Defining Dystonic Tremor

A strong association between dystonia and tremor has been known for more than a century. Two forms of tremor in dystonia are currently recognized: 1) dystonic tremor, which is tremor produced by dystonic muscle contraction and 2) tremor associated with dystonia, which is tremor in a body part that is not dystonic, but there is dystonia elsewhere. Both forms of tremor in dystonia frequently resemble essential tremor or another pure tremor syndrome (e.g., isolated head and voice tremors and task-specific writing tremor), and relationships among these tremor disorders have long been debated. Misdiagnosis is common because mild dystonia is frequently overlooked in patients with tremor. It is now clear that essential tremor is a syndrome, not a specific disease, and the use of essential tremor as a specific clinical diagnosis is arguably an impediment to elucidating this and other pure tremor syndromes and their relationship to dystonia. A new classification, primary tremor, is proposed and would be used for any disorder in which tremor is the sole or principal abnormality with no identifiable etiology other than possible genetic inheritance. This classification scheme would facilitate tremor research by moving the focus from the narrow question “Is it essential tremor?” to a broader consideration of what genetic and environmental factors cause primary tremor disorders, and how do they relate to dystonia and other neurological disorders.