Diagnosis of inflammatory demyelination in biopsy specimens: a practical approach

Kuhlmann, Tanja; Lassmann, Hans; Brück, Wolfgang

doi:10.1007/s00401-007-0320-8

Cited by 104 publications

(90 citation statements)

References 93 publications

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“…In contrast to MS lesions, demyelination is generally limited to perivascular areas with inflammatory infiltrates and the large confluent demyelinated lesions typical for MS do not form. 10,11 In very acute ADEM lesions, perivascular complement deposits can be found, possibly pointing toward an antibody-/complement-mediated pathogenesis. 12,13 Although NMO was once considered a variant of MS, the discovery of the autoantibody marker NMO-IgG directed against aquaporin-4 (AQP4) 14 has since established NMO as a pathophysiologically distinct entity.…”

Section: General Aspects Of Cns Inflammatory Demyelinating Diseases Mmentioning

confidence: 99%

Immunopathophysiology of pediatric CNS inflammatory demyelinating diseases

et al. 2016

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Elucidating pathophysiologic mechanisms underlying the spectrum of pediatric-onset CNS demyelinating diseases, particularly those that may distinguish multiple sclerosis (MS) from other entities, promises to both improve diagnostics and guide more-informed therapeutic decisions. Observations that pediatric-and adult-onset MS share the same genetic and environmental risk factors support the view that these conditions represent essentially the same illness manifesting at different ages. Nonetheless, special consideration must be given when CNS inflammation manifests in early life, at a time when multiple organs (including immune and nervous systems) are actively maturing. CSF analysis in pediatric-onset MS points to chronic CNS inflammation, supported by observations from limited pathologic material available for study. Emerging results implicate abnormalities in both effector and regulatory T cell subsets, and potentially immune senescence, in children with MS. Although CNS-directed antibodies (including antibodies recognizing myelin antigens; Kir4.1) can be documented in pediatric-onset MS, their pathophysiologic significance (as in adults) remains unclear. This is in contrast to the presence of serum and/or CSF antibodies recognizing aquaporin-4, which, when measured using validated cell-based assays, supports the diagnosis of a neuromyelitis optica spectrum disorder, distinct from MS. Presence of anti-myelin oligodendrocyte glycoprotein antibodies documented with similar cell-based assays may also be associated with pathophysiologically distinct disease phenotypes in children. The substantial impact of pediatriconset MS on normal brain development and function underscores the importance of elucidating both the immunobiology and neurobiology of disease. Ongoing efforts are aimed at developing and validating biological measures that define pathophysiologically distinct monophasic and chronic forms of pediatric CNS demyelination. Neurology ® 2016;87 (Suppl 2):S12-S19 GLOSSARY ADEM 5 acute disseminated encephalomyelitis; AQP4 5 aquaporin-4; BBB 5 blood-brain barrier; CBA 5 cell-based assay; EDSS 5 Expanded Disability Status Scale; MBP 5 myelin basic protein; MOG 5 myelin oligodendrocyte glycoprotein; MS 5 multiple sclerosis; NMO 5 neuromyelitis optica; NMOSD 5 NMO spectrum disorder; OCB 5 oligoclonal band; ON 5 optic neuritis; OPC 5 oligodendrocyte precursor cell; TM 5 transverse myelitis.There is presently limited insight into the pathophysiologic mechanisms underlying childhoodonset inflammatory demyelinating diseases. Because these disorders occur in the context of the developing immune and nervous systems, understanding the implications to both disease mechanisms and efficacy and safety profiles of potential therapeutics will help guide optimal management approaches for these children and adolescents. Elucidating the biology of pediatric-onset multiple sclerosis (MS) may provide key insights into earliest targets and processes involved in disease pathogenesis as well as into the broader spectrum of CNS...

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Section: General Aspects Of Cns Inflammatory Demyelinating Diseases Mmentioning

confidence: 99%

Immunopathophysiology of pediatric CNS inflammatory demyelinating diseases

et al. 2016

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“…This seems partly because, in contrast to those of MS, few cases of ADEM with immunohistochemical analysis have been available so far, including MEDM variant. A variability in the appearance and nature of T and B lymphocytes has been reported 7 .…”

Section: On the Coexistence Of Adem And Ms Pathologymentioning

confidence: 99%

Commentary: Is ADEM a subgroup of MS?

Ohara¹

2017

J Neurol Neuromedicine

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Acute disseminated encephalomyelitis (ADEM) and Multiple sclerosis (MS) are both immunologically mediated inflammatory demyelinating disease of the CNS. ADEM and MS have long been considered as a separate disease entities, but clinical differentiation of ADEM from the first attack of MS is often difficult because of overlapping clinical features. Pathologically, perivenous demyelination and discrete confluent demyelination (plaque) have been generally regarded as the hallmark of ADEM and MS, respectively. It is also known that in contrast to MS, which shows quite diverse heterogeneous pathologic patterns, ADEM shows generally homogenous pathological features of inflammatory demyelination. However, hybrid cases showing pathological features of both ADEM and MS do exist, suggesting that ADEM may share some common underlying pathologic mechanisms with certain stages or subgroups of MS. TextAcute disseminated encephalomyelitis (ADEM) and multiple sclerosis (MS) have been considered as clinically and pathologically distinct phenotype of inflammatory demyelinating disease of CNS 1,2 . ADEM, typically but not always anteceded by infectious illness or vaccination, presents with a monophasic course with relatively favorable prognosis, while MS typically exhibits a relapsing and remitting course with accumulating neurological deficits with each exacerbation. However, despite several clinical and/or radiological criteria proposed to differentiate ADEM from MS, none has been proven to unequivocally separate them. With many similarities in clinical presentation, MRI findings and putative pathogenesis, Hartung and Grossmann speculated that ADEM may not be a distinctive disease but a part of the MS spectrum 3 . On the other hand, pathological differentiation between ADEM and MS has been regarded as most reliable. ADEM is characterized by perivenous demyelination and MS by confluent demyelination, with these two patterns seldom coexisting in a single patient 4 .We have recently experienced a patient clinically diagnosed as ADEM whose brain biopsy revealed pathologically features indistinguishable from active lesions of MS in addition to the characteristic foci of perivenular inflammation and demyelination of ADEM 5. This minireview is based on this report. Case presentationDetailed clinical information of the patient has been reported previously 5 . Briefly, a 51-year female presented with progressive aphasia and right-sided hemiparesis after a month history of new onset increasing headache. There was no apparent antecedent flu-

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“…However, demonstrating atypical mitotic figures in a benign reactive lesion is not something new, but is rare finding in both experimental and surgical pathology related to inflammatory or regenerative processes [1][2][3][4][5][6][7][8]. One may argue that some of above mentioned chronic pathological processes, for example, long standing ulcerative colitis [4] and gastric intestinal metaplasia [5], are associated with malignant potential.…”

Section: Case Reportmentioning

confidence: 99%

“…After over one century, a general consensus has been reached in the surgical pathology society, stating that an atypical mitotic figure may indicate a malignant process while it can also be seen in benign lesions. For example, atypical mitotic figures can be occasionally found in granulation tissue following ionizing-radiation exposur, ischemic colitis, long standing ulcerative colitis, gastric intestinal metaplasia, endometrium associated with chorionic tissue effect, benign cutaneous lesions induced by taxane therapy, and abnormal but non-neoplastic astrocytes [2][3][4][5][6][7][8]. Herein, a rare case of ischemic fasciitis, also named as atypical decubital fibroplasia, is reported, which shows typical clinical and histological features, except that atypical mitosis is evident.…”

Section: Introductionmentioning

confidence: 99%

Benign reactive lesion with atypical mitosis: New example of an old story

Zhong¹,

Chekmareva²,

Deen³

et al. 2014

IJCRI

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International Journal of Case Reports and Images (IJCRI) is an international, peer reviewed, monthly, open access, online journal, publishing high-quality, articles in all areas of basic medical sciences and clinical specialties.Aim of IJCRI is to encourage the publication of new information by providing a platform for reporting of unique, unusual and rare cases which enhance understanding of disease process, its diagnosis, management and clinico-pathologic correlations. IJCRI publishes Review

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Diagnosis of inflammatory demyelination in biopsy specimens: a practical approach

Cited by 104 publications

References 93 publications

Immunopathophysiology of pediatric CNS inflammatory demyelinating diseases

Immunopathophysiology of pediatric CNS inflammatory demyelinating diseases

Commentary: Is ADEM a subgroup of MS?

Benign reactive lesion with atypical mitosis: New example of an old story

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