Chorea (Huntington's Chorea Disease) Treatment in Delhi, India

Chorea is a movement disorder that causes sudden, involuntary, random, non-predictable jerky muscle movements. This disorder makes the person look like he/she is dancing or fidgeting.

Chorea is not a life-threatening disorder, but it may be a sign of various underlying diseases such as Huntington's disease. The symptoms can be mild to severe, where flailing movements are observed in the patient. Chorea is also a symptom of many infectious diseases, endocrine system disorders, side effects of drugs, pregnancy, and many more.

In serious cases, this disorder can also interfere with normal activities such as swallowing, speaking, standing, sitting, walking, etc.

Signs and Symptoms

The signs and symptoms of chorea depend upon the conditions that are causing it. Sometimes, the symptoms are severe, while sometimes, they are mild. Following are the signs and symptoms of chorea-

• Milkmaid's grip- It is one of the most common symptoms of chorea, where individuals tend to have improper and uncoordinated hand movements. As a result, they tend to squeeze and relax their hands as if they are milking while shaking hands
• Tongue moving in and out of the mouth (known as "Jack-in-the-Box tongue)
• Dance or piano-playing-like movements- Some individuals may exhibit dance or piano-playing-like symptoms. Individuals may start showing abnormal wavy movements as if they are dancing or playing the piano.
• Headaches and seizures in children with rheumatic fever
• However, the movements may look different within various diseases, often get worse when the person is anxious and disappear when sleeping.

There are some diseases that may lead to the occurrence of Chorea

Huntington's Disease

It is an autosomal dominant neurodegenerative disorder, which is characterised by motor, cognitive and psychiatric symptoms. The commonest symptom of Huntington's disease includes chorea, such as involuntary writhing or jerking movements. The symptoms of Chorea are milder in the beginning and can get worse over time. The lower, as well as the upper limbs, may get affected, and such individuals may start showing abnormal involuntary hand and leg movements. Other symptoms include reduced manual dexterity, slurred speech, swallowing difficulties, balance problems and falls.

Chorea-acanthocytosis

The condition is characterised by involuntary jerking movements (chorea), abnormal star-shaped red blood cells (acanthocytosis), and involuntary stiffening of various muscles (dystonia), such as those in the limbs, face, mouth, tongue, and throat. A person suffering from this disorder has variable symptoms such as abnormal hand and leg movements, pelvic thrusts, seizures, etc. Individuals suffering from this type of chorea can also show the symptoms of dystonia, such as teeth grinding, difficulty in speaking, lip and tongue biting, involuntary belching, etc.

Sydenham's Chorea

This type of chorea is mainly found in children and adults. It is usually a complication or aftermath of a streptococcal infection and rheumatic fever. It majorly affects the face, hands, and arms. Individuals suffering from this type of Chorea exhibit difficulty in performing day-to-day tasks such as getting dressed. This disorder also leads to the patient's abnormal walking patterns or gait, slurred speech, reduced muscle tone, etc. Another characteristic feature of this type of chorea is the appearance of the "Harlequin tongue", in which the person loses the ability to stick the tongue out, rather pop the tongue in and out randomly and involuntarily.

Causes

Chorea is also supposed to be associated with various causes, both acute as well as chronic-

• AIDS
• Pregnancy (Chorea gravidarum in the first three months of the pregnancy)
• Stroke
• Endocrine and metabolic disorders (e.g. Hypoglycemia, Hyperthyroidism, Hypoparathyroidism, Hyponatremia, Hypernatremia, Polycythemia vera, Hypomagnesemia, Hypocalcemia, Hepatic Failure, etc.)
• Immune disorders (e.g. Systemic lupus erythematosus, Multiple Sclerosis, Sarcoidosis, Sjogren syndrome, Behcet disease)
• Medications such as levodopa, neuroleptics, oral contraceptives, amphetamines, anticonvulsants, tricyclic antidepressants, etc.
• Infections such as streptococcal infection, as in Sydenham's Chorea, Legionnaire disease, encephalitis, meningitis, Legionnaire disease, Creutzfeldt-Jakob disease, etc.
• Genetic Disorders (Huntington's Disease)

Diagnosis

Since there are various causes of developing Chorea, diving deep into the previous medical history is very necessary while going for the diagnosis of Chorea. The Chorea doctor should be asking certain questions to the individuals , such as-

• The time of the appearance of the noticeable symptoms
• Factors that trigger the worsening of Chorea, such as stress
• Any familial history of Huntington's Disease
• Medications or what drugs the person is consuming
• There are also some laboratory tests that help in indicating chorea, such as the detection of abnormal copper levels in the body, as in the case of Wilson's Disease, which is a genetic disorder
• Peripheral blood smear for the detection of spiky or star-shaped RBCs indicating chorea-acanthocytosis
• Magnetic Resonance Imaging (MRI) or Computed Tomography (CT scan) of the brain helps in diagnosing Huntington's disease
• Genetic tests can also be conducted for Huntington's Disease

Treatment

Treatment of chorea varies largely according to the type of disorder due to which it arises. Most of the pharmacological treatments or medications are prescribed to the patient to reduce the involuntary muscle actions of the body, such as-.

• In the case of Huntington's Disease, doctors prescribe deutetrabenazine or tetrabenazine for controlling the random movements since there is no permanent cure for Huntington's Disease.
• If chorea is present as a part of tardive dyskinesia syndrome, drugs like valbenazine and deutetrabenazine are usually prescribed to the patient.
• Antipsychotic drugs are also considered if the patient has both chorea and mental health disorders such as irritability, anxiety, stress, etc.
• Baclofen and Benzodiazepine: some patients will respond to baclofen or benzodiazepines, and it is worthwhile to prescribe the drugs and closely observe the effects and side effects, usually related to sedation and falls
• For rheumatic fever-associated chorea, antibiotics are prescribed to the patient.
• Children with Sydenham chorea are not prescribed medicines as they usually get better in a few years. However, to control the movements, the patient may be prescribed medications for symptomatic relief.
• If chorea has appeared due to intake of a certain drug, the patient is then asked to discontinue the drug.

Surgical options

In some patients with chorea, deep brain stimulation may be used. Stimulation of the globus pallidus is an established treatment in generalised, and in some forms of focal dystonia, and early reports suggested a modest benefit in choreoathetosis and in chorea acanthocytosis. However, its use is subject to more research.

Review

Reviewed by Dr. Vivek Kumar, Senior Director, Neurosciences & Neurology.