Chorea

Chorea is a hyperkinetic movement disorder with various causes. Treatment depends on the underlying condition. In Huntington's disease, dopamine depleting agents like tetrabenazine are used. Other treatments include antipsychotics and surgical options. Chorea in Sydenham chorea may be treated with antibiotics and chorea-suppressing medications. Wilson's disease is managed with copper-chelating agents and zinc acetate.

2024-01-06 20:55:37 - Editor

Chorea Overview:

Chorea is a hyperkinetic involuntary and non- patterned movement disorder characterised by sudden and unpredictable muscle contractions affecting mostly distal limbs. Also, it could affect the face and/or the trunk. Chorea is usually classified as primary (hereditary) (Huntington chorea) or secondary (acquired). Acquired chorea could be either medication induced, such as, metoclopramide, prochlorperazine, vascular as a consequence of acute ischemic, hemorrhagic stroke, Sydenham chorea (SC), paraneoplastic chorea in small cell lung cancer, pregnancy related (Chorea gravidarum), Wilson disease or metabolic chorea by hyperglycaemia, hypernatremia, hypomagnesaemia and hypocalcaemia. Other causes of acquired chorea include infections by HIV, toxoplasmosis, TB and syphilis. (1, 2)

Non-pharmacological Treatment:

Treatment is indicated if chorea is prominent, interferes with daily activities and impedes patient’s quality of life. Non-pharmacological treatment includes providing patients with a calm and structured atmosphere, use of assistive equipment such as helmets, padded reclining chairs and low beds. Genetic counselling of relatives of the affected patient is very important in Huntington chorea. (3)

Pharmacological Therapy for Huntington Chorea:

Pharmacological therapy for Huntington chorea includes treatment with dopamine depleting agents for patients who have moderate to severe chorea which is refractory to non-pharmacological measures. Dopamine depleting agents act primarily by inhibiting presynaptic vesicular monoamine transporter type 2 (VMAT2). They include tetrabenazine (12.5 mg daily) and deutetrabenazine (6mg/day up to a maximum of 48mg/ day). These agents could cause severe depression and suicide, and they should not be tapered abruptly. Benzodiazepines may be added intermittently when there is a transient worsening of chorea in stressful situations. (4)

Combined Pharmacotherapy:

For patients with debilitating chorea who fail treatment with first line monotherapy, combining tetrabenazine or deutetrabenazine with a second generation antipsychotic agent may be beneficial. These agents

Alternative Treatment Options:

Other treatment options include amantadine (100 mg twice daily) and Levetiracetam ( 500 mg twice daily). The effectiveness of these agents has not yet established. (7, 8)

Surgical Options:

Surgical options including pallidotomy, thalamotomy and deep brain stimulation (DBS) is not well studied and patients should be selected carefully. (9, 10)

Associated Symptoms of Huntington Disease:

In addition to chorea, Huntington disease is associated with other symptoms including depression which could be managed with selective serotonin reuptake inhibitors including risperidone (1 to 2 mg daily) and aripiprazole at 2 to 5 mg daily. Psychosis usually responds to quetiapine at 12.5 mg daily. For management of cognitive impairment, donepezil at a dose of 5 mg daily is suggested. (4, 5, 6)

Treatment of Sydenham Chorea:

Treatment of patients with SC consists of chronic antibiotic therapy to prevent recurrence and minimise the development of rheumatic heart disease. In addition, patients with significant impairment related to their chorea may be treated with chorea suppressing medications or immune suppressing treatment. Antibiotic therapy with long- acting penicillin G benzathine is required. Symptomatic treatment for chorea when it interferes with daily activities with dopamine- 2 receptor blocking agent such as, fluphenazine (2 mg daily) or haloperidol (1 to 2 mg/day). Alternative options include carbamazepine (100 to 200 mg/day) or guanfacine (0.5mg/day). Immune suppressing treatment is with oral prednisolone 1 -2 mg/kg daily for two weeks and then taper over two weeks). (11, 12, 13)

Management of Wilson Disease:

Patients with Wilson disease should be maintained on a low copper diet and avoid copper- rich food. Medical treatment is through the use of copper- chelating agents including D- penicillamine (at a starting dose of 250 to 500 mg daily) or Trientine at an initial dose of 20 mg/kg/day in two to four divided doses, with a maintenance dose of 15 mg/kg/day in two to three divided doses. Also, oral zinc acetate which interferes with copper absorption at 150 mg per day is advisable. (14, 15, 16)

References

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