Abstract
Autoimmune encephalitis (AIE) poses a diagnostic challenge due to its heterogeneous clinical presentation, which overlaps with various neurological and psychiatric diseases. During the diagnostic work-up, cerebrospinal fluid (CSF) is routinely obtained, allowing for differential diagnostics as well as for the determination of antibody subclasses and specificities. In this monocentric cohort study, we describe initial and serial CSF findings of 33 patients diagnosed with antibody-associated AIE (LGI1 (n=8), NMDA (n=7), CASPR2 (n=3), IgLON5 (n=3), AMPAR (n=1), GAD65/67 (n=4), Yo (n=3), Ma-1/2 (n=2), CV2 (n=2)). Routine CSF parameters of 12.1% of AIE patients were in normal ranges, while 60.6% showed elevated protein levels and 45.4% had intrathecal oligoclonal bands (OCBs). Repeated CSF analyses showed a trend towards normalization of initial pathological CSF findings, while relapses were more likely to be associated with increased cell counts and total protein levels. OCB status conversion in anti-NMDARE patients coincided with clinical improvement. In summary, we show that in routine CSF analysis at diagnosis, a considerable number of patients with AIE did not exhibit alteration in the CSF and therefore, diagnosis may be delayed if antibody testing is not performed. Moreover, OCB status in anti-NMDAR AIE patients could represent a potential prognostic biomarker, however further studies are necessary to validate these exploratory findings.
Highlights
Autoimmune encephalitides (AIEs) are an inflammatory disease spectrum affecting the central nervous system (CNS) and can be difficult to diagnose accurately and promptly due to their different clinical presentation [1, 2]
cerebrospinal fluid (CSF) analysis is a routine examination in the workup of patients with suspected AIE
We present CSF findings of a monocentric cohort of patients with confirmed AIE
Summary
Autoimmune encephalitides (AIEs) are an inflammatory disease spectrum affecting the central nervous system (CNS) and can be difficult to diagnose accurately and promptly due to their different clinical presentation [1, 2]. A large number of auto-antibodies with either direct pathogenic effects or implications of yet unknown significance have been discovered and associated with different clinical and neuropathological entities in the spectrum of AIEs [3]. Immunotherapy is associated with better outcomes, especially when patients mount an antibody response against surface antigens in contrast to antibodies binding to intracellular antigens [4, 5]. Diagnostic assessment for the suspected antibodies can take up to several weeks and is not accessible everywhere, which may delay diagnosis and start of immunotherapy
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