Chorea

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

Introduction

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. 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

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.

They are predominantly of 2 types

• Acquired Causes
• Genetic Causes

Acquired Causes of Chorea

Genetic Causes of Chorea

Phenomenological Features

• 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.

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

Diagnosis

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

Differential Diagnosis

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

• Huntington disease
• Lyme disease
• Multiple system atrophy
• Neuroacanthocytosis
• Pediatric torticollis surgery
• Ramsay Hunt syndrome
• Striatonigral degeneration
• Tourette syndrome
• Viral encephalitis

Multimodal Management

Chorea requires a Multidisciplinary Approach to be treated.

The team includes:

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

Medical Management

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. 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

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

Goals of Management

• Gait re-education
• Balance retraining
• Fall prevention/management
• Aerobic capacity
• Muscle strengthening
• Wheelchair prescription and training
• Respiratory function
• Task-specific reach, grasp, and manipulation.