Clinical and radiological characteristics of 17 Chinese patients with pathology confirmed tumefactive demyelinating diseases: Follow-up study

Yao, Jiarui; Huang, Dehui; Gui, Qiuping; Chen, Xiaolei; Lou, Xin; Wu, Lei; Chen, Cheng; Li, Jie; Wu, Wei

doi:10.1016/j.jns.2014.11.027

Cited by 8 publications

(5 citation statements)

References 26 publications

(60 reference statements)

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“…Studies by Song et al have highlighted dynamic enhancement patterns in TDL, with ring enhancement more common during the subacute phase 47 . This current study also noted restricted diffusion in 91.7% of the patients, primarily in the peripheral areas, consistent with prior research 36 , 41 . Co-existing demyelinating lesions were identified from brain MRI in 38.5% of patients, and from spinal MRI in 19% of patients, although these could not fulfill the diagnostic criteria of RRMS due to TDL not being counted as one region in the 'dissemination in space' criteria of the 2017 McDonald criteria 28 .…”

Section: Discussionsupporting

confidence: 91%

“…Nevertheless, in the majority of patients, symptoms could still be localized to a specific anatomical region (monofocal), primarily due to the solitary nature of most TDL in this study. These clinical findings aligned with previous research 40 , 41 . Regarding the median duration from initial hospital visit to definitive diagnosis, which averaged 1 month with a maximum of 9 months, this underscored the issue of delayed diagnosis among TDL patients, potentially impacting treatment and outcomes.…”

Section: Discussionsupporting

confidence: 91%

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Tumefactive demyelinating lesions: a retrospective cohort study in Thailand

Ongphichetmetha,

Aungsumart,

Siritho

et al. 2024

Sci Rep

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Tumefactive demyelinating lesions (TDL), characterized by large (≥ 2 cm) demyelinating lesions mimicking tumors, are a rare manifestation of the central nervous system inflammatory demyelinating diseases (CNS-IDD). Distinguishing TDL from other brain lesions can be challenging, often necessitating biopsy or advanced diagnostics. The natural history of TDL varies among races. This study aimed to assess demographics, clinical and radiological features, laboratory findings, management, and outcomes of Thai patients with TDL. We retrospectively reviewed records of twenty-six patients with TDL from the Multiple Sclerosis and Related Disorders registry from two tertiary medical centers. Among 1102 CNS-IDD patients, 26 (2.4%) had TDL. The median age at TDLs onset was 34.5 years (range 17–75); 69.2% were female. Over 70% manifested TDL as their initial CNS-IDD presentation. Common presenting symptoms included motor deficits, sensory disturbances, and cognitive problems. About two-fifths exhibited multiple lesions, most frequently in the frontoparietal region (46.2%). Half of the patients showed an incomplete ring on post-contrast T1-weighted imaging, with peripheral diffusion-weighted imaging restriction in twenty-one patients. T2-hypointense rims were present in thirteen (56.5%) patients. Brain biopsy was performed in 12 cases (46.1%). Serum aquaporin-4 immunoglobulin was positive in 16.7% of tested (4/24) cases. Serum myelin oligodendrocyte glycoprotein immunoglobulin was negative in all thirteen patients tested. Twenty patients (76.9%) received intravenous corticosteroids for TDL attacks. After the median follow-up period of 48 months (range 6–300), 23.1% experienced CNS-IDD relapses. Median Expanded Disability Status Scale at TDL diagnosis was 4.3 (range 0.0–9.5), and improved to 3.0 (range 0.0–10.0) at the last follow-up. This study suggested that TDL were rare among Thai CNS-IDD patients, frequently presenting as a monophasic condition with a favorable outcome.

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Section: Discussionsupporting

confidence: 91%

Section: Discussionsupporting

confidence: 91%

Tumefactive demyelinating lesions: a retrospective cohort study in Thailand

Ongphichetmetha,

Aungsumart,

Siritho

et al. 2024

Sci Rep

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“…Regarding B cells, TDLs are composed of B cells with variable tissue presence ranging from no B cells to rare, few, and intense B cell infiltrates. These are mainly distributed in the perivascular spaces ( 53 , 54 ). Only few studies have assessed plasma cells in TDLs, and these are mostly described in tumefactive lesions of patients with NMO ( 53 ).…”

Section: Tumefactive Demyelinated Lesions–immunopathologymentioning

confidence: 99%

“…These are mainly distributed in the perivascular spaces ( 53 , 54 ). Only few studies have assessed plasma cells in TDLs, and these are mostly described in tumefactive lesions of patients with NMO ( 53 ). Plasma cells have been classically detected scattered in the CNS parenchyma of patients with remitting-relapsing and secondary progressive MS, but predominantly at the periphery of B-cell follicles/eLFs in patients with SPMS.…”

Section: Tumefactive Demyelinated Lesions–immunopathologymentioning

confidence: 99%

“…Data analysis of the microglia phenotype in TDL is missing. Only a few studies have described microglia cells and most of them without assessing their activated or homeostatic status or exact prevalence among infiltrating cells in lesions ( 53 , 54 ). Interestingly, a new study has shown that activated microglia and macrophages (HLADR positive cells as a marker) form a rim at the lesion borders and show a perivenous accentuation in MOG-positive patients manifesting tumefactive lesions ( 51 ).…”

Section: Tumefactive Demyelinated Lesions–immunopathologymentioning

confidence: 99%

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Immunopathology of Tumefactive Demyelinating Lesions-From Idiopathic to Drug-Related Cases

et al. 2022

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Tumefactive demyelinating lesions (TDL) represent a diagnostic dilemma for clinicians, and in rare atypical cases a collaboration of a neuroradiologist, a neurologist, and a neuropathologist is warranted for accurate diagnosis. Recent advances in neuropathology have shown that TDL represent an umbrella under which many different diagnostic entities can be responsible. TDL can emerge not only as part of the spectrum of classic multiple sclerosis (MS) but also can represent an idiopathic monophasic disease, a relapsing disease with recurrent TDL, or could be part of the myelin oligodendrocyte glycoprotein (MOG)- and aquaporin-4 (AQP4)-associated disease. TDL can appear during the MS disease course, and increasingly cases arise showing an association with specific drug interventions. Although TDL share common features with classic MS lesions, they display some unique features, such as extensive and widespread demyelination, massive and intense parenchymal infiltration by macrophages along with lymphocytes (mainly T but also B cells), dystrophic changes in astrocytes, and the presence of Creutzfeldt cells. This article reviews the existent literature regarding the neuropathological findings of tumefactive demyelination in various disease processes to better facilitate the identification of disease signatures. Recent developments in immunopathology of central nervous system disease suggest that specific pathological immune features (type of demyelination, infiltrating cell type distribution, specific astrocyte pathology and complement deposition) can differentiate tumefactive lesions arising as part of MS, MOG-associated disease, and AQP4 antibody-positive neuromyelitis optica spectrum disorder. Lessons from immunopathology will help us not only stratify these lesions in disease entities but also to better organize treatment strategies. Improved advances in tissue biomarkers should pave the way for prompt and accurate diagnosis of TDL leading to better outcomes for patients.

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Pediatric Tumefactive Demyelination

Lopez

Lotze

2017

Pediatric Demyelinating Diseases of the Central Nervous System and Their Mimics

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Clinical and radiological characteristics of 17 Chinese patients with pathology confirmed tumefactive demyelinating diseases: Follow-up study

Cited by 8 publications

References 26 publications

Tumefactive demyelinating lesions: a retrospective cohort study in Thailand

Tumefactive demyelinating lesions: a retrospective cohort study in Thailand

Immunopathology of Tumefactive Demyelinating Lesions-From Idiopathic to Drug-Related Cases

Pediatric Tumefactive Demyelination

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