Hyperkinetic Movement Disorder

Original Editor - Muskan Rastogi Top Contributors - Muskan Rastogi and Kim Jackson



Introduction[edit | edit source]

Hyperkinetic movement disorders(HMD's) also referred to as Dyskinesias are characterized by abnormal, often repetitive, involuntary movements overlapped to normal motor activity. Its 5 major types are Tremors, Chorea, Dystonia, Myoclonus and Tics.

Etiology[edit | edit source]

Common etiologies seen in Hyperkinetic Movement Disorders-[1]

  1. Genetic abnormalities
  2. Neurodegenerative diseases
  3. Structural lesions
  4. Infection
  5. Drugs
  6. Psychogenic problems
  7. Others

Pathophysiology[edit | edit source]

There seems to be decrease in neural firing rates in the inhibitory output nuclei of the basal ganglia which results into a subsequent disinhibition of thalamocortical activity and sensory abnormalities might also have some role.[1]

Cardinal Features[edit | edit source]

Hyperkinetic movement disorders can be grouped according to distinct cardinal features, which can be described in terms of[2]

  • Time
  • Space distribution
  • Body state’s impact.

Sub- features[edit | edit source]

Rhythmicity[2]

A rhythmic movement repeats over time at a fixed interval of time. If the movement can be defined with a frequency during an observation period, it has a regular rhythm (e.g., essential tremor, parkinsonian tremor), if the movement repeats with a more complex temporal pattern, it has an irregular rhythm (e.g., cortical myoclonus), and finally, if the movement repeats over time at no fixed interval of time, it is arrhythmic (e.g., chorea, athetosis, ballism, tics, akathitic movements).

Speed[2]

The second temporal item is the speed of the movement . It can be very fast, such as in myoclonus or hemifacial spasm, fast las in ballism or tics, intermediate as in chorea and tremors, or slow as in athetosis or akathitic movements.

Duration of the Muscular Contraction[2]

Finally, in terms of the time spectrum, we need to describe the duration of the muscular contraction and the duration of the whole movement . The duration of the muscular contraction can be sustained or not. A sustained muscular contraction is fixed and doesn’t change during time, unlike the non-sustained muscular contraction.

Duration of the Movement[2]

The duration of the movement can be defined as paroxysmal, if the movement repeats with a sudden recurrence (e.g., paroxysmal dyskinesia, paroxysmal ataxia); continual, if the movement repeats over and over again without a sudden recurrence (e.g., ballism, chorea), or continuous, if the movement continues without stopping (e.g., abdominal dyskinesias).

Body Distribution[2]

According to space characteristics, we can classify hyperkinetic movements according to body distribution, i.e., the body part involved in the involuntary movement. Dystonia can be classified as :

  • Focal: 1 body part is affected
  • Segmental: Equal to or more than 2 contiguous body parts are affected
  • Multifocal: Equal to or more than 2 non-contiguous body parts are affected
  • Hemi -dystonia: Ipsilateral arm and leg are involved
  • Generalized: Equal to or more than 3 body parts are affected, including the trunk and Equal to or more than 2 other sites; with or without leg involvement

Muscular Pattern[2]

Another important space feature is the muscular pattern activated in dystonia . In patterned movement, the involuntary movements involve the same group of muscles in a repetitive way.

Amplitude[2]

Finally, in terms of space characteristics, it is important to define the amplitude of the movement: large (e.g., ballism), medium (e.g., chorea), or small (e.g., tremor).

Body State’s Impact[2]

The last main feature needed to classify a hyperkinetic movement is the impact of body state on the movement. Can the involuntary movement be modified by a voluntary movement? Is it suppressible? Is it modified by wakefulness?

Action Rule[2]

Some movements are present only during rest. Some movements are present during voluntary movement only. Other movements are present during both rest and voluntary movement.

Suppressibility[2]

Another important feature is suppressibility. We need to detect if the movement is totally or partially voluntary suppressible (e.g., stereotypies, tics, akathitic movements), or if it is not suppressible (e.g., myoclonus).

Wakefulness[2]

Finally, we need to understand the relationship between movement and wakefulness, e.g., if it is present while awake or sleeping. Some movements appear during sleep only (e.g., REM sleep behavior disorder, periodic movements in sleep), or persist during sleep (e.g., spinal myoclonus, myokimia, moving toes) and others are present only while awake.

Below is Chart representing Cardinal features of hyperkinetic disorders.

Hyperkinetic disorder relationship chart.jpg

Classification[edit | edit source]

There are 2 types of hyperkinetic disorders-[3]

Jerky Movements

  1. Myoclonus- Myoclonus presents as a sudden brief jerk caused by involuntary muscle activity.
  2. Chorea-a state of excessive, spontaneous movements, irregularly timed, non-repetitive, randomly distributed and abrupt in character
  3. Tics- involuntary, rapid, abrupt, repetitive, recurrent, and nonrhythmic movements or vocalizations. Tics are suppressible and almost always disappear at sleep and can be exacerbating with stress, excitement and anxiety.

Non-jerky Movements

  1. Tremor- an involuntary movement that is rhythmic (i.e., regularly recurrent) and oscillatory (i.e., rotating around a central plane).
  2. Dystonia- The involuntary contractions of muscles occur. It is characterised by sustained muscle contractions and abnormal trunk, neck, face, arms, and legs postures.

Assessment[edit | edit source]

Things that are need to be assessed-[3]

  • Gait and Balance for fall prevention
  • Functional tasks
  • Speech and cognition
  • Dysphagia
  • Mental Health (behavior/affective state)

Diagnosis[edit | edit source]

  • To correctly diagnose movement disorders one needs to establish the phenomenology of the clinical syndrome.[3]The phenomenology is dogged from the specific amalgamation of the dominant movement disorder, the presence of any accessory abnormal movements, and any further neurological or non-neurological abnormalities.
  • Both neurological and non-neurological conditions can mimic various movement disorders, and it is vital not to miss these lookalikes.[3]
  • A systematic approach is recommended when clinicians see patients who present with one or more combination of movement disorder.[3]

Management[edit | edit source]

Management of Chorea

Management of Dystonia

Management of Tremor

Management of Tics

Management of Myoclonus

References[edit | edit source]

  1. 1.0 1.1 Hyperkinetic Movement Disorders.Available from :https://now.aapmr.org/hyperkinetic-movement-disorders-including-dystonias-choreas/ (accessed 26th september 2022)
  2. 2.00 2.01 2.02 2.03 2.04 2.05 2.06 2.07 2.08 2.09 2.10 2.11 di Biase L, Di Santo A, Caminiti ML, Pecoraro PM, Di Lazzaro V. Classification of Dystonia. Life. 2022 Jan 29;12(2):206.
  3. 3.0 3.1 3.2 3.3 3.4 Abdo WF, Van De Warrenburg BP, Burn DJ, Quinn NP, Bloem BR. The clinical approach to movement disorders. Nature Reviews Neurology. 2010 Jan;6(1):29-37.