Bayesian Interpretation of Essential Tremor Plus

Essential tremor (ET) plus is a new tremor classification that was introduced in 2018 by a task force of the International Parkinson and Movement Disorder Society. Patients with ET plus meet the criteria for ET but have one or more additional systemic or neurologic signs of uncertain significance or relevance to tremor (soft signs). Soft signs are not sufficient to diagnose another tremor syndrome or movement disorder, and soft signs in ET plus are known to have poor interrater reliability and low diagnostic sensitivity and specificity. Therefore, the clinical significance of ET plus must be interpreted probabilistically when judging whether a patient is more likely to have ET or a combined tremor syndrome, such as dystonic tremor. Such a probabilistic interpretation is possible with Bayesian analysis. This review presents a Bayesian analysis of ET plus in patients suspected of having ET versus a dystonic tremor syndrome, which is the most common differential diagnosis in patients referred for ET. Bayesian analysis of soft signs provides an estimate of the probability that a patient with possible ET is more likely to have an alternative diagnosis. ET plus is a distinct tremor classification and should not be viewed as a subtype of ET. ET plus covers a more-comprehensive phenotyping of people with possible ET, and the clinical interpretation of ET plus is enhanced with Bayesian analysis of associated soft signs.

Automated summary

Essential tremor plus (ET plus) is a new tremor classification that includes patients who meet the criteria for essential tremor but also have additional soft signs. Soft signs are not sufficient for diagnosing other tremor syndromes or movement disorders. Bayesian analysis is used to estimate the probability of alternative diagnosis in patients with possible ET. ET plus is a distinct classification and should not be considered a subtype of essential tremor. The clinical interpretation of ET plus is enhanced with the Bayesian analysis of associated soft signs.