Purpose of review: Essential tremor has not been defined or used consistently in clinical diagnosis and research. Other monosymptomatic disorders are often referred to as essential tremor variants.
Recent findings: There is now solid evidence that essential tremor, however defined, is a syndrome with multiple causes.
Summary: A new tremor classification scheme is being developed by the International Parkinson and Movement Disorder Society Task Force on Tremor. In this scheme, tremor in the absence of other neurological signs is called isolated tremor, and tremor in combination with other signs is called combined tremor. Many isolated and combined tremor syndromes can be defined on the basis of commonly recurring or unique clinical symptoms and signs, including historical features (age at onset, family history, and temporal evolution) and tremor characteristics (body distribution, activation condition, associated features). Essential tremor, however defined, is simply a syndrome and not a specific disease. Essential tremor should be defined and used consistently, or this term should be abandoned. As essential tremor is an arbitrarily defined syndrome, it makes no sense to refer to other tremors as variants of essential tremor or essential tremor syndromes.