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The past decade has seen major changes in the field of infectious diseases. In particular, many new infections of the nervous system have been recognised, including the lethal infections of Enterovirus 71, and the Nipah and West Nile viruses. Increased interest in prion diseases has occurred, following the recognition of animal-to-human transmission in Europe. Familiar bacteria such as the pneumococcus continue to cause problems due to increasing resistance to multiple antibiotics. Furthermore, human immunodeficiency virus-infected and other immunocompromised patients are under the constant threat of opportunistic infections, many of which are targeted towards the brain and spinal cord. This paper reviews the changing world of nervous system infections, highlighting some of the most significant recent developments.