Autoimmune encephalitis: suspicion in clinical practice and mimics

Costa, Diogo; Sardoeira, Ana; Carneiro, Paula; Neves, Esmeralda; Santos, Ernestina; Silva, Ana Martins da; Samões, Raquel

doi:10.1016/j.jneuroim.2022.577824

Cited by 5 publications

(12 citation statements)

References 21 publications

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“…In two later studies 4,5 the sensitivity of Graus' criteria did not exceed 80%, and the specificity was approximately 84% to 94%, with an established linear relationship between sensitivity and AIE progression. 4 In another two retrospective studies that were conducted in leading clinics in the USA and specialist centers in Portugal, the numbers of mimicking conditions were 107 of 393 in the study by Flanagan et al 12 and 26 of 39 in the study by Costa et al, 5 and similar studies in other countries were lacking. Additionally, the prevalence of mimicking conditions greatly surpassed that of AIE.…”

Section: Discussionmentioning

confidence: 91%

“…(except for 83 patients in a retrospective study in which the final diagnosis was not mentioned). 5 A total of 356 patients were included, with a slight female preponderance. The patient age ranged between 3 and 93 years (Table 1).…”

Section: Resultsmentioning

confidence: 99%

“…11 Later, this term expanded to be a part of AIE. 5 In this context, AIE consists of a set of neuropathies caused by an immune-mediated process and shares the presence of gray matter inflammation in the brain, which may extend to involve the white matter, meninges, and spinal cord. 2 According to the definition, AIE can be classified anatomically depending on the location (limbic, cortical/subcortical, striatal, diencephalic, brainstem, cerebellar, encephalomyelitis, meningoencephalitis, or combined) 2 or pathologically depending on the antibody type and the immune process involved 1 (cytotoxic T-cell-mediated antibodies against intracellular antigens vs antibodies against surface antigens that involve humoral immunity).…”

Section: Discussionmentioning

confidence: 99%

“…2 Therefore, the diagnosis is difficult and depends on a high clinical suspicion because, first, the progression of symptoms is gradual and sometimes it takes weeks before the clinical picture becomes clear. 5 Second, MRI results demonstrate nonspecific inflammatory findings or appear normal in most cases. 1 AIE is a rare disease (the incidence is approximately 3 to 9 per million) 5 but encompasses a high rate of misdiagnosed cases, 5 which may lead to medical errors because of unnecessary application of steroids and immunosuppressive therapy.…”

Section: Discussionmentioning

confidence: 99%

“…2 The Graus criteria for AIE are the most well-known criteria for AIE diagnosis, although two later studies showed that the sensitivity and specificity of these criteria did not exceed 80% and 84% to 94%, respectively. 4,5 Moreover, these criteria do not take into account that AIE tends to develop gradually before manifesting the classical clinical, laboratory, and radiological picture that fulfills the diagnosis, 5 and the diffuse inflammation caused by antibodies leads to different syndromes depending on its location. 2 Those syndromes may sometimes overlap, 2 and therefore it is difficult to establish a single criterion encompassing them all.…”

Section: Introductionmentioning

confidence: 99%

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Mimickers of autoimmune encephalitis: a literature review

Alshutaihi,

Mazketly,

Tabbakh

et al. 2024

J Int Med Res

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Autoimmune encephalitis (AIE) is a rapid, progressive neurological disorder characterized by nervous system inflammation. While the Graus criteria are the best known criteria for AIE diagnosis, other differential diagnoses meeting the Graus criteria must be considered before management. This narrative review discusses the most common etiologies that resemble AIE. We suggest routine exclusion of mimickers meeting the Graus criteria before confirming an AIE diagnosis. We reviewed 28 studies including 356 patients. The main initial diagnosis was AIE, then paraneoplastic limbic encephalitis and anti-N-methyl-D-aspartate receptor encephalitis. Only 194 patients met the possible Graus criteria. The most frequent conditions among the total population were dementia, other neurodegenerative diseases, and psychiatric and functional neurological disorders. AIE is often misdiagnosed, leading to unnecessary treatment. Despite publication of the Graus criteria, medical cases mimicking this condition are being published. Many neurological diseases entering the differential diagnosis of AIE could be excluded through a detailed history, neurological examination, laboratory analysis, and other investigations, including cerebrospinal fluid and brain magnetic resonance imaging. However, some differential diagnoses complied with the possible Graus criteria, with some having concurrent antineuronal antibodies, which were considered true mimickers. AIE diagnosis suspicion is primarily clinical, but a definitive diagnosis requires various diagnostic tools.

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Section: Discussionmentioning

confidence: 91%

Section: Resultsmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

Mimickers of autoimmune encephalitis: a literature review

Alshutaihi,

Mazketly,

Tabbakh

et al. 2024

J Int Med Res

View full text Add to dashboard Cite

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Recent advances in autoimmune encephalitis

Ferreira,

Disserol,

de Freitas Dias

et al. 2024

Arq Neuropsiquiatr

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Since the description of autoimmune encephalitis (AE) associated with N-methyl-D-aspartate receptor antibodies (anti-NMDARE) in 2007, more than 12 other clinical syndromes and antibodies have been reported. In this article, we review recent advances in pathophysiology, genetics, diagnosis pitfalls, and clinical phenotypes of AE associated with cell surface antibodies and anti-GAD associated neurological syndromes. Genetic studies reported human leukocyte antigen (HLA) associations for anti-LGI1, anti-Caspr2, anti-IgLON5, and anti-GAD. Follow-up studies characterized cognitive dysfunction, psychiatric symptoms, sleep disorders, and adaptative behavior dysfunction, mainly for anti-NMDARE. Late-onset anti-NMDARE and anti- GABA-B receptor (GABA-BR) encephalitis patients were described to have worse prognoses and different tumor associations. Additionally, the clinical spectrum of anti-LGI1, anti-AMPAR, anti-CASPR2, and anti-IgLON5 was expanded, comprising new differential diagnoses. The diagnostic criteria for AE were adapted to the pediatric population, and a diagnostic algorithm was proposed, considering potential mimics and misdiagnosis. We also review the limitations of commercial assays for AE and treatment recommendations, as well as clinical scales for short and long-term assessment of AE patients, along with cognitive evaluation.

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Mimics of Autoimmune Encephalitis

Van Steenhoven,

de Vries,

Bruijstens

et al. 2023

Neurol Neuroimmunol Neuroinflamm

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Background and ObjectivesThe clinical criteria for autoimmune encephalitis (AE) were proposed by Graus et al. in 2016. In this study, the AE criteria were validated in the real world, and common AE mimics were described. In addition, criteria for probable anti-LGI1 encephalitis were proposed and validated.MethodsIn this retrospective cohort study, patients referred to our national referral center with suspicion of AE and specific neuroinflammatory disorders with similar clinical presentations were included from July 2016 to December 2019. Exclusion criteria were pure cerebellar or peripheral nerve system disorders. All patients were evaluated according to the AE criteria.ResultsIn total, 239 patients were included (56% female; median age 42 years, range 1–85). AE was diagnosed in 104 patients (44%) and AE mimics in 109 patients (46%). The most common AE mimics and misdiagnoses were neuroinflammatory CNS disorders (26%), psychiatric disorders (19%), epilepsy with a noninflammatory cause (13%), CNS infections (7%), neurodegenerative diseases (7%), and CNS neoplasms (6%). Common confounding factors were mesiotemporal lesions on brain MRI (17%) and false-positive antibodies in serum (12%). Additional mesiotemporal features (involvement extralimbic structures, enhancement, diffusion restriction) were observed more frequently in AE mimics compared with AE (61% vs 24%;p= 0.005). AE criteria showed the following sensitivity and specificity: possible AE, 83% (95% CI 74–89) and 27% (95% CI 20–36); definite autoimmune limbic encephalitis (LE), 10% (95% CI 5–17) and 98% (95% CI 94–100); and probable anti-NMDAR encephalitis, 50% (95% CI 26–74) and 96% (95% CI 92–98), respectively. Specificity of the criteria for probable seronegative AE was 99% (95% CI 96–100). The newly proposed criteria for probable anti-LGI1 encephalitis showed a sensitivity of 66% (95% CI 47–81) and specificity of 96% (95% CI 93–98).DiscussionAE mimics occur frequently. Common pitfalls in AE misdiagnosis are mesiotemporal lesions (predominantly with atypical features) and false-positive serum antibodies. As expected, the specificity of the criteria for possible AE is low because these criteria represent the minimal requirements for entry in the diagnostic algorithm for AE. Criteria for probable AE (-LGI1, -NMDAR, seronegative) and definite autoimmune LE are applicable for decisions on immunotherapy in early disease stage, as specificity is high.

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Autoimmune encephalitis: suspicion in clinical practice and mimics

Cited by 5 publications

References 21 publications

Mimickers of autoimmune encephalitis: a literature review

Mimickers of autoimmune encephalitis: a literature review

Recent advances in autoimmune encephalitis

Mimics of Autoimmune Encephalitis

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