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Neuroleptic malignant syndrome is rare, but potentially fatal complication of treatment with neuroleptics and other psychotropic drugs. The authors review the relevant literature and discuss the incidence and possible etiology of this disorder. Most studies have confirmed the role of dopamine and other neuromediators in the etiology of neuroleptic malignant syndrome. Risk factors for neuroleptic malignant syndrome include previous episodes of neuroleptic malignant syndrome, dehydration, agitation, high dosage and parenteral administration of neuroleptics. Neuroleptic malignant syndrome is more frequent in patients with organic brain disease, and in subjects receiving lithium. Clinical symptoms include hyperthermia, muscular rigidity, disturbances of consciousness and autonomic dysfunction. Neuroleptic malignant syndrome with an uncomplicated course lasts about 7 to 10 days. Patient with neuroleptic malignant syndrome are given non-specific supportive treatment in intensive care units. Dopamine agonists and dantrolene are useful in more severe cases. According to the literature data, appropriate therapy has decreased mortality rates associated with neuroleptic malignant syndrome from 25% to 10% in the recent years.