Clinical subtypes of essential tremor

Background: There is clinical variability in essential tremor (ET), but it is not clear whether this variability is because of the existence of distin ct clinical subtypes of ET (ie, forms of ET that may differ in their etiolo gy, rate of progression, or response to treatment). Objectives: To examine in a group of ET cases the age of onset, anatomic di stribution, and rate of progression of tremor, and to look for associations between these factors. Methods: Cases of ET were ascertained from a community (n=60) and a tertiar y referral clinic (n=55) in northern Manhattan, New York, NY. All subjects underwent an interview and videotaped tremor examination. Rate of progressi on was estimated based on the tremor severity and reported disease duration at the time of evaluation. Results: Age of onset was bimodally distributed in clinic cases. There were differences in the anatomic distribution of the tremor (arm tremor only vs head and ann tremor vs isolated head tremor). Rate of progression was dist ributed exponentially; there was a large cluster of subjects with slower ra tes of progression, and a smaller number who had faster rates. There was an association between age of onset and rate of progression (r = 0.46-0.50, P <.002); cases with older age of onset (>60 years) progressed more rapidly ( P<.001). In addition, upper limb tremor progressed more slowly among those with concomitant head tremor (P=.03). Conclusions: Essential tremor is not a homogeneous condition. There are dif ferences in age of onset, anatomic distribution of tremor, and rate of prog ression. The ET in several groups of patients in this study (those with age of onset >60 years and those without head tremor) progressed moro rapidly, suggesting that these ET cases may define distinct clinical subtypes. Thes e subtypes should be further assessed for etiologic and genetic heterogenei ty as well as differences in responsiveness to therapeutic agents.