Diagnosing autoimmune limbic encephalitis

Budhram, Adrian; Leung, Andrew; Nicolle, Michael W.; Burneo, Jorge G.

doi:10.1503/cmaj.181548

Cited by 68 publications

(69 citation statements)

References 49 publications

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“…Unilateral FLAIR or diffusion-weighted hyperintensities in the medial temporal lobe were frequent and have been previously reported in one patient with COVID-19 (10). The latter is frequently observed in case of infectious encephalitis (especially with some viruses like herpes simplex virus, human herpesvirus 6, or Epstein-Barr virus) or in association with autoimmune limbic encephalitis (19).…”

Section: Neuroimaging Findingsmentioning

confidence: 61%

Brain MRI Findings in Severe COVID-19: A Retrospective Observational Study

Kremer¹,

Lersy²,

Sèze³

et al. 2020

Radiology

404

449

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is the seventh member of the family of coronaviruses that infect humans (1) and induces coronavirus disease 2019 (COVID-19). Human coronaviruses have neuroinvasive capacities and may be neurovirulent by two main mechanisms (2-4): viral replication into glial or neuronal cells of the brain or autoimmune reaction with a misdirected host immune response (5). Thus, a few cases of acute encephalitislike syndromes with human coronaviruses were reported in the past 2 decades (5-8). In regard to COVID-19, current data on central nervous system involvement are uncommon but growing (9-17), demonstrating the high frequency of neurologic symptoms. However, the delineation of a large cohort of confirmed brain MRI parenchymal signal abnormalities (excluding ischemic infarcts) related to COVID-19 has never been performed, and the underlying pathophysiologic mechanisms remain unknown. The purpose of the current study was to describe the neuroimaging findings (excluding ischemic infarcts) in patients with severe COVID-19 and report the clinicobiologic profile of these patients. Materials and Methods This retrospective observational national multicenter study was initiated by the French Society of Neuroradiology in collaboration with neurologists, intensivists, and infectious disease specialists and brought together 16 hospitals. The study was approved by the ethical committee of Strasbourg University Hospital (CE-2020-37) and was in accordance with the 1964 Helsinki Declaration and its later amendments. Because of the emergency in the context of the COVID-19 pandemic responsible for

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Section: Neuroimaging Findingsmentioning

confidence: 61%

Brain MRI Findings in Severe COVID-19: A Retrospective Observational Study

Kremer¹,

Lersy²,

Sèze³

et al. 2020

Radiology

404

449

View full text Add to dashboard Cite

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“…AA involved in autoimmune encephalitis are classified as group I antibodies (e. g., an-tiHU, antiCV2, antiglutamic acid decarboxylase, anti-Ma/Ta, antiglutamic acid decarboxylase) targeting intracellular neuronal antigens, are more closely associated with an underlying malignancy, and use the same cytotoxic T-cell mechanisms when targeting the intracellular neuronal antigens and onconeuronal antigens as part of the immune response to cancer [3][4][5][6]. Group II antibodies (N-methyl D-aspartate receptor, voltage-gated potassium channel, voltage-gated calcium channel, gamma-aminobutyric acid receptor, alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor) target cell surface neuronal antigens, are less likely to be associated with an underlying malignancy, and use more "restricted" humoral immune mechanisms of neurotoxicity that typically respond better to early immunomodulatory therapy [3][4][5][6]. In addition, typical NAD with gastrointestinal manifestation include the area postrema syndrome in neuromyelitis optica, the dipeptidyl-peptidase-like protein 6-associated encephalitis, and the autoimmune autonomic and enteric neuropathies [2,4,7].…”

Section: Discussionmentioning

confidence: 99%

“…In addition, typical NAD with gastrointestinal manifestation include the area postrema syndrome in neuromyelitis optica, the dipeptidyl-peptidase-like protein 6-associated encephalitis, and the autoimmune autonomic and enteric neuropathies [2,4,7]. Among these AA, ANNA-1 targeting intracellular neuronal antigens as part of the immune response is most frequently seen in paraneoplastic syndromes [3][4][5][6]. For example, ANNA-1 is associated in more than 95 % with small cell lung cancer [8] and SOX-1 in small cell lung cancer and Hodgkin disease [9].…”

Section: Discussionmentioning

confidence: 99%

“…AA-triggered gastrointestinal dysmotility [3][4][5][6][7] does not follow a specific pattern. Therefore, the type of gastrointestinal motility disturbances does not necessarily point to NAD.…”

Section: Discussionmentioning

confidence: 99%

“…For example, antibodies targeting chemoreceptors (such as the area postrema) or enteric neurons may result in severe vomiting, early satiety, dysphagia, or diarrhea [2]. The autoantibodies (AA) in autoimmune encephalitis affect not only CNS structures but also gastrointestinal functions [3][4][5][6][7]. Among these, limbic encephalitis is the most consistent finding, representing a spectrum of immune-mediated diseases involving various regions of the central nervous system such as the neocortex, striatum, hindbrain, spine, and peripheral nervous system [3][4][5][6].…”

Section: Introductionmentioning

confidence: 99%

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Autoimmune encephalitis and gastrointestinal dysmotility: achalasia, gastroparesis, and slow transit constipation

et al. 2020

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Background Neurological autoimmune disorders (NAD) are caused by autoimmune inflammation triggered by specific antibody subtypes. NAD may disturb the gut-brain axis at several levels including brain, spinal cord, peripheral, or enteric nervous system. Case report We present a case with antinuclear neuronal Hu (ANNA-1)- and antiglial nuclear (SOX-1) autoimmune antibody-positive limbic encephalitis and significant gastrointestinal dysmotility consisting of achalasia type II, gastroparesis, altered small intestinal interdigestive motility, and severe slow transit constipation. The autoantibodies of the patient’s serum labeled enteric neurons and interstitial cells of Cajal but no other cells in the gut wall. Achalasia was treated successfully by pneumatic cardia dilation and gastrointestinal dysmotility successfully with prucalopride. Conclusion NAD may disturb gastrointestinal motility by altering various levels of the gut-brain axis.

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Prominent cerebral veins on susceptibility‐weighted angiography in acute meningoencephalitis

Jung,

Park,

Joo

et al. 2023

Brain and Behavior

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Background and purposeWe have commonly observed prominent cerebral veins on susceptibility‐weighted angiography (SWAN) in acute meningoencephalitis. This study aimed to investigate the clinical significance of these findings.MethodsCerebral veins on SWAN of 98 patients with acute meningoencephalitis diagnosed from February 2016 through October 2020 were classified into three groups according to the degree of venous prominence (mild, 23; moderate, 53; and prominent, 22). Clinical variables and laboratory findings were compared between these groups. The influence of variables on the prediction of prominent cerebral veins was measured by random forest (RF) and gradient boosting machine (GBM).ResultsAs cerebral veins became more prominent, cerebrospinal fluid (CSF) glucose level decreased (69.61 ± 29.05 vs. 59.72 ± 22.57 vs. 48.36 ± 20.29 mg/dL, p = .01) and CSF protein level increased (100.73 ± 82.98 vs. 104.73 ± 70.99 vs. 159.12 ± 118.15 mg/dL, p = .03). The etiology of meningoencephalitis, neurological symptoms, and increased intracranial pressure (ICP) signs differed between groups (p < .05). RF and GBM demonstrated that CSF protein level was the variable with the highest power to predict the prominent cerebral vein (mean decrease in node impurity: 4.19, relative influence: 50.66).ConclusionThe presence of prominent cerebral veins on SWAN in acute meningoencephalitis was significantly associated with a low CSF glucose level and a high CSF protein level, as well as ICP. Thus, the visual grade of the cerebral veins on SWAN may be utilized for the management of patients with acute meningoencephalitis.

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Diagnosing autoimmune limbic encephalitis

Cited by 68 publications

References 49 publications

Brain MRI Findings in Severe COVID-19: A Retrospective Observational Study

Brain MRI Findings in Severe COVID-19: A Retrospective Observational Study

Autoimmune encephalitis and gastrointestinal dysmotility: achalasia, gastroparesis, and slow transit constipation

Prominent cerebral veins on susceptibility‐weighted angiography in acute meningoencephalitis

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