While several paediatric NSAS features are similar to the adult population, some important differences could be highlighted (Table 2B), the most striking being the rarer paraneoplastic aetiology in children (5/94, 5.3% of the whole cohort) (1–5), especially in anti-amphiphysin autoimmunity (where >80% of adult have an associated malignancy vs. 0/4 of our included pediatric patients), but except for anti-mGluR5 syndromes (associated with Hodgkin lymphoma in 3/4 children identified in our review). This evidence concerns the gene GRM5 and Down syndrome.