Pearls &amp; Oy-sters: A rare presentation of Whipple disease

Kloek, Anne T.; Piet, Jurgen R.; Adriani, Kirsten S.

doi:10.1212/wnl.0000000000008976

Cited by 1 publication

(1 citation statement)

References 10 publications

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“…Bilateral mesiotemporal hyperintensities on T2/FLAIR images (E) and parenchymal enhancement in corresponding regions. (This patient was also published elsewhere by Kloek et al 48 ). (G) Neurofibromatosis type 1 (NF-1): 52-year-old male patient with a chronic course focal epilepsy and cognitive decline.…”

Section: Resultssupporting

confidence: 64%

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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Section: Resultssupporting

confidence: 64%