Is essential tremor a family of diseases or a syndrome? A family of diseases

Int Rev Neurobiol. 2022:163:7-29. doi: 10.1016/bs.irn.2022.02.001. Epub 2022 Mar 23.

Abstract

It is now well-established that essential tremor (ET) can manifest with different clinical presentations and progressions (i.e., upper limb tremor, head tremor, voice tremor, lower limb tremor, task- or position-specific tremor, or a combination of those). Common traits and overlaps are identifiable across these different subtypes of ET, including a slow rate of progression, a response to alcohol and a positive family history. At the same time, each of these manifestations are associated with specific demographic, clinical and treatment-response characteristics suggesting a family of diseases rather than a spectrum of a syndrome. Here we summarize the most important clinical, demographic, neuropathological and imagingfeatures of ET and of its subtypes to support ET as a family of identifiable conditions. This classification has relevance for counseling of patients with regard to disease progression and treatment response, as well as for the design of therapeutic clinical trials.

Keywords: Brain networks; Essential tremor; Genetics; Head tremor; Neuroimaging; Task-specific tremor; Tremor subtypes; Voice tremor.

MeSH terms

  • Essential Tremor* / diagnosis
  • Essential Tremor* / therapy
  • Humans
  • Syndrome
  • Tremor