Tremor

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Definition

  • Is an involuntary, rhythmic, oscillatory movement of a body part.

Physiological tremor

  • The limbs and head, when unsupported, exhibit slight tremor, referred to as physiological tremor.
  • Is generally not visible or symptomatic unless it is enhanced by fatigue or anxiety, whereas pathological tremor is usually visible and persistent

Classification has two main axes

Clinical features

Medical history
  • Age of onset
    • Infancy (birth to 2 years)
    • Childhood (3-12 years)
    • Adolescence (13-20 years)
    • Early adulthood (21- 45 years)
    • Middle adulthood (46-60 years)
    • Late adulthood (>60 years).
  • Family history
  • Past medical history
  • Temporal evolution
  • Exposure to drugs and toxins
Tremor characteristics
  • Body distribution
  • Activation condition
    • Rest tremor
      • Tremor in a body part that is not voluntarily activated.
      • It should be assessed when the patient is attempting to relax and is given adequate opportunity to relax the affected body part.
        • This may require complete support of the body part (e.g., the head) against gravity.
      • Increase while
        • Walking or
        • When performing movements of another body part
      • Also occur in advanced ET,
        • But rest tremor does not subside during voluntary movements in ET and in dystonic tremor
      • The re-emergent tremor of PD should be regarded as an action tremor irrespective of the waveform similarities with rest tremor.
      Action tremor
      • Occurs while
        • Voluntarily maintaining a position against gravity (postural tremor and orthostatic tremor) OR
        • During any voluntary movement (kinetic tremor).
      • Kinetic tremor
        • Subdivided into
          • Simple kinetic tremor in which tremor is roughly the same throughout a movement (e.g., waiving with the hands at a slow speed), and
          • Intention tremor in which a crescendo increase in tremor occurs as the affected body part approaches its visual target
          • Task-specific kinetic tremor in which a specific task such as writing.
      • Position-specific postural tremor
        • Occurs when maintaining a specific position or posture
      • Isometric tremor
        • Occurs when a muscle contraction against a rigid stationary object
        • Eg: when making a fist or squeezing an examiner’s fingers.
  • Frequency
Associated signs
  • Associated or concomitant signs that may aid in clinical diagnosis.
    • We propose two broad categories of tremor in Axis 1:
      • Isolated tremor
        • In which tremor is the only abnormal sign
      • Combined tremor
        • In which other abnormal signs are present.
        • May occur with
          • Other neurological signs
            • (e.g., dystonic postures, rigidity, bradykinesia, or myoclonus)
          • With relevant systemic signs
            • (e.g., Kayser-Fleischer ring, hepatosplenomegaly, or exophthalmos).
Anatomical distribution of tremor
  • Focal
    • Only one body region is affected,
    • Eg: voice, head, jaw, one limb, etc.
  • Segmental
    • >2 contiguous body parts in the upper or lower body are affected
    • e.g., head and arm, or when tremor is bibrachial or bicrural
  • Hemitremor
    • One side of the body is affected
  • Generalized
    • Tremor affects the upper and lower body
Additional laboratory tests
  • Electrophysiological tests
  • Structural imaging
  • Receptor imaging
  • Serum and tissue biomarkers

Etiology

  • Acquired
  • Genetically defined
  • Idiopathic
    • Familial
    • Sporadic

Frequency and amplitude of different tremor

  • Frequency is not very helpful in diagnosis. Most accurately measured with a motion transducer or electromyography.
    • <4:
      • Myorhythmia
      • Some palatal tremors
    • 4 to 8
    • 8 to 12
      • Central neurogenic component of physiological tremor
      • Rhythmic cortical myoclonus
    • >12 Hz
      • Primary orthostatic tremor
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  • Different types of tremor
    • Tremor Type
      Description
      Essential tremor
      The most common tremor disorder and is a low-amplitude, bilateral action and postural tremor with a frequency of 6-8 Hz. The tremor usually has its onset in adulthood and worsens over time, but it may begin in childhood and can coexist with other movement disorders. ET involves the upper limbs in more than 90% of patients. It less commonly involves the head, legs, or voice. Patients commonly first complain of difficulty with tasks requiring fine coordination, such as threading a needle, tying knots, or writing. Alcohol may temporarily alleviate symptoms, and family history is often positive. May be due to unstructural cerebellar dysfunction with ET patients commonly have an intention tremor and difficulty with tandem gait
      Dystonic tremor
      Dystonic tremor is a jerky postural and task/action 3-8 Hz tremor that is abolished by complete rest and occurs in a body part affected by dystonia (e.g., limbs, trunk, head, vocal cords, or face). This contrasts with essential tremor in which the kinetic component is more or less constant throughout all postures. Dystonic tremor may occur with other involuntary movements including blepharospasm, torticollis, or spasmodic dysphonia. Isolated head tremor can be particularly challenging to distinguish between essential tremor and dystonic tremor (the latter of which can be exquisitely responsive to pharmacological treatment or botulinum toxin injections). The tremor caused by cervical dystonia is usually present as “no-no” kind of head shaking
      Physiological tremor
      Non-pathologic postural tremor, which typically has a frequency of 8-12 Hz. Both ET and physiologic tremor can be elicited by posture, both are fairly symmetrical, and both occur predominantly in the arms. Observing the progression of a tremor over time will often reveal whether a given patient has ET or physiologic tremor
      Holme’s tremor
      Although predominantly an action tremor, Holmes tremor frequently has a significant resting component. The amplitude of movement tends to be large and it can sometimes adopt a “wing-beating” appearance. It is also among the slowest tremors, with frequencies often less than 4 Hz. It can occur with lesions affecting not only the red nucleus and rubral spinal tract in the brainstem but also the cerebellum and thalamus. The tremor may appear weeks to months after a known lesion (e.g., stroke), and some patients may have associated dystonia
      Neuropathic tremor
      Tremor may accompany diseases of the anterior horn cell (e.g., amyotrophic lateral sclerosis) and peripheral neuropathies. It is unclear whether tremor associated with peripheral neuropathy is due to enhanced physiologic tremor secondary to weakness, to an abnormality in the central nervous system, or both
      Drug-induced tremor
      The onset of tremor should be temporally related to drug ingestion and may be due to an enhancement of physiologic tremor (e.g., amiodarone, antidepressants, antiepileptic medications, beta-agonist bronchodilators, caffeine, lithium, neuroleptics, nicotine, steroids, and sympathomimetics) or production of cerebellar tremor (immunosuppressive agents and acute/chronic alcohol)
      Psychogenic tremor
      Clinical features suggesting psychogenic tremor include sudden onset with severe presentations, inconsistent combinations of resting and postural or kinetic tremor, entrainment (change in frequency of tremor to that of a task performed in another body part, e.g., a patient with left hand tremor who taps at various frequencies with the right hand will have a left hand that acquires those frequencies), and tremor that diminishes with distraction
      Parkinsonian tremor
      A 4-9 Hz low-amplitude rest tremor, often with a “pill-rolling” quality. A typical pattern of spread is for the dominant hand to be affected first, followed by the dominant foot and then the non-dominant hand. Re-emergent tremor occurs while sustaining a prolonged position and most likely represents a rest tremor that has been reset by the relative stasis of a persistent position. Postural tremor that begins immediately on adopting a position is seen in as many as 93% of patients with PD and correlates with the degree of functional disability. Treatment with levodopa improves bradykinesia and rigidity more reliably than it does tremor
      Cerebellar tremor
      Cerebellar tremor is characterized as a jerky, low-frequency (2-5 Hz), high-amplitude action tremor. This tremor may be accompanied by other cerebellar signs such as ataxia, dysdiadochokinesia, dysarthria, dysmetria, and telegraphic speech
      Post-traumatic tremor
      The character of the tremor depends on the region of the brain that is damaged. Damage to the brainstem may produce rest tremor if it affects the substantia nigra and related pathways. Damage to the cerebellum may result in a low-frequency action tremor. Because multiple regions are usually damaged, post-traumatic tremors are generally mixed in character. Post-traumatic tremor is often accompanied by myoclonus
      Primary orthostatic tremor
      Uncommon condition that starts in late adulthood with feeling of tremulousness in the legs when standing (but not sitting or lying). The diagnosis is helped by specific surface EMG showing a 14-18-Hz oscillating tremor in the musculature of the legs when standing, disappearing with rest or movement
      Palatal tremor (Palatal myoclonus/nystagmus)
      A rare disorder presenting as unilateral or bilateral rhythmic involuntary movements of the soft palate. The movement consists of repetitive rather than oscillatory movements of agonist muscles only, thus having some similarity with myoclonus. The tremors in essential palatal tremor produce audible click due to the contraction of the tensor veli palatini muscle which disappear during sleep, whereas in symptomatic palatal tremor there is no audible click but it continues during sleep

Syndromes

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  • Isolated rest tremor syndromes
    • Most commonly occur in an upper or lower limb or as a hemitremor, but may occur elsewhere (e.g., lips, jaw, or tongue). It is crucial to determine, using Axis 1 characteristics, whether the rest tremor is isolated or combined with other clinical features.
  • Isolated voice tremor
    • Is a visible and/or audible tremor of the vocal apparatus.
  • Isolated head tremor
    • Is a shaking of the head in yes-yes, no-no, or variable directions.
  • Palatal tremor
    • Is characterized by rhythmic movement of the soft palate at 0.5 to 5 Hz.
  • Primary orthostatic tremor
    • Is a generalized high- frequency (13-18 Hz) isolated tremor syndrome that occurs when standing.
    • Confirmation of the tremor frequency is needed, typically with an electromyography (EMG).
  • Dystonic tremor syndromes
    • Tremor syndromes combining tremor and dystonia as the leading neurological signs.
    • Different syndromes are separated on clinical grounds.
    • Tremor combined with parkinsonism (bradykinesia and rigidity)
      • Typically a 4- to 7-Hz rest tremor of the hand (“pill-rolling” tremor), lower limb, jaw, tongue, or foot. This is called classic parkinsonian tremor.
      • Other types of tremor may coexist in patients with parkinsonism, such as postural or kinetic tremor with the same or different frequency as the rest tremor.
    • Intention tremor syndromes
      • Consist of intention tremor at <5 Hz, with or without other localizing signs.
    • Holmes tremor
      • Is a syndrome of rest, postural, and intention tremor that usually emerges from proximal and distal rhythmic muscle contraction at low frequency (<5 Hz).
  • Myorhythmia
    • Very rare rhythmic movement disorder of cranial or limb muscles at rest or during action and is classified here as a tremor.
    • The frequency is 1 to 4 Hz. It is usually associated with localizing brainstem signs and usually with a diagnosable aetiology.
  • Functional (a.k.a., psychogenic) tremor is
    • Characterized by distractibility, frequency entrainment, or antagonistic muscle coactivation.
  • Indeterminate tremor syndrome
    • Is reserved for a patient who does not fit into an established syndrome or who needs further observation to clarify the tremor syndrome.

Treatment

  • Indication
    • Symptom refractory medical treatment
    • Causing disability
    • No Contraindication
  • Surgical target
    • VIM
      • Common side effect
        • Cognitive
        • Balance and gait
  • DBS vs Lesioning
    • RF thalamotomy
      DBS
      Cheaper
      Expensive
      No implant
      Hardware maintenance
      Quicker surgery
      Longer surgery
      No habituation
      Habituation
      Irreversible
      Reversible to some degree
      Less forgiving
      More forgiving if got wrong
      Unilateral (usually)
      Staged bilateral (VIM)
      Requires expertise
      Other targets (bilateral)
      Post-imaging studies (less artefact)
      Recurrence
      Risk of deafferentation pain