Posttransplant autoimmune encephalitis

Cohen, Devon A.; López-Chiriboga, A. Sebastian; Pittock, Sean J.; Gadoth, Avi; Ζεκερίδου, Αναστασία; Boilson, Barry A.; Poterucha, John J.; Wilton, Katelynn M.; Lin, Yi; Flanagan, Eoin P.

doi:10.1212/nxi.0000000000000497

Cited by 26 publications

(20 citation statements)

References 6 publications

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“…It is possibly due to imbalance between B- and T-cell as anti-rejection immunosuppression are predominantly T-cell targeting treatments. Testing neural antibodies in this scenario is reasonable and the addition of antibody depleting treatments (plasma exchange, rituximab) in addition to maintenance anti-rejection immunosuppression should be considered ( 119 ).…”

Section: Diagnosis Of Antibody-mediated Cns Disordersmentioning

confidence: 99%

Antibody-Mediated Autoimmune Diseases of the CNS: Challenges and Approaches to Diagnosis and Management

Sechi

Flanagan

2021

Front. Neurol.

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Antibody-mediated disorders of the central nervous system (CNS) are increasingly recognized as neurologic disorders that can be severe and even life-threatening but with the potential for reversibility with appropriate treatment. The expanding spectrum of newly identified autoantibodies targeting glial or neuronal (neural) antigens and associated clinical syndromes (ranging from autoimmune encephalitis to CNS demyelination) has increased diagnostic precision, and allowed critical reinterpretation of non-specific neurological syndromes historically associated with systemic disorders (e.g., Hashimoto encephalopathy). The intracellular vs. cell-surface or synaptic location of the different neural autoantibody targets often helps to predict the clinical characteristics, potential cancer association, and treatment response of the associated syndromes. In particular, autoantibodies targeting intracellular antigens (traditionally termed onconeural autoantibodies) are often associated with cancers, rarely respond well to immunosuppression and have a poor outcome, although exceptions exist. Detection of neural autoantibodies with accurate laboratory assays in patients with compatible clinical-MRI phenotypes allows a definite diagnosis of antibody-mediated CNS disorders, with important therapeutic and prognostic implications. Antibody-mediated CNS disorders are rare, and reliable autoantibody identification is highly dependent on the technique used for detection and pre-test probability. As a consequence, indiscriminate neural autoantibody testing among patients with more common neurologic disorders (e.g., epilepsy, dementia) will necessarily increase the risk of false positivity, so that recognition of high-risk clinical-MRI phenotypes is crucial. A number of emerging clinical settings have recently been recognized to favor development of CNS autoimmunity. These include antibody-mediated CNS disorders following herpes simplex virus encephalitis or occurring in a post-transplant setting, and neurological autoimmunity triggered by TNFα inhibitors or immune checkpoint inhibitors for cancer treatment. Awareness of the range of clinical and radiological manifestations associated with different neural autoantibodies, and the specific settings where autoimmune CNS disorders may occur is crucial to allow rapid diagnosis and early initiation of treatment.

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Section: Diagnosis Of Antibody-mediated Cns Disordersmentioning

confidence: 99%

Antibody-Mediated Autoimmune Diseases of the CNS: Challenges and Approaches to Diagnosis and Management

Sechi

Flanagan

2021

Front. Neurol.

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“…This is thought to be due to an imbalance between T and B cell immunosuppression that may result in systemic or neurological autoimmunity. 2 …”

Section: Emerging Clinical Settingsmentioning

confidence: 99%

“…These disorders were initially recognized to occur as an idiopathic autoimmune phenomenon or as a cancer-related immune response (paraneoplastic disorder). It is now recognized that autoimmune dementias can occur in other novel settings, such as post-herpes virus encephalitis, 1 post-transplant, 2 or after cancer treatment with immune checkpoint inhibitors (ICI). 3 The expanding range of clinical scenarios in which autoimmune dementia can arise emphasizes the need for all neurologists to recognize and understand these disorders.…”

Section: Introductionmentioning

confidence: 99%

Autoimmune encephalopathies presenting as dementia of subacute onset and rapid progression

Banks

Sechi

Flanagan

2021

Ther Adv Neurol Disord

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The terms autoimmune dementia and autoimmune encephalopathy may be used interchangeably; autoimmune dementia is used here to emphasize its consideration in young-onset dementia, dementia with a subacute onset, and rapidly progressive dementia. Given their potential for reversibility, it is important to distinguish the rare autoimmune dementias from the much more common neurodegenerative dementias. The presence of certain clinical features [e.g. facio-brachial dystonic seizures that accompany anti-leucine-rich-glioma-inactivated-1 (LGI1) encephalitis that can mimic myoclonus] can be a major clue to the diagnosis. When possible, objective assessment of cognition with bedside testing or neuropsychological testing is useful to determine the degree of abnormality and serve as a baseline from which immunotherapy response can be judged. Magnetic resonance imaging (MRI) head and cerebrospinal fluid (CSF) analysis are useful to assess for inflammation that can support an autoimmune etiology. Assessing for neural autoantibody diagnostic biomarkers in serum and CSF in those with suggestive features can help confirm the diagnosis and guide cancer search in paraneoplastic autoimmune dementia. However, broad screening for neural antibodies in elderly patients with an insidious dementia is not recommended. Moreover, there are pitfalls to antibody testing that should be recognized and the high frequency of some antibodies in the general population limit their diagnostic utility [e.g., anti-thyroid peroxidase (TPO) antibodies]. Once the diagnosis is confirmed, both acute and maintenance immunotherapy can be utilized and treatment choice varies depending on the accompanying neural antibody present and the presence or absence of cancer. The target of the neural antibody biomarker may help predict treatment response and prognosis, with antibodies to cell-surface or synaptic antigens more responsive to immunotherapy and yielding a better overall prognosis than those with antibodies to intracellular targets. Neurologists should be aware that autoimmune dementias and encephalopathies are increasingly recognized in novel settings, including post herpes virus encephalitis and following immune-checkpoint inhibitor use.

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“…The mechanisms behind loss of tolerance despite immunosuppression associated with transplant are poorly understood, but an association with Epstein–Barr virus (EBV) has been proposed, with a few cases reporting positive EBV testing in the CSF corroborating an association. 54 – 56 …”

Section: Post-infectious Autoimmune Neurological Disordersmentioning

confidence: 99%

Post-infectious neurological disorders

Blackburn

Wang

2020

Ther Adv Neurol Disord

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A multitude of environmental factors can result in breakdown of immune tolerance in susceptible hosts. Infectious pathogens are among the most important environmental triggers in the pathogenesis of autoimmunity. Certain autoimmune disorders have a strong association with specific infections. Several neurological autoimmune disorders are thought to occur through post-infectious mechanisms. In this review, we discuss the proposed mechanisms underlying pathogen-induced autoimmunity, and highlight the clinical presentation and treatment of several post-infectious autoimmune neurological disorders. We also highlight post-infectious neurological disorders in the setting of recent outbreaks.

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Posttransplant autoimmune encephalitis

Cited by 26 publications

References 6 publications

Antibody-Mediated Autoimmune Diseases of the CNS: Challenges and Approaches to Diagnosis and Management

Antibody-Mediated Autoimmune Diseases of the CNS: Challenges and Approaches to Diagnosis and Management

Autoimmune encephalopathies presenting as dementia of subacute onset and rapid progression

Post-infectious neurological disorders

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