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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.

Resumo: A Granulomatose Eosinofílica com Poliangeíte (EGPA), previamente denominada Síndrome de Churg-Strauss, é uma vasculite associada ao anticorpo anti-citoplasma dos neutrófilos (ANCA) que atinge vasos de pequeno calibre. Os autores descrevem o caso de uma mulher com instalação subaguda de parestesias, disestesias, paraparésia crural a impossibilitar a marcha e lesões purpúricas no tornozelo. A electromiografia mostrou mononeuropatia múltipla com envolvimento dos nervos Mediano esquerdo, Cubital e Femoral direitos. Estudo analítico com eosinofilia e ANCA positivo. A biópsia cutânea mostrou vasculite necrotizante e infiltrado eosinofílico. Cumpre a definição de Chapel Hill Conference Consensus e reúne os critérios do American College of Rheumatology pelo que foi assumido o diagnóstico de EGPA. Iniciou corticoterapia e ciclofosfamida, com melhoria clínica e laboratorial. Este caso clínico ilustra a importância do reconhecimento do padrão da neuropatia periférica na apresentação desta entidade rara que pode ser incapacitante se houver atraso no diagnóstico e tratamento. Abstract: Eosinophilic Granulomatosis with Polyangiitis (EGPA), formerly known as Churg-Strauss Syndrome, is a anti-neutrophil cytoplasmic antibody (ANCA) associated vasculites affecting small vessels. We describe a women with subacute complaints of paresthesias, disesthesias, crural paraparesis with lost of gait and purpuric lesions on her ankle. Electromyogram revealed multiplex mononeuropathy with left median nerve, right ulnar and femoral nerves involvement. Laboratory analysis with eosinophilia and positive ANCA. She fulfilled the Chapel Hill Conference Consensus definition and the criteria of American College of Rheumatology for EGPA. Corticosteroids and cyclophosphamide were begun with good clinical and laboratorial response. This report ilustrates the importance of identifying the neuropatic pattern as initial manifestation of this rare condition that can cause significant disability if there is delay on the diagnosis and treatment.