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Can J Neurol Sci. 2019 Jan 28:1-7. doi: 10.1017/cjn.2018.384. [Epub ahead of print]

Phenotypic Variability of Essential Tremor Based on the Age at Onset.

Author information

1
Department of Clinical Neurosciences,National Institute of Mental Health and Neurosciences,Bangalore,India.
2
Department of Biostatistics,National Institute of Mental Health and Neurosciences,Bangalore,India.
3
Department of Neurology,National Institute of Mental Health and Neurosciences,Bangalore,India.

Abstract

BACKGROUND:

Essential tremor (ET) is reported to have a bimodal distribution of age at onset (AAO) with phenotypic variability based on the AAO. This study aims to explore the distribution of AAO based on mathematical modeling and ascertain the differences, if any, in the clinical features of groups.

METHODS:

A chart review was conducted for 252 patients with ET diagnosed based on the Consensus statement of the Movement Disorder Society on Tremor. Finite mixture modeling was performed to identify groups of the cohort based on the AAO.

RESULTS:

Three groups were defined: early onset (EO): AAO ≤ 22 years, n = 63, intermediate onset (IO): 23 ≤ AAO ≤ 35 years, n = 43, and late onset (LO): AAO ≥ 36 years, n = 146. There were no significant differences related to family history or responsiveness to alcohol. The EO group had significantly higher prevalence of upper limb and lower limb tremor. Head tremor and voice tremor was more prevalent in the IO and LO groups. Cerebellar signs showed a significant increase with an increase in AAO.

CONCLUSIONS:

ET shows significant phenotypic variability based on the AAO. Patients with an early AAO are more likely to develop an appendicular tremor, whereas the probability of axial tremor and cerebellar signs increases with increasing AAO.

KEYWORDS:

Age at onset; Clinical features; Early onset; Essential tremor; Late onset

PMID:
30688180
DOI:
10.1017/cjn.2018.384

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