A case of ADEM-like presentation with anti-MOG antibody following tumefactive demyelinating lesion

Miyaue, Noriyuki; Yamanishi, Yuki; Tada, Satoshi; Ando, Rina; Yabe, Hayato; Nagai, Masahiro; Nomoto, Masahiro

doi:10.1016/j.msard.2019.03.018

Cited by 15 publications

(12 citation statements)

References 5 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Furthermore, we have two patients that showed tumefactive demyelinating lesion on MRI, which is defined as an acute large (>2 cm) tumor-like demyelinating lesion in the CNS that may occur with surrounding edema, mass effect, and ring enhancement (34). It was a not uncommon white matter change seen in brain MRI and was also reported in MOGAD cases in previous literature (35,36). In this study, we incorporated tumefactive demyelinating lesion into MRI patterns of MOG-E, which enlarged brain MRI phenotypes of MOG-E on the basis of Hacohen's study.…”

Section: Discussionmentioning

confidence: 89%

Clinical and Imaging Features of Patients With Encephalitic Symptoms and Myelin Oligodendrocyte Glycoprotein Antibodies

Wang

Qiu

et al. 2021

Front. Immunol.

View full text Add to dashboard Cite

BackgroundMyelin oligodendrocyte glycoprotein-antibody (MOG-ab)-associated disease (MOGAD) has highly heterogenous clinical and imaging presentations, in which encephalitis is an important phenotype. In recent years, some atypical presentations in MOG-ab-associated encephalitis (MOG-E) have been increasingly reported but have not yet been described well. The aim of the study was to describe the clinical and imaging features of patients with MOG-E in our center. Atypical phenotypes would be reported, which is expected to expand the spectrum of MOGAD.MethodsWe reviewed medical records of 59 patients with MOGAD diagnosed in our center and identified cases who had ever experienced encephalitic symptoms. Three hundred ten patients with autoimmune encephalitis (AE) were also reviewed, and cases with positive MOG-ab were identified. Besides, patients with chronically progressive encephalitis were identified from 13 MOG-E and 310 AE patients. We collected demographic, clinical, laboratory, radiological, and outcome data to explore clinical and imaging characteristics in MOG-E, especially in the atypical phenotype of chronically progressive encephalitis.ResultsWe identified 13 patients (7 males, 6 females) with MOG-E. The median age at onset was 33 years (range 13~62 years). Most (9/13, 69.2%) of patients showed acute or subacute onset of encephalitic symptoms. Brain MRI abnormalities were observed in all patients. The most common lesion locations on MRI were cortical/subcortical (11/13, 84.6%), deep/periventricular white matter (10/13, 76.9%) and corpus callosum (4/13, 30.8%). Brain MRI patterns were categorized into four phenotypes. The most common pattern was cortical encephalitis with leptomeningeal enhancement/brain atrophy (10/13, 76.9%). Eight (8/13, 61.5%) patients had a good response to immunotherapy. Four (4/13, 30.8%) patients with chronically progressive course were identified from MOG-E cohort. They showed leukodystrophy-like pattern, multifocal hazy lesions, or cortical encephalitis on MRI. With immunotherapy, they only showed mild or no improvement. We also identified four (4/310, 1.3%) patients with chronically progressive course from AE cohort. They had better outcomes than counterparts in MOG-E.ConclusionsThis study demonstrates that encephalitic presentations in MOGAD had complex clinical patterns. Chronically progressive encephalitis may be a new phenotype of MOGAD. We recommend to test MOG-ab in subacute and chronic progressive dementia with leukodystrophy-like MRI lesions.

show abstract

Section: Discussionmentioning

confidence: 89%

Clinical and Imaging Features of Patients With Encephalitic Symptoms and Myelin Oligodendrocyte Glycoprotein Antibodies

Wang

Qiu

et al. 2021

Front. Immunol.

View full text Add to dashboard Cite

show abstract

“…which is usually associated with to multiple sclerosis. 5 Recent studies have shown the anti-MOG antibody-positive cases presented supratentorial TDL, 6,7 or cortical lesions 8 but not in the brainstem.…”

Section: Discussionmentioning

confidence: 99%

“…TDL is defined radiologically as tumor‐ or mass‐like white matter dominant lesions exceeding 2 cm in size, which is usually associated with to multiple sclerosis . Recent studies have shown the anti‐MOG antibody‐positive cases presented supratentorial TDL, or cortical lesions but not in the brainstem. Thus, anti‐MOG antibody may accounts for TDL and cortical lesions in the present case.…”

Section: Discussionmentioning

confidence: 99%

A unique case with positive anti‐myelin oligodendrocyte glycoprotein antibody presenting multiple brain lesions

Matsumoto

Sato

Hishikawa

et al. 2020

Neurology & Clinical Neurosc

View full text Add to dashboard Cite

The accurate diagnosis of anti‐myelin oligodendrocyte glycoprotein (MOG) antibody‐associated diseases is sometimes challenging due to its various central nervous system (CNS) lesions. Here, we report a 22‐year‐old man with positive anti‐MOG antibody presenting multiple brain lesions including a brainstem tumefactive demyelinating lesion (TDL), cortical edematous lesions, and a periventricular white matter lesion. These variety of lesions made the diagnosis challenging, especially because TDL is quite rare among MOG antibody‐associated disease patients. The present case suggests the importance to check anti‐MOG antibody for patients presenting atypical multiple CNS lesions.

show abstract

“…Nevertheless, TDL might occur in patients with other atypical demyelinating syndromes such as Acute Disseminated Encephalomyelitis (ADEM), AQ4 IgG seropositive or MOG-seropositive Neuromyelitis Optica Spectrum Disorders (NMOSD), as well as other neuroinflammatory disorders, such as Neurosarcoidosis or Behçet's disease (4). Of note, the spectrum of MOG antibodyassociated encephalomyelitis the last years has expanded to involve cases with TDL and ADEM-like presentation, clinical presentations that could fit to our patient history (11,16). Nevertheless, our patient was negative for both MOG and AQ4 autoantibodies.…”

Section: Discussionmentioning

confidence: 99%

Recurrent Fulminant Tumefactive Demyelination With Marburg-Like Features and Atypical Presentation: Therapeutic Dilemmas and Review of Literature

et al. 2020

View full text Add to dashboard Cite

Atypical forms of demyelinating diseases with tumor-like lesions and aggressive course represent a diagnostic and therapeutic challenge for neurologists. Herein, we describe a 50-year-old woman presenting with subacute onset of left hemiparesis, memory difficulties and headache. Brain MRI revealed a tumefactive right frontal-parietal lesion with perilesional edema, mass effect and homogenous post-contrast enhancement, along with other small atypical lesions in the white-matter. Brain biopsy of cerebral lesion ruled out lymphoma or any other neoplastic process and patient placed on corticosteroids with complete clinical/radiological remission. Two years after disease initiation, there was disease exacerbation with reappearance of the tumor-like mass. The patient initially responded to high doses of corticosteroids but soon became resistant. Plasma-exchange sessions were not able to limit disease burden. Resistance to therapeutic efforts led to a second biopsy that showed perivascular demyelination, predominantly consisting of macrophages, with a small number of T and B lymphocytes, and the presence of reactive astrocytes, typical of Creutzfeldt-Peters cells. The patient received high doses of cyclophosphamide with substantial clinical/radiological response but relapsed after 7-intensive cycles. She received 4-weekly doses of rituximab with disease exacerbation and brainstem involvement. She eventually died with complicated pneumonia. We present a very rare case of recurrent tumefactive demyelinating lesions, with atypical tumor-like characteristics, with initial response to corticosteroids and cyclophosphamide, but subsequent development of drug-resistance and unexpected exacerbation upon rituximab administration. Our clinical case raises therapeutic dilemmas and points to the need for immediate and appropriate immunosuppression in difficult to treat tumefactive CNS lesions with Marburg-like features.

show abstract

A case of ADEM-like presentation with anti-MOG antibody following tumefactive demyelinating lesion

Cited by 15 publications

References 5 publications

Clinical and Imaging Features of Patients With Encephalitic Symptoms and Myelin Oligodendrocyte Glycoprotein Antibodies

Clinical and Imaging Features of Patients With Encephalitic Symptoms and Myelin Oligodendrocyte Glycoprotein Antibodies

A unique case with positive anti‐myelin oligodendrocyte glycoprotein antibody presenting multiple brain lesions

Recurrent Fulminant Tumefactive Demyelination With Marburg-Like Features and Atypical Presentation: Therapeutic Dilemmas and Review of Literature

Contact Info

Product

Resources

About