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Since its initial description 10\u00a0years ago, an increasing number of autopsies has led to the observation of a broader spectrum of neuropathologies underlying a particular constellation of clinical symptoms. In this study, we describe the neuropathological findings in 22 patients with anti-IgLON5 disease from 9 different European centers. In 15 patients (68%), we observed a hypothalamic and brainstem-predominant tauopathy of varying severity in which the original research neuropathological criteria were readily applicable. This pathology was observed in younger patients (median age at onset 61\u00a0years) with a long disease duration (median 9\u00a0years). In contrast, in 7 (32%) patients, the originally described brainstem tauopathy was nearly absent or only minimal in the form of delicate threads, despite mild-to-moderate neurodegenerative features, consistent clinical symptoms and the presence of anti-IgLON5 antibodies in CSF and serum. These patients were older at onset (median 79\u00a0years) and had shorter disease duration (median\u2009&lt;\u20091\u00a0year). Overall, about one-third of the patients showed concomitant TDP-43 pathology within the regions affected by tau pathology and\/or neurodegeneration. Based on these observations and in view of the spectrum of the tau burden in the core regions involved in the disease, we propose a simple staging system: <jats:italic>stage 1<\/jats:italic> mild neurodegeneration without overt or only minimal tau pathology,<jats:italic> stage 2<\/jats:italic> moderate neurodegeneration and mild\/ moderate tauopathy and <jats:italic>stage 3<\/jats:italic> prominent neurodegeneration and tau pathology. This staging intends to reflect a potential (age- and time-dependent) progression of tau pathology, supporting the current notion that tau accumulation is a secondary phenomenon related to the presence of anti-IgLON5 antibodies in the CNS. Finally, we adapt the original research criteria of the anti-IgLON5 disease-related tauopathy to include the spectrum of pathologies observed in this larger postmortem series.<\/jats:p>","DOI":"10.1007\/s00401-024-02805-y","type":"journal-article","created":{"date-parts":[[2024,10,14]],"date-time":"2024-10-14T15:02:30Z","timestamp":1728918150000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":43,"title":["Neuropathological spectrum of anti-IgLON5 disease and stages of brainstem tau pathology: updated neuropathological research criteria of the disease-related 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