Essential tremor (ET), the most common form of pathological tremor, aff ects up to 4% of the population. Accord ing to the traditional concept, ET is a monosymp tomatic benign clinical entity where tremor is associated with longevity and positive family history. Despite the high prevalence and suspected genetic component, research failed so far to fi nd a causative gene mutation for ET. The main reason is probably related to an imperfectly bounded nosological concept. There are still uncertainties around clinical defi nition of ET, which does not include isolated head tremor, voice tremor or task-specifi c tremors that often overlap with dystonic tremor. Moreover, numerous studies have shown the incidence of parkinsonism, cerebellar signs and cognitive disorders in ET. Age of onset turns out as a key factor, characterized by two peaks in the adolescence and in the old age. The classical concept of ET corresponds to early onset dis ease characterized by a positive family history, more benign progression, and relief after alcohol. Conversely, so called aging-related tremor is usually characterized by late-onset, sporadic occurrence, rapid progression, and also by cognitive dysfunction and other symp toms and signs suggestive of neurodegenerative disorder. Thus, rather than a monosymp tomatic nosological entity, ET appears as a group of dis eases featur ing action tremor. Besides unclear etiology, the possible contribution of neurodegenerative processes remains an open question in the pathogenesis of ET. Thereafter, a thorough description of the individual phenotype along with the methods of genome-wide association studies can contribute to fi nd ing causal gene polymorphisms in ET and to elucidate the pathogenesis of various forms of the dis ease. [ABSTRACT FROM AUTHOR]