Clinical-Instrumental patterns of neurodegeneration in Essential Tremor: A data-driven approach

Parkinsonism Relat Disord. 2021 Jun:87:124-129. doi: 10.1016/j.parkreldis.2021.05.011. Epub 2021 May 14.

Abstract

Introduction: Essential Tremor (ET) is increasingly recognized as a complex disorder with additional clinical signs other than tremor. It is still unknown whether a unique pathophysiologic or neurodegenerative process underlies progression and prognosis of the disease. The aim of the study was to identify ET phenotypes through a clinical-instrumental data-driven approach and to characterize possible patterns of neurodegeneration.

Methods: ET patients were categorized using spatio-temporal and kinematic variables related to mobility and dynamic stability processed by motion transducers. Differences between the identified groups in clinical-demographic variables, neuropsychological performances and retinal parameters by Optical Coherence Tomography (OCT) segmentation analysis were tested.

Results: Twenty-five ET patients were studied. Based on clustering of kinematic and spatio-temporal gait parameters, two independent groups were identified: cluster "A" (N = 15) and cluster "B" (N = 10). Compared to group A, group B had overall worse performance in mobility, especially on turning tasks. Identified clusters did not differ in terms of age, age at onset and disease duration. Patients in group B had more head tremor and more severe action tremor in the upper limbs as compared to group A, demonstrating also worse performances on cognitive assessments. Based on OCT analysis, group B presented a reduced thickness of the retinal inner layer as compared to group A, suggesting underlying neurodegenerative processes.

Conclusions: The presence of gait and mobility impairment, associated with midline tremor, cognitive decline and retinal degeneration suggests a subtype of ET associated with neurodegeneration.

Keywords: Essential tremor; Neurodegeneration; Optical coherence tomography; Timed up and go test.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Aged
  • Aged, 80 and over
  • Cognitive Dysfunction / etiology
  • Cognitive Dysfunction / physiopathology
  • Essential Tremor / classification
  • Essential Tremor / complications
  • Essential Tremor / pathology*
  • Essential Tremor / physiopathology*
  • Female
  • Gait Disorders, Neurologic / etiology
  • Gait Disorders, Neurologic / physiopathology
  • Humans
  • Male
  • Neurodegenerative Diseases / diagnostic imaging
  • Neurodegenerative Diseases / pathology*
  • Neurodegenerative Diseases / physiopathology*
  • Retina / diagnostic imaging
  • Retina / pathology*
  • Tomography, Optical Coherence