A Rare Case of Adult Acute Disseminated Encephalomyelitis Associated with Primary Epstein-Barr Virus Infection

Ali, Deeba; Cardos, Benoît; Gorur, Yilmaz; Villalba, Noël Lorenzo; Janssen, N.; Bartha, Carmen; Desfontaines, Phillippe; Weerts, J; Fajardo, Pascal

doi:10.12890/2019_001094

Cited by 6 publications

(11 citation statements)

References 10 publications

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“…EBV infection is mostly asymptomatic in healthy individuals, but a small number can develop CNS complications including demyelinating disease, acute encephalitis, acute cerebellar ataxia, meningitis, or myelitis [8]. A few cases of EBV infection-related ADEM have been reported (Table 1 [8][9][10][11][12][13][14]).…”

Section: Discussionmentioning

confidence: 99%

Epstein-Barr virus-associated acute disseminated encephalomyelitis successfully treated with rituximab: a case report

2021

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Acute disseminated encephalomyelitis (ADEM) is an inflammatory demyelinating disorder of the central nervous system (CNS) characterized by acute polyfocal neurologic symptoms with encephalopathy. In most cases, brain magnetic resonance imaging (MRI) shows abnormalities consistent with demyelination. Typical MRI findings are multiple, hyperintense, poorly-marginated lesions with bilateral asymmetric cerebral involvement in T2-weighted and fluid-attenuated inversion recovery imaging [1]. Tumefactive demyelinating lesions resembling CNS neoplasia or pseudoleukodystrophic patterns have been reported [2,3]. Most patients show an excellent response to systemic steroids, and serial MRI usually reveals complete or partial resolution of previous lesions [1]. However, approximately 20% of patients with ADEM require additional immunotherapy such as intravenous immunoglobulin (IVIg) or plasmapheresis, and these pa-

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Section: Discussionmentioning

confidence: 99%

Epstein-Barr virus-associated acute disseminated encephalomyelitis successfully treated with rituximab: a case report

2021

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“…This case report highlights the importance of considering the possibility of a broad spectrum of causes for neuropsychiatric manifestations in a patient with SLE. And in a patient with presumptive diagnosis of TTP and neurological manifestations who does not show an adequate response to TPE, an alternative or superadded aetiology for his/her neurological manifestations [9,10] should be thought of and investigated for. Lastly, it should be noted that prompt administration of more potent immunosuppressive therapy beyond the standard treatment in ADEM occurring in a background of an autoimmune condition like SLE, can be life saving like in the case of this young patient.…”

Section: Discussionmentioning

confidence: 99%

A case of thrombotic microangiopathy and acute demyelinating central nervous system lesions as the first manifestation of systemic lupus erythematosus

Ellepola¹,

Higgoda²,

Jayasinghe³

et al. 2021

SL J. Med.

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Systemic lupus erythematosus (SLE) is a chronic autoimmune disorder with a relapsing and remitting course and multi-organ involvement which can present with a wide range of neuropsychiatric manifestations. Acute disseminating encephalomyelitis (ADEM) is an autoimmune demyelinating disorder of the central nervous system (CNS). Thrombotic thrombocytopenic purpura (TTP) is a medical emergency which manifests with microangiopathic haemolytic anaemia (MAHA), thrombocytopenia and neurological abnormalities and is known to be a rare association of SLE. We report a case of a 14 year old female who presented with fever and altered consciousness, subsequently diagnosed to have SLE with thrombotic microangiopathy and acute demyelinating CNS lesions in an ADEM-distribution

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“…The clinical features include acute onset, multifocal neurological deficits with a monophasic course, and a typical clinical course of rapid neurological deterioration over several days. Most patients have viral prodromal symptoms followed by the development of focal neurological deficits and a good prognosis after symptomatic treatment, although some cases experience severe complications and mortality ( Bahadori et al, 2007 ; Phowthongkum et al, 2007 ; Di Carlo et al, 2011 ; Ali et al, 2019 ).…”

Section: Ebv and Acute Disseminated Encephalomyelitismentioning

confidence: 99%

“…EBV can cause encephalopathy directly in the CNS or indirectly via autoimmune ADEM( Wong et al, 1999 ; Bahadori et al, 2007 ). The immune-mediated process of EBV-related acute disseminated encephalitis and infected encephalomyelitis can lead to the multifocal demyelination of peripheral venous white matter, positive EBV IgM in CSF, and positive EBV DNA in the serum ( Saeed et al, 2014 ; Ali et al, 2019 ). One study found cross-reactivity between EBNA-1 and myelin oligodendrocyte glycoprotein (MOG), in which EBV infection triggered the production of anti-MOG antibodies thereby elevating MOG concentrations and inducing the development of ADEM ( Selter et al, 2010 ; Lalive et al, 2011 ; Nakamura et al, 2017 ).…”

Section: Ebv and Acute Disseminated Encephalomyelitismentioning

confidence: 99%

Epstein-Barr Virus and Neurological Diseases

Zhang

Zuo

Jiang

et al. 2022

Front. Mol. Biosci.

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Epstein-Barr virus (EBV), also known as human herpesvirus 4, is a double-stranded DNA virus that is ubiquitous in 90–95% of the population as a gamma herpesvirus. It exists in two main states, latent infection and lytic replication, each encoding viral proteins with different functions. Human B-lymphocytes and epithelial cells are EBV-susceptible host cells. EBV latently infects B cells and nasopharyngeal epithelial cells throughout life in most immunologically active individuals. EBV-infected cells, free viruses, their gene products, and abnormally elevated EBV titers are observed in the cerebrospinal fluid. Studies have shown that EBV can infect neurons directly or indirectly via infected B-lymphocytes, induce neuroinflammation and demyelination, promote the proliferation, degeneration, and necrosis of glial cells, promote proliferative disorders of B- and T-lymphocytes, and contribute to the occurrence and development of nervous system diseases, such as Alzheimer’s disease, Parkinson’s disease, multiple sclerosis, acute cerebellar ataxia, meningitis, acute disseminated encephalomyelitis, and brain tumors. However, the specific underlying molecular mechanisms are unclear. In this paper, we review the mechanisms underlying the role of EBV in the development of central nervous system diseases, which could bebeneficial in providing new research ideas and potential clinical therapeutic targets for neurological diseases.

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A Rare Case of Adult Acute Disseminated Encephalomyelitis Associated with Primary Epstein-Barr Virus Infection

Cited by 6 publications

References 10 publications

Epstein-Barr virus-associated acute disseminated encephalomyelitis successfully treated with rituximab: a case report

Epstein-Barr virus-associated acute disseminated encephalomyelitis successfully treated with rituximab: a case report

A case of thrombotic microangiopathy and acute demyelinating central nervous system lesions as the first manifestation of systemic lupus erythematosus

Epstein-Barr Virus and Neurological Diseases

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