postpubertal), presence or absence of clinical encephalopathy, identification of serum autoantibodies (eg, myelin oligodendrocyte glycoprotein [MOG] and aquaporin-4), presence of intrathecally unique oligoclonal bands, and location/extent of radiologic abnormalities. Collaborative international research efforts have facilitated understanding of the safety and efficacy of currently available immunotherapies in children with acquired demyelinating disorders, particularly multiple sclerosis.SUMMARY: Although many of the demyelinating disorders presented in this article can affect children and adults across the age spectrum, the clinical and radiologic phenotypes, treatment considerations, and long-term prognoses are often distinct in children.

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Section: Acquired Demyelinating Syndromesmentioning

confidence: 99%

Pediatric Acquired Demyelinating Disorders

Brenton¹

2022

CONTINUUM: Lifelong Learning in Neurology

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“…Involvement of the basal ganglia and posterior fossa lesions is common. 25 Various patterns of Gd enhancement (nodular, diffuse, a complete ring, or a partial ring) are seen in 30% of lesions. 21 Multifocal TDLs are seen in ADEM.…”

Section: Demyelinating Diseasesmentioning

confidence: 99%

“…24 Disease course varies from monophasic ADEM in >70% of patients, second episode in the same site of the brain in 10%, and multiple episodes in different sites in 10% of patients. 25 The first demyelinating event might also represent the first manifestation of a chronic demyelinating relapsing disease, such as MS, NMOSD, or myelin oligodendrocyte glycoprotein associated disease (MOGAD). 21 Characteristic MRI features include bilateral but asymmetric, diffuse, large (>1-2 cm) FLAIR and T2-hyperintensities with indistinct borders that predominately involve the cerebral white matter of the brain (Figure 2(a-c)) and spine.…”

Section: Acute Demyelinating Encephalomyelitismentioning

confidence: 99%

“…ataxia, limb weakness, sensory changes, visual loss, cranial nerve impairment, or speech dysfunction), and seizure. 24 Disease course varies from monophasic ADEM in >70% of patients, second episode in the same site of the brain in 10%, and multiple episodes in different sites in 10% of patients. 25 The first demyelinating event might also represent the first manifestation of a chronic demyelinating relapsing disease, such as MS, NMOSD, or myelin oligodendrocyte glycoprotein associated disease (MOGAD).…”

Section: Demyelinating Diseasesmentioning

confidence: 99%

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Autoimmune diseases of the brain, imaging and clinical review

et al. 2021

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There is an extensive spectrum of autoimmune entities that can involve the central nervous system, which has expanded with the emergence of new imaging modalities and several clinicopathologic entities. Clinical presentation is usually non-specific, and imaging has a critical role in the workup of these diseases. Immune-mediated diseases of the brain are not common in daily practice for radiologists and, except for a few of them such as multiple sclerosis, there is a vague understanding about differentiating them from each other based on the radiological findings. In this review, we aim to provide a practical diagnostic approach based on the unique radiological findings for each disease. We hope our diagnostic approach will help radiologists expand their basic understanding of the discussed disease entities and narrow the differential diagnosis in specific clinical scenarios. An understanding of unique imaging features of these disorders, along with laboratory evaluation, may enable clinicians to decrease the need for tissue biopsy.

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“…ADEM is an immune-mediated inflammatory demyelinating disease (56, 90–92). Distinct from multiple sclerosis, ADEM is characterized by a monophasic course, affects mostly children (93), and is more prevalent among men (90, 94–96). There is general agreement on the seasonal onset of ADEM in winter and spring (94).…”

Section: Adem and Pregnancymentioning

confidence: 99%

Pregnancy-Related Immune Changes and Demyelinating Diseases of the Central Nervous System

Qiu

Chen

et al. 2019

Front. Neurol.

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Demyelinating diseases of the central nervous system comprise a heterogeneous group of autoimmune disorders characterized by myelin loss with relative sparing of axons occurring on a background of inflammation. Some of the most common demyelinating diseases are multiple sclerosis, acute disseminated encephalomyelitis, and neuromyelitis optica spectrum disorders. Besides showing clinical, radiological, and histopathological features that complicate their diagnosis, demyelinating diseases often involve different immunological processes that produce distinct inflammatory patterns. Evidence of demyelination diseases derives mostly from animal studies of experimental autoimmune encephalomyelitis (EAE), a model that relies on direct antibody–antigen interactions induced by encephalitogenic T cells. Pregnancy is characterized by non-self-recognition, immunomodulatory changes and an altered Th1/Th2 balance, generally considered a Th2-type immunological state that protects the mother from infections. During pregnancy, the immune response of patients with autoimmune disease complicated with pregnancy is different. Immune tolerance in pregnancy may affect the course of some diseases, which may reach remission or be exacerbated. In this review, we summarize current knowledge on the immune status during pregnancy and discuss the relationship between pregnancy-related immune changes and demyelinating diseases of the central nervous system.

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Acute Disseminated Encephalomyelitis: A Gray Distinction

Cited by 8 publications

References 14 publications

Pediatric Acquired Demyelinating Disorders

Pediatric Acquired Demyelinating Disorders

Autoimmune diseases of the brain, imaging and clinical review

Pregnancy-Related Immune Changes and Demyelinating Diseases of the Central Nervous System

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