Acute infectious diseases of the nervous system are potentially life threatening, inherently carrying a high long-term morbidity and mortality. Therefore, the earliest possible diagnosis is absolutely essential. Examination of cerebrospinal fl uid frequently leads the way towards correct diagnosis and allows for focused antimicrobial and adjunctive therapy. This chapter deals with acute infectious diseases of the nervous system, diagnostic procedures being indispensable. Encephalitis, meningitis, poliomyelitis and polyradiculoneuritis are the most important clinical/neurological entities in case of viral infection. It is the acute bacterial meningitis for which the earliest possible diagnosis carries the most important prognostic implication. For this disease, the appropriate examination of the cerebrospinal fl uid (including glucose, cell count, lactate) is of utmost importance. Besides antimicrobial chemotherapy, the best possible adjunctive therapies are essential for acute bacterial meningitis. Fungal, protozoal and helminthic infections of the central nervous system are detailed with respect to diagnostic aspects and therapeutic implications (e.g. eosinophilic meningitis, radiculitis, etc.); for some of these diseases, e.g. cerebral malaria, a normal CSF leads the way to correct diagnosis in a patient with severe impairment of consciousness, high fever and history of exposure to Plasmodium falciparum , thereby easily mistaken for viral encephalitis or acute bacterial meningitis.