Acute Disseminated Encephalomyelitis in COVID-19: presentation of two cases and review of the literature

ABSTRACT Background: Neurological manifestations of COVID-19 are still incompletely understood. Neurological manifestations may be due to direct viral effect on neurons and glial cells, to an immune-mediated response to the virus, or to a hypercoagulable state and associated endothelial damage, as well as to severe systemic disease with prolonged intensive care unit stay. Objective: To describe two patients with severe SARS-CoV-2 infection and delayed recovery of consciousness after sedation withdrawal, in whom MRI disclosed multifocal white matter brain lesions, compatible with the diagnosis of acute disseminated encephalomyelitis. Methods: Observational report of two cases of severe COVID-19 infection in patients from two tertiary hospitals in São Paulo, Brazil. Results: These patients underwent neurologic and systemic evaluation for delayed awakening after sedation withdrawal. MRI displayed multifocal centrum semiovale lesions, suggestive of demyelinating inflammation. Cerebrospinal fluid (CSF) polymerase chain reaction (PCR) for SARS-CoV-2 was negative in both cases. Conclusion: A recurrent pattern of multifocal white matter lesions can occur in COVID-19 patients, possibly associated with delayed awakening. Additional studies are necessary to elucidate the role of the viral infection and of inflammatory and immune-mediated associated changes in neurological manifestations of COVID-19.

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The Snowball Effect

Neuroimmunology ◽

10.1093/med/9780190693190.003.0010 ◽

2018 ◽

pp. 55-60

Author(s):

Aaron E. Miller ◽

Tracy M. DeAngelis ◽

Michelle Fabian ◽

Ilana Katz Sand

Keyword(s):

Hearing Loss ◽

White Matter ◽

Vision Loss ◽

Demyelinating Disease ◽

White Matter Lesions ◽

Leptomeningeal Involvement ◽

Immune Mediated ◽

Retinal Artery ◽

Deep Gray Matter ◽

The Brain

Susac syndrome is a rare disease involving a triad of subacute encephalopathy, vision loss involving branch retinal artery occlusions (BRAOs), and sensorineural hearing loss. Clinical presentation is variable and generally does not involve the entire triad. The suspected pathophysiology is an immune-mediated endotheliopathy affecting the precapillary arterioles of the brain, retina, and inner ear. MRI of the brain reveals abnormal white matter lesions similar to demyelinating disease, but with distinct characteristic central callosal lesions, as well as deep gray matter and leptomeningeal involvement. There is no standardized therapy for Susac’s, but suspicion of an immune-mediated endotheliopathy has informed empirical treatment with corticosteroids and various immunosuppressant regimens, which appear to improve the prognosis.

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THU0201 Cognitive impairment in behçet’s disease is not associated to systemic disease activity, cumulative prednisone dose or white-matter lesions

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2012-eular.2166 ◽

2013 ◽

Vol 71 (Suppl 3) ◽

pp. 223.2-223

Author(s):

L.A. Dutra ◽

A.W.S. De Souza ◽

H. Alessi ◽

P. Braga Neto ◽

B.D.V.S. Guedes ◽

...

Keyword(s):

Cognitive Impairment ◽

White Matter ◽

Disease Activity ◽

Behçet’S Disease ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Systemic Disease ◽

White Matter Lesions ◽

Prednisone Dose ◽

Behcet's Disease

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Neurological Manifestations in Chagas Disease without Cardiac Dysfunction: Correlation between Dysfunction of the Parasympathetic Nervous System and White Matter Lesions in the Brain

Journal of Neuroimaging ◽

10.1111/j.1552-6569.2008.00305.x ◽

2009 ◽

Vol 19 (4) ◽

pp. 332-336 ◽

Cited By ~ 8

Author(s):

Marco Py ◽

Roberto Pedrosa ◽

Juliana Silveira ◽

Aline Medeiros ◽

Charles Andre

Keyword(s):

Nervous System ◽

White Matter ◽

Chagas Disease ◽

Cardiac Dysfunction ◽

Parasympathetic Nervous System ◽

White Matter Lesions ◽

Neurological Manifestations ◽

The Brain

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Acute disseminated encephalomyelitis following endonasal resection of a craniopharyngioma: illustrative case

Journal of Neurosurgery: Case Lessons ◽

10.3171/case21293 ◽

2021 ◽

Vol 2 (16) ◽

Author(s):

Konrad Gag ◽

Jonas Müller ◽

Marie Süße ◽

Robert Fleischmann ◽

Henry W. S. Schroeder

Keyword(s):

White Matter ◽

White Matter Lesions ◽

Acute Disseminated Encephalomyelitis ◽

Intravenous Immunoglobulins ◽

Neurological Deficits ◽

Illustrative Case ◽

Sensorimotor Deficits ◽

Fluid Analysis ◽

Magnetic Resonance Imaging Mri

BACKGROUND Acute disseminated encephalomyelitis (ADEM) is a rare, acquired demyelination syndrome that causes cognitive impairment and focal neurological deficits and may be fatal. The potentially reversible disease mainly affects children, often after vaccination or viral infection, but may be seen rarely in adults. OBSERVATIONS A 50-year-old woman presented with loss of visual acuity of the left eye. Magnetic resonance imaging (MRI) revealed an intra- and suprasellar mass, which was removed successfully. On postoperative day 1, MRI showed gross total resection of the lesion and no surgery-related complications. On postoperative day 2, the patient presented with a progressive left-sided hemiparesis, hemineglect, and decline of cognitive performance. MRI showed white matter edema in both hemispheres. Cerebrospinal fluid analysis revealed mixed pleocytosis (355/µL) without further evidence of infection. In synopsis of the findings, ADEM was diagnosed and treated with intravenous immunoglobulins. Shortly thereafter, the patient recovered, and no sensorimotor deficits were detected in the follow-up examination. LESSONS Pituitary gland pathologies are commonly treated by transsphenoidal surgery, with only minor risks for complications. A case of ADEM after craniopharyngioma resection has not been published before and should be considered in case of progressive neurological deterioration with multiple white matter lesions.

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Endothelial damage, vascular bagging and remodeling of the microvascular bed in human microangiopathy with deep white matter lesions

Acta Neuropathologica Communications ◽

10.1186/s40478-018-0632-z ◽

2018 ◽

Vol 6 (1) ◽

Cited By ~ 7

Author(s):

Karin M. E. Forsberg ◽

Yingshuang Zhang ◽

Johanna Reiners ◽

Martina Ander ◽

Alexandra Niedermayer ◽

...

Keyword(s):

White Matter ◽

White Matter Lesions ◽

Endothelial Damage ◽

Deep White Matter

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Neurological manifestations of coronavirus infections: a systematic review

10.1101/2020.08.26.20182196 ◽

2020 ◽

Author(s):

Jesper Almqvist ◽

Tobias Granberg ◽

Antonios Tzortzakakis ◽

Stefanos Klironomos ◽

Evangelia Kollia ◽

...

Keyword(s):

Systematic Review ◽

Cerebrovascular Disorders ◽

Acute Disseminated Encephalomyelitis ◽

Neurological Complications ◽

Diagnostic Workup ◽

Neurological Manifestations ◽

Chronic Disability ◽

Neuropsychiatric Complications ◽

Immune Mediated ◽

Depth Analysis

In order to optimize diagnostic workup of the current severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic, we systematically reviewed neurological and neuroradiological manifestations of SARS-CoV-2 and all other known human coronavirus species (HCoV). Which lessons can we learn? We identified relevant publications (until July 26h 2020) using systematic searches in PubMed, Web of Science and Ovid EMBASE with predefined search strings. A total of 4571 unique publications were retrieved, out of which 378 publications were selected for in-depth analysis by two raters, including a total of 17549 (out of which were 14418 SARS-CoV-2) patients. Neurological complications and associated neuroradiological manifestations are prevalent for all HCoVs (HCoV-229E, HKU1, NL63, OC43, Middle East respiratory syndrome (MERS)-CoV, SARS-CoV-1 and SARS-CoV-2). Moreover, there are similarities in symptomatology across different HCoVs, particularly between SARS-CoV-1 and SARS-CoV-2. Common neurological manifestations include fatigue, headache and smell/taste disorders. Additionally, clinicians need to be attentive for at least five classes of neurological complications: (1) Cerebrovascular disorders including ischemic stroke and macro/micro-hemorrhages, (2) encephalopathies, (3) para-/postinfectious immune-mediated complications such as Guillain-Barre syndrome and acute disseminated encephalomyelitis, (4) (meningo-)encephalitis, potentially with concomitant seizures and (5) neuropsychiatric complications such as psychosis and mood disorders. Our systematic review highlights the need for vigilance regarding neurological complications in patients infected by SARS-CoV-2 and other HCoVs, especially since some complications may result in chronic disability. Neuroimaging protocols should be designed to specifically screen for these complications. Therefore, we propose practical imaging guidelines to facilitate the diagnostic workup and monitoring of patients infected with HCoVs.

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