Background <p>Early recognition of autoimmune encephalitis (AE) is critical but remains challenging because of overlapping clinical features with viral encephalitis (VE) and delays in antibody testing. The UT-Hopkins Score was recently developed to assist in the clinical differentiation of AE from VE at presentation. We aimed to assess the sensitivity of this score in a large national cohort of antibody-mediated AE and across major antibody-defined subgroups.</p> Methods <p>We conducted a retrospective cohort study including 350 patients with antibody-mediated AE diagnosed according to international consensus criteria at the French Reference Center. Antibodies included anti-N-methyl-D-aspartate receptor (NMDAR), leucine-rich glioma-inactivated 1 (LGI1), contactin-associated protein-like 2 (CASPR2), γ-aminobutyric acid type B receptor (GABA<sub><i>B</i></sub>R), and glutamic acid decarboxylase 65 (GAD65). The UT-Hopkins Score was calculated using four binary variables. Sensitivity was assessed using a cutoff of ≥ 2 points and analyzed overall, in a reweighted model reflecting national antibody distribution, and across antibody subtypes.</p> Results <p>Overall, 90.3% (95% CI, 86.7–93.0) of patients scored ≥ 2. In the reweighted model, sensitivity increased to 92.9% (95% CI, 86.3–96.6). Sensitivity was highest in GAD65 (100%) and NMDAR (99%) subgroups, remained high in LGI1 (94%) and CASPR2 (90%), but was lower in GABA<sub><i>B</i></sub>R (56%), characterized by higher comorbidity burden, acute onset, fewer psychiatric or memory features, and robust CSF inflammation.</p> Conclusions <p>In this large cohort of antibody-mediated AE, the UT-Hopkins Score demonstrated high sensitivity across most antibody subtypes, supporting its use to detect autoimmune causes early in patients presenting with encephalitis.</p>

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Sensitivity of the UT-Hopkins Score in 350 patients with antibody-mediated autoimmune encephalitis

  • Pauline Dumez,
  • Marion Le Maréchal,
  • Marie Benaiteau,
  • Chloé Buttard,
  • Elise Peter,
  • Bastien Joubert,
  • Hanna Hutten,
  • Florian Lamblin,
  • Lucia Campetella,
  • Macarena Villagràn-Garcia,
  • Géraldine Picard,
  • John Probasco,
  • Arun Venkatesan,
  • Rodrigo Hasbun,
  • Jérôme Honnorat

摘要

Background

Early recognition of autoimmune encephalitis (AE) is critical but remains challenging because of overlapping clinical features with viral encephalitis (VE) and delays in antibody testing. The UT-Hopkins Score was recently developed to assist in the clinical differentiation of AE from VE at presentation. We aimed to assess the sensitivity of this score in a large national cohort of antibody-mediated AE and across major antibody-defined subgroups.

Methods

We conducted a retrospective cohort study including 350 patients with antibody-mediated AE diagnosed according to international consensus criteria at the French Reference Center. Antibodies included anti-N-methyl-D-aspartate receptor (NMDAR), leucine-rich glioma-inactivated 1 (LGI1), contactin-associated protein-like 2 (CASPR2), γ-aminobutyric acid type B receptor (GABABR), and glutamic acid decarboxylase 65 (GAD65). The UT-Hopkins Score was calculated using four binary variables. Sensitivity was assessed using a cutoff of ≥ 2 points and analyzed overall, in a reweighted model reflecting national antibody distribution, and across antibody subtypes.

Results

Overall, 90.3% (95% CI, 86.7–93.0) of patients scored ≥ 2. In the reweighted model, sensitivity increased to 92.9% (95% CI, 86.3–96.6). Sensitivity was highest in GAD65 (100%) and NMDAR (99%) subgroups, remained high in LGI1 (94%) and CASPR2 (90%), but was lower in GABABR (56%), characterized by higher comorbidity burden, acute onset, fewer psychiatric or memory features, and robust CSF inflammation.

Conclusions

In this large cohort of antibody-mediated AE, the UT-Hopkins Score demonstrated high sensitivity across most antibody subtypes, supporting its use to detect autoimmune causes early in patients presenting with encephalitis.