Introduction: Association between Motor Neuron Disease (MND) and neoplastic processes is rarely described. It is unclear whether MND occurs as a paraneoplastic syndrome. Methods: We retrospectively analysed 35 patients with MND followed in our Hospital from January 2013 to December 2016. Results: A neoplastic process was found in four patients. All of them had definite clinical and electromyographic criteria supporting MND according to standard Awaji criteria. Results: Onconeuronal antibodies were positive in two patients (anti-Yo and anti-PNMA2). The types of tumour were variable: two breast cancers, one lung cancer and one thyroid Hürthle cell neoplasm. Discussion: In our cohort, 11.4% of MND patients were diagnosed with a neoplastic process being a rather high frequency of co-occurrence. Treating the neoplastic process may potentially halt or reverse the progression of MND.

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