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Comparative Study
. 2009 Aug 15;24(11):1606-12.
doi: 10.1002/mds.22570.

Older onset essential tremor: More rapid progression and more degenerative pathology

Elan D Louis  1 , Phyllis L Faust, Jean-Paul G Vonsattel, Lawrence S Honig, Claire Henchcliffe, Rajesh Pahwa, Kelly E Lyons, Eileen Rios, Cordelia Erickson-Davis, Carol B Moskowitz, Arlene Lawton
Affiliations
Comparative Study

Older onset essential tremor: More rapid progression and more degenerative pathology

Elan D Louis et al. Mov Disord. .

Abstract

There are few data on rate of progression in essential tremor (ET). To quantify the rate of tremor progression in a cross-sectional sample of 348 ET cases in an epidemiological study; characterize the relationship between age of tremor onset and rate of tremor progression in that sample; and characterize the relationship between age of tremor onset, rate of tremor progression, and severity of underlying brain changes in 9 cases from a brain repository. Rate of tremor progression was defined as tremor severity / duration. The degeneration index = number of torpedoes per section / Purkinje cell linear density. In the epidemiological study, older age of tremor onset was associated with faster rate of tremor progression (P < 0.001). In the brain repository, older age of tremor onset was associated with higher degeneration index (P = 0.037), and higher degeneration index was associated with faster rate of tremor progression (P = 0.018). In a large clinical sample, older age of onset was associated with more rapid tremor progression. In a brain bank, older age of onset was associated with more degenerative pathology in the cerebellum. As in several neurodegenerative disorders, in older onset cases, it is possible that the disease advances more rapidly.

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Conflict of interest statement

Disclosure: The authors report no conflicts of interest.

Figures

Figure 1
Figure 1
Rate of tremor progression by age of tremor onset (r = 0.74, p < 0.001). Fit line is shown; a logarithmic relationship is seen.
Figure 2
Figure 2
Degeneration index by age of tremor onset (r = 0.67, p = 0.05). The degeneration index was defined as the number of torpedoes per section divided by the Purkinje cell linear density.
Figure 3
Figure 3
Rate of tremor progression by degeneration index (Pearson’s r = 0.76, p = 0.03). The degeneration index was defined as the number of torpedoes per section divided by the Purkinje cell linear density. Complete spiral data were present in eight of nine ET cases. Fit line is shown.
Figure 4
Figure 4
Age of tremor onset by rate of tremor progression (Pearson’s r = 0.76, p = 0.03). Complete spiral data were present in eight of nine ET cases. Fit line is shown.
See this image and copyright information in PMC

References

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