Autoimmune Encephalitis—Misdiagnosis, Misconceptions, and How to Avoid Them

Dalmau, Josep; Graus, Francesc

doi:10.1001/jamaneurol.2022.4154

Cited by 27 publications

(36 citation statements)

References 15 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…In addition to commercially available assays, tissue-based assays and live cell-based assays are extremely useful to detect novel reactivities that may otherwise be missed; thus, referring samples to expert reference laboratories is of utmost importance. 35,36 Finally, the application of the 2021 diagnostic criteria for PNS demonstrates that only 5 of 21 patients fulfill the criteria for definite PNS, whereas most of them are classified either as probable or as non-PNS. The absence of autoantibodies or the presence of atypical oncological accompaniments justifies the different levels of diagnostic certainty encountered.…”

Section: Discussionmentioning

confidence: 99%

Autoimmune Movement Disorders Complicating Treatment with Immune Checkpoint Inhibitors

Dinoto,

Trentinaglia,

Carta

et al. 2024

Movement Disord Clin Pract

View full text Add to dashboard Cite

BackgroundImmune checkpoint inhibitors (ICI) may trigger autoimmune neurological conditions, including movement disorders (MD).ObjectivesThe aim of this study was to characterize MDs occurring as immune‐related adverse events (irAEs) of ICIs.MethodsA systematic literature review of case reports/series of MDs as irAEs of ICIs was performed.ResultsOf 5682 eligible papers, 26 articles with 28 patients were included. MDs occur as a rare complication of cancer immunotherapy with heterogeneous clinical presentations and in most cases in association with other irAEs. Inflammatory basal ganglia T2/fluid attenuated inversion recovery abnormalities are rarely observed, but brain imaging is frequently unrevealing. Cerebrospinal fluid findings are frequently suggestive of inflammation. Half of cases are associated with a wide range of autoantibodies. Steroids and ICI withdrawal usually lead to improvement, even though some patients experienced relapses or a severe clinical course.ConclusionMDs are a rare complication of ICIs that should be promptly recognized to offer patients a correct diagnosis and treatment.

show abstract

Section: Discussionmentioning

confidence: 99%

Autoimmune Movement Disorders Complicating Treatment with Immune Checkpoint Inhibitors

Dinoto,

Trentinaglia,

Carta

et al. 2024

Movement Disord Clin Pract

View full text Add to dashboard Cite

show abstract

“…The clinical onset of antibody-mediated encephalitides is frequently subacute (i.e., ≤ 3 months), although it can be more insidious in some cases, for example in LGI1, contactin-associated protein-like 2 (Caspr2) or Ig-like domain-containing protein 5 (IgLON5) encephalitis [73 ▪▪ ]. Neurological symptoms are usually severe, and often require intensive care management.…”

Section: Introductionmentioning

confidence: 99%

Mechanisms of autoimmune encephalitis

Papi,

Milano,

Spatola

2024

Current Opinion in Neurology

View full text Add to dashboard Cite

Purpose of review To provide an overview of the pathogenic mechanisms involved in autoimmune encephalitides mediated by antibodies against neuronal surface antigens, with a focus on NMDAR and LGI1 encephalitis. Recent findings In antibody-mediated encephalitides, binding of IgG antibodies to neuronal surface antigens results in different pathogenic effects depending on the type of antibody, IgG subclass and epitope specificity. NMDAR IgG1 antibodies cause crosslinking and internalization of the target, synaptic and brain circuitry alterations, as well as alterations of NMDAR expressing oligodendrocytes, suggesting a link with white matter lesions observed in MRI studies. LGI1 IgG4 antibodies, instead, induce neuronal dysfunction by disrupting the interaction with cognate proteins and altering AMPAR-mediated signaling. In-vitro findings have been corroborated by memory and behavioral changes in animal models obtained by passive transfer of patients’ antibodies or active immunization. These models have been fundamental to identify targets for innovative therapeutic strategies, aimed at counteracting or preventing antibody effects, such as the use of soluble ephrin-B2, NMDAR modulators (e.g., pregnenolone, SGE-301) or chimeric autoantibody receptor T cells (CAART) in models of NMDAR encephalitis. Summary A deep understanding of the pathogenic mechanisms underlying antibody-mediated encephalitides is crucial for the development of new therapeutic approaches targeting brain autoimmunity.

show abstract

“…10,11 Although AIE is considered a rare disease, with estimated prevalence of 7-13 cases per 100.000 inhabitants, 5 it is a frequent differential diagnosis of many neurological and psychiatric conditions such as first psychosis episode, catatonia, rapidly progressive dementia, developmental delay, refractory status epilepticus, infectious encephalitis, and abnormal movement disorders. 6,9 Given its treatable nature, many neurologists face the problem of inadequate diagnosis by treating empirically patients with steroids and IVIG, an approach that may add cost to the health care system.…”

mentioning

confidence: 99%

“…Optimal diagnostic practices recommend screening serum and CSF for antibodies using the two techniques due to variations in sensitivity depending on the sample. 9 Unfortunately, despite specific clinical phenotypes and diagnostic clues, antibody positivity cannot be reliably predicted based solely on clinical presentation and ancillary investigations.…”

mentioning

confidence: 99%

Diagnosis and treatment of autoimmune encephalitis in Brazil: an urgent call to action

Dutra

2024

Arq Neuropsiquiatr

View full text Add to dashboard Cite

Autoimmune Encephalitis—Misdiagnosis, Misconceptions, and How to Avoid Them

Cited by 27 publications

References 15 publications

Autoimmune Movement Disorders Complicating Treatment with Immune Checkpoint Inhibitors

Autoimmune Movement Disorders Complicating Treatment with Immune Checkpoint Inhibitors

Mechanisms of autoimmune encephalitis

Diagnosis and treatment of autoimmune encephalitis in Brazil: an urgent call to action

Contact Info

Product

Resources

About