Chorea

Original Editor - Muskan Rastogi Top Contributors - Muskan Rastogi, Lucinda hampton, Chelsea Mclene and Cindy John-Chu

Introduction[edit | edit source]

Rheumatic chorea

Chorea is defined as "a state of excessive, spontaneous movements, irregularly timed, non-repetitive, randomly distributed and abrupt in character. These movements may vary in severity from restlessness with mild intermittent exaggeration of gesture and expression, fidgeting movements of the hands, unstable dance-like gait to a continuous flow of disabling, violent movements."[1]

  1. The word Chorea has Greek origins meaning "to dance". It has a dance-like appearance due to the random and flowing quality of the movement.
  2. Chorea is a type of hyperkinetic movement disorder.
  3. The involuntary movements flow from one body area to another which cannot be predicted beforehand.

Etiology[edit | edit source]

Chorea traditionally is described in Huntington disease, however other disorders that can lead to chorea, for example: Spinocerebellar ataxia subtypes; Wilson disease; Sydenham chorea, and structural disease of the basal ganglia. Generally, the root of the pathology is in the basal ganglia.[1]

They are predominantly of 2 types[2]

  • Acquired Causes
  • Genetic Causes
Acquired Causes of Chorea


Genetic Causes of Chorea


Phenomenological Features[edit | edit source]

  • Randomness
  • Flowing Quality
  • Parakinesia: Patients blend their chorea-induced movements with their own normal movements.
  • Motor impersistence: the patient is unable to perform sustained motor activities.
  • Ballism: Variant of chorea which shows large-amplitude flinging movements involving proximal extremities.
  • Athetosis: Slow writhing movements involving distal limbs sometimes may be facing.
  • Varying velocity of movements: quick velocity and low-amplitude movements accompanied by jerks.[2]

This video displays the dance-like movements performed by a patient.[3]

Diagnosis[edit | edit source]

The phenomenological features mentioned above are observed to make a diagnosis of chorea.

Differential Diagnosis[edit | edit source]

Specific diagnosis can be made on the basis of symptoms/clinical features experienced by the patient.[2]Differential diagnoses include:

Multimodal Management[edit | edit source]

Mutimodal management chorea.jpg

Chorea requires a Multidisciplinary Approach to be treated.[4]

The team includes:

  • Psychiatrist
  • Physical medicine and Rehabilitation Specialist
  • Physical therapist
  • Occupational therapist
  • Speech therapists
  • Geneticist
  • Genetic Counselor
  • Social worker

Medical Management[edit | edit source]

There is no cure for this disease but symptoms can be managed. Importantly an evaluation and creation of a patient's support system is needed. With disease progression, the patient will need specialized care. Nutrition management is important, due to difficulty swallowing.[1]

1. Dopamine-depleting agents: Tetrabenazine, Deutetrabenazine, Valbenazine.

2. Dopamine D2 receptor-blocking agents: Haloperidol, Clozapine, Olanzapine.

3. Anticonvulsants: Valproic acid, Carbamazepine, Benzodiazepines, Levetiracetam.

4. Anti-glutamatergic agents: Amantadine, Riluzole.

5. Cannabinoids: Nabilone.

6. Deep Brain Stimulation

Physiotherapy Management[edit | edit source]

The management will be same as of Huntington's Disease.

Goals of Management[edit | edit source]

References[edit | edit source]

  1. 1.0 1.1 1.2 1.3 Merical B, Sánchez-Manso JC. Chorea. Available: https://www.ncbi.nlm.nih.gov/books/NBK430923/#!po=15.0000(accessed 19.9.2022)
  2. 2.0 2.1 2.2 Termsarasab P. Chorea. CONTINUUM: Lifelong Learning in Neurology. 2019 Aug 1;25(4):1001-35.
  3. neurosigns.org. Chorea. Available from https://www.youtube.com/watch?v=RxWEilu-Mf4&ab_channel=neurosigns.org
  4. Feinstein E, Walker R. An update on the treatment of chorea. Current Treatment Options in Neurology. 2018 Oct;20(10):1-5.