We report a 68-year-old woman who developed refractory non-convulsive generalized status epilepticus secondary to anti-Hu antibodies, detected by immunofluorescence and confirmed by Western immunoblotting. The patient presented with rapidly evolving impairment in consciousness and electroencephalographic evidence of lateralized pseudoperiodic sharp-wave discharges. Ataxia and sensory neuropathy developed within the first two weeks. To our knowledge, this is the first description of a very rapidly progressive non-convulsive status epilepticus of paraneoplastic origin. Serum anti-Hu antibodies deserve to be considered among the investigations required in the evaluation of rapidly progressive epileptic syndromes even when little or no imaging abnormalities are found.