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This review focuses on recent findings on the aetiological, clinical, pathological and genetic heterogeneity of essential tremor and new therapeutic approaches.Although essential tremor is one of the most common movement disorders, understanding of the causes and mechanisms of the disease is still very limited. Studies on the clinical presentation of essential tremor have expanded the clinical dimension, now including nontremor manifestations such as cerebellar signs, neuropsychological characteristics, distinct personality traits and behavioural symptoms. Results of neuropathologic and imaging studies are conflicting, with hints of neurodegeneration or a nondegenerative disturbance of functional circuits or receptors. Genetic heterogeneity of essential tremor has been demonstrated by linkage to three different chromosomal loci so far, and several negative genetic studies. New animal models are reinforcing previous hypotheses about γ-aminobutyrate (GABA)-ergic mechanisms in essential tremor. New therapeutic agents for essential tremor have been tested and demonstrated to be partly effective.The traditional view of essential tremor as a single disease entity has been replaced with the concept that this disorder is a complex and heterogeneous disease. Heterogeneity of the condition, and lack of diagnostic criteria and objective diagnostic tests add to this problem. Many conflicting results may be due to differences in patient selection.