The Problem of Questionable Dystonia in the Diagnosis of ‘Essential Tremor-Plus’

In a recent consensus statement on tremor, the task force of the International Parkinson and Movement Disorder Society proposed a new term, ‘essential tremor-plus (ET-plus)’ which includes patients with the characteristics of essential tremor (ET) and additional soft neurological signs of uncertain significance such as questionable dystonic posturing. The clinical interpretation of questionable dystonia has been left to the investigator. The consensus statement also stated that the ET-plus syndrome does not include other clearly defined syndromes like dystonic tremor. However, the boundary between questionable dystonia and definite dystonia is not distinct leading to diagnostic uncertainty in a clinical setting. A similar case may be classified as ET-plus by one observer and dystonic tremor by another. Following the new definition, many studies have reclassified their ET cohort, and they have highlighted the problem of defining questionable dystonia in the diagnosis of ET plus. ET-plus is likely to be a mixture of patients that actually have dystonia and those that don’t, and clinically all we can do is to be suspicious that there might be dystonia. For example, it is not clear whether we should consider spooning and index finger pointing as a sign of questionable or definite dystonia. There are major research and possible therapeutic implications of questionable dystonia in the diagnosis of ET-plus. The concept of ET-plus is extremely difficult to implement without definite guidelines. The resolution will need a biomarker such as physiology or imaging.