Essential Tremor Syndrome

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Description

  • Essential tremor is a postural (occurring with voluntary maintenance of a position against gravity) or kinetic (occurring during voluntary movement) flexion–extension tremor that is slow and rhythmic and primarily affects the hands, forearms, head, or voice with a frequency of 4 to 12 Hz. Symptoms must be present for ≥3 years for a formal diagnosis of essential tremor.
  • Older patients tend to have lower frequency tremors, whereas younger patients exhibit frequencies in the higher range.
  • The tremor occurs in the absence of other neurologic signs such as dystonia, ataxia, or parkinsonism. The disease may be familial, sporadic, or associated with other movement disorders. Incidence and prevalence increase with age, but symptom onset can occur at any age. The tremor can be intermittent and exacerbated by emotional or physical stressors, fatigue, and caffeine.
  • Systems affected include neurologic, musculoskeletal, ear/nose/throat (ENT) (voice).

Epidemiology

Essential tremor is the most common pathologic tremor in humans.

Incidence

Can occur at any age, but bimodal peaks exist in the 2nd and 6th decades. Incidence rises significantly after age 49 years.

Prevalence

The overall prevalence for essential tremor has been estimated between to be about 1%, but is increased in older patients with an estimated prevalence of >5% at age 60 years and up to 22% at age 95 years. The prevalence of essential tremor is similar in males and females.

Etiology and Pathophysiology

  • Suspected to originate from an abnormal oscillation within thalamocortical and cerebello-olivary loops, as lesions in these areas tend to reduce essential tremor
  • Essential tremor is not a homogenous disorder; many patients have other motor manifestations and nonmotor features, including cognitive and psychiatric symptoms.

Genetics

  • Positive family history in 30–70% of patients; autosomal dominant inheritance is demonstrated in many families with poor penetrance. Twin studies suggest that environmental factors are also involved.
  • A link to genetic loci exists on chromosomes 2p22–2p25, 3q13, and 6p23. In addition, a Ser9Gly variant in the dopamine D3 receptor gene on 3q13 has been suggested as a risk factor.

Commonly Associated Conditions

  • Can be present in 10% of patients with Parkinson disease (PD); characteristics of PD that distinguish it from essential tremor include 3- to 5-Hz resting tremor; accompanying rigidity, bradykinesia, or postural instability; and no change with alcohol consumption
  • Patients with essential tremor have a 4% risk of developing PD.
  • Resting tremor, typically of the arm, may be seen in up to 20–30% of patients with essential tremor. Although action tremor is the hallmark feature of essential tremor, it is commonly found in patients with PD as well.

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