Chorea
Introduction[edit|edit source]
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]
- 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.
- Chorea is a type ofhyperkinetic movement disorder．
- 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: Spinocerebellarataxiasubtypes;Wilson disease; Sydenham chorea, and structural disease of the basal ganglia. Generally, the root of the pathology is in thebasal ganglia．[1]
They are predominantly of 2 types[2]
- Acquired Causes
- Genetic Causes
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舞蹈病,显示大幅度fl -变体inging 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:
- Huntington disease
- Lyme disease
- Multiple system atrophy
- Neuroacanthocytosis
- Pediatric torticollis surgery
- Ramsay Huntsyndrome
- Striatonigral degeneration
- Tourette syndrome
- Viralencephalitis[1]
Multimodal Management[edit|edit source]
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 caregiving. 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. You can read ithere．
Goals of Management[edit|edit source]
- Gait re-education
- Balance retraining
- Fallprevention/management
- Aerobic capacity
- Musclestrengthening
- Wheelchairprescription and training
- Respiratoryfunction
- Task-specific reach, grasp, and manipulation.
References[edit|edit source]
- ↑1.01.11.21.3Merical B, Sánchez-Manso JC. Chorea. Available:https://www.ncbi.nlm.nih.gov/books/NBK430923/#!po=15.0000(accessed 19.9.2022)
- ↑2.02.12.2Termsarasab P.Chorea．续INUUM: Lifelong Learning in Neurology. 2019 Aug 1;25(4):1001-35.
- ↑neurosigns.org. Chorea. Available fromhttps://www.youtube.com/watch?v=RxWEilu-Mf4&ab_channel=neurosigns.org
- ↑Feinstein E, Walker R.An update on the treatment of chorea. Current Treatment Options in Neurology．2018 Oct;20(10):1-5.
