The recent report of a single atypical case of sporadic ALS manifesting with predominantly upper motor neuron dysfunction with extrapyramidal features who at autopsy demonstrated pyramidal neuron FUS basophilic inclusions that colocalized with AT8 tau immunoreactivity in motor neurons, in addition to a 4R predominant tauopathy, provides support for such a proposed role of FUS in contributing to alterations in tau metabolism in ALS (104). The gene discussed is FUS; the disease is tauopathy.