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Dystonia and Tremor: The Clinical Syndromes with Isolated Tremor Cover

Dystonia and Tremor: The Clinical Syndromes with Isolated Tremor

Open Access
|Apr 2016

Figures & Tables

tre-06-319-7522-1-g001.jpg
Figure 1

Tremor generation by an oscillating circuit. An oscillation may arise whenever there is a delay in a negative feedback loop or an increase in gain in the control signal. A reference signal provides input about the target or goal state; a comparator compares the sensed information and the reference signal; gain transforms comparison into a control signal (i) that brings the sensed position closer to the reference signal, thus negating the error; the plant converts control signals into real output.

Table 1

Common Tremor Disorders Classified According to Two Main Criteria

Relation to Voluntary MovementRelation to Body Part
Rest tremorHead tremor
 Parkinson disease Cerebellar disease
 Other parkinsonian syndromes Dystonia
 Tardive (drug-induced) parkinsonism Essential tremor (rarely when isolated)
 Vascular parkinsonismChin tremor
 Hydrocephalus Parkinson disease
 Psychogenic (functional) tremor Hereditary geniospasm
Action tremorJaw tremor
Postural tremor Parkinson disease
 Physiologic tremor and enhanced physiologic tremor Dystonia
 Essential tremorPalatal tremor
 Dystonic tremor Idiopathic (essential)
 Parkinsonism Owing to brainstem lesions (secondary)
 Fragile X premutation (fragile X tremor–ataxia syndrome) Owing to degenerative disease (adult-onset Alexander disease)
 NeuropathiesArm tremor
 Tardive tremor Cerebellar disease
 Toxins (e.g., mercury) Distonia
 Metabolic disorder (e.g., hyperthyroidism, hypoglycemia) Essential tremor
 Psychogenic (functional) tremor Parkinson disease
Kinetic tremorLeg tremor
 Cerebellar disease Parkinson disease
 Holmes tremor Orthostatic tremor
 Wilson disease
 Psychogenic (functional) tremor
Table 2

Clinical Criteria for the Physical Signs Observed in Patients with Dystonia

Physical SignDescription
Dystonic posturesMuscle contractions may be continuous, forcing limbs and trunk into sustained postures (not available for blepharospasm or laryngeal dystonia)
 A body part is flexed or twisted along its longitudinal axis
 Slowness and clumsiness for skilled movements are associated with sensation of rigidity and traction in the affected part
Dystonic movementsThese features have to be looked for in all movement disorders, either fast or slow, also when the immediate impression is that of a tremor, tic, chorea, or myoclonus
 Tremor is a feature of dystonic movements and may appear as isolated tremor
 Movements are repetitive and patterned (i.e., consistent and predictable) or twisting
 Movements are often sustained at their peak to lessen gradually in a preferred posture (usually opposite to the direction of movement)
Gestes antagonistes (“sensory tricks”)Are voluntary actions performed by patients that reduce or abolish the abnormal posture or the dystonic movements?
 They are usually simple movements involving, or directed to, the body region affected by dystonia
 These movements are natural and graceful, not consisting in forceful opposition to the phenomenology of dystonia
 The movement does not push or pull the affected body part, but simply touches it (“sensory trick”) or accompanies it during alleviation of dystonia
 Alleviation of dystonia occurs during the geste movement, usually soon after its start
 Alleviation may last for as long as the geste or slowly reverses spontaneously before its end
Mirror dystoniaIt is evaluated in the upper or lower limbs. At least three different types of repetitive tasks (e.g., finger sequence, normal writing, or piano-like movements) are performed at low and fast speed in the non-affected limb
It is a unilateral posture or movement with same or similar characteristics to the patient’s dystonia (usually postures and some movements) that can be elicited, usually in the more severely affected side, when contralateral movements or actions are performed
Overflow dystoniaIt is observed at least once, usually ipsilaterally, in coincidence with the peak of dystonic movements
It is an unintentional muscle contraction accompanying the most prominent dystonic movement, but in an anatomically distinct neighboring body region
Table 3

Features Considered Typical of the Essential Tremor Syndrome

FeatureDescription
Tremor4–12 Hz action tremor that occurs when patients voluntarily attempt to maintain a steady posture against gravity (postural tremor) or move (kinetic tremor)
Tremor may be suppressed by performing skilled manual tasks
Tremor resolves when the body part relaxes as well as during sleep
Tremor at rest is not uncommon and observed in approximately 20% of patients
Age at onsetAdolescence (15–20 years) or late adulthood (50–70 years)
DistributionBilateral with minimal asymmetry
Affected body sites1Upper limbs >> head >> voice >> face/jaw >> tongue >> trunk >> lower limbs
ProgressionTremor may initially be intermittent, occurring during periods of emotional activation, and then becomes persistent over time
Response to alcoholBeneficial alcohol response present in 50–75% of patients
Family historyPositive family history present in 30–60% of patients

1 Listed from most to least prevalent site affected.

DOI: https://doi.org/10.5334/tohm.315 | Journal eISSN: 2160-8288
Language: English
Submitted on: Apr 16, 2015
Accepted on: Feb 21, 2016
Published on: Apr 5, 2016
Published by: Columbia University Libraries/Information Services
In partnership with: Paradigm Publishing Services
Publication frequency: 1 issue per year

© 2016 Alberto Albanese, Francesca Del Sorbo, published by Columbia University Libraries/Information Services
This work is licensed under the Creative Commons License.