PEDBASE.org - The Pediatric Database - Detailed information of ESSENTIAL TREMOR

ESSENTIAL TREMOR

DEFINITION:

A slowly progressive symmetric tremor affecting the arms and hands.

EPIDEMIOLOGY:

incidence: ?
age of onset:
- usually between 40-50 years of age but can begin in infancy or childhood
risk factors:
- familial - autosomal dominant
  - chrom.#: ?
  - gene: ?
- M = F
- Sweden

PATHOGENESIS:

1. Background

also called Benign Essential Tremor, Heredofamilial Tremor, and Familial (Essential) Tremor

2. Pathogenesis

etiology unknown

CLINICAL FEATURES:

1. Neurological Manifestations

1. Tremor

has a frequency of 4-9 Hz and is symmetric
usually limited to the arms and hands but may eventually involve the facial muscles, tongue, head, and neck
worse when arms and hands are placed in certain positions (i.e., antigravity)
diminishes at rest and disappears with sleep
enhanced by fatigue and stress
alcohol may attenuate
may be slowly progressive

2. Complications

20% of patients eventually develop rigidity
dysarthria with significant involvement of the head and neck

INVESTIGATIONS:

all investigations are normal

MANAGEMENT:

1. Medical

propranolol or primidone may be indicated if the tremor interferes with writing or activities of daily living

2. Prognosis

normal life span
varying degrees of tremor and rigidity

ADDITIONAL REFERENCES:

1. Rudolph, A.M., Rudolph's Pediatrics (19th Edition). p. 1869, (1991).

Pediatric Database - ESSENTIAL TREMOR

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