Comparative immunopathogenesis of acute disseminated encephalomyelitis, neuromyelitis optica, and multiple sclerosis

  title={Comparative immunopathogenesis of acute disseminated encephalomyelitis, neuromyelitis optica, and multiple sclerosis},
  author={Dean M. Wingerchuk and Claudia F. Lucchinetti},
  journal={Current Opinion in Neurology},
Purpose of reviewAdvanced immunopathological techniques hold promise for more precise diagnosis of idiopathic demyelinating diseases of the central nervous system. We review recent progress in differentiating and understanding the disease mechanisms of acute disseminated encephalomyelitis, neuromyelitis optica, and classical multiple sclerosis. Recent findingsFour distinct immunopathological patterns have been described in multiple sclerosis patients, potentially implicating different… 

Epidemiology, immunopathogenesis and management of pediatric central nervous system inflammatory demyelinating conditions

  • D. Pohl
  • Medicine, Psychology
    Current opinion in neurology
  • 2008
Characteristics of pediatric inflammatory demyelinating central nervous system diseases, including monofocal and potentially monophasic disorders like optic neuritis and transverse myelitis, and multifocal chronic diseases like relapsing neuromyelitis optica and multiple sclerosis are discussed.

Myelin oligodendrocyte glycoprotein antibody-associated disease: an immunopathological study.

This study suggests that ADEM-like perivenous inflammatory demyelination with MOG-dominant myelin loss is a characteristic finding of MOG antibody-associated disease regardless of whether the diagnostic criteria of ADEM are met.

Neuromyelitis optica IgG serostatus in fulminant central nervous system inflammatory demyelinating disease.

This study is the first to compare NMO IgG serostatus among patients with fulminant central nervous system inflammatory demyelinating disease (CNS IDD) and concludes that N MO IgG is a specific biomarker for NMO spectrum disorders and is not simply a marker of destructive CNS IDD.

The Spectrum of Neuromyelitis Optica (NMO) in Childhood

Current clinical, pathological, and pathogenetic knowledge is reviewed with a focus on clinical presentation, neuroimaging findings, serological investigations, and treatment of children with disorders within the spectrum of central nervous system aquaporin-4 autoimmunity.

Acute disseminated encephalomyelitis

  • S. Tenembaum
  • Medicine, Psychology
    Handbook of Clinical Neurology
  • 2013

Pathogenesis, diagnosis and treatment of neuromyelitis optica: Changing concept of an old disease

Current concepts of diagnosis and treatment of NMO and NMO spectrum diseases are summarized and treatment decisions in different clinical situations are shown by discussion of cases.

Differential diagnosis of neuromyelitis optica spectrum disorders

Despite some similarity in their phenotypes, these NMOSD andNMOSD-mimics are distinct from each other in their pathogenesis, prognosis, and most importantly treatment.

New onset neuromyelitis optica in a young Nigerian woman with possible antiphospholipid syndrome: a case report

Neuromyelitis optica should be considered in the differential diagnoses of acute myelopathy in Africans and the unusual association with antiphospholipid syndrome should be highlighted.

Neuromyelitis optica pathogenesis and aquaporin 4

Dysregulation of tolerance associated with autoimmune disease appears to have a role in NMO and reagents and experimental questions that need to be developed and addressed are identified to enhance the understanding of the pathogenesis of NMO.

Prevalence of neuromyelitis optica spectrum disorder and phenotype distribution

A low prevalence of NMO and related disorders among demyelinating inflammatory diseases in a Caucasian population is confirmed and an unexpectedly high prevalence of limited and atypical variants of this disease is demonstrated, not previously documented.



A role for humoral mechanisms in the pathogenesis of Devic's neuromyelitis optica.

The extent of complement activation, eosinophilic infiltration and vascular fibrosis observed in the Devic NMO cases is more prominent compared with that in prototypic multiple sclerosis, and supports a role for humoral immunity in the pathogenesis of NMO.

Pattern-specific loss of aquaporin-4 immunoreactivity distinguishes neuromyelitis optica from multiple sclerosis.

Findings strongly support a role for a complement activating AQP4-specific autoantibody as the initiator of the NMO lesion, and further distinguish NMO from MS.

Acute disseminated encephalomyelitis: an update.

A number of recently conducted observational case series have substantially broadened the understanding about the clinical phenotype, diagnosis, and prognosis of acute disseminated encephalomyelitis.

Loss of aquaporin-4 in active perivascular lesions in neuromyelitis optica: a case report.

The findings suggest that astrocytic impairment associated with humoral immunity against AQP4 may be primarily involved in the lesion formation of N MO, and that the pathomechanisms of NMO are different from those of MS in which demyelination is the primary pathology.

Heterogeneity of multiple sclerosis lesions: Implications for the pathogenesis of demyelination

At a given time point of the disease, the patterns of demyelination were heterogeneous between patients, but were homogenous within multiple active lesions from the same patient, suggesting that MS may be a disease with heterogeneous pathogenetic mechanisms.

Brain abnormalities in neuromyelitis optica.

MRI brain findings in NMO justify revision of diagnostic criteria for NMO to allow for brain involvement, and asymptomatic brain lesions do not exclude the diagnosis of NMO.

Identification of new serum autoantibodies in neuromyelitis optica using protein microarrays

A protein microarray approach was used to profile the Ag reactivity of serum IgG from NMO patients and found that following treatment with rituximab, patients experienced improved strength and sensation in their legs and recovered spontaneously without additional treatment.

Clinical course, pathological correlations, and outcome of biopsy proved inflammatory demyelinating disease

Most patients undergoing biopsy, who had pathologically confirmed demyelinating disease, were likely to develop MS and remain ambulatory after a median disease duration of 4.4 years, and data suggest that pathogenic implications derived largely from MS biopsy studies may be extrapolated to the general MS population.

Activation of humoral immunity and eosinophils in neuromyelitis optica

It is suggested that neuromyelitis optica is associated with a major humoral immune response (particularly anti-MOG IgM production) and eosinophil activation present exclusively in CSF.

Immunopathogenesis and immunotherapy of multiple sclerosis

Current knowledge of the immunopathogenesis of MS and corresponding animal models of disease is reviewed, new immunointerventional treatment strategies based on changing pathogenetic concepts are discussed, and new immunotherapeutic agents developed and evaluated in clinical trials are discussed.