• The major and most common symptom of chorea is “milkmaid grip” which is characterized by the action of holding a utensil, but the thumb is pointed in the upward direction. Affected individuals also experience an involuntary movement of the tongue which often sticks out of the mouth.
• Chorea being described as a hyperkinetic movement disorder is pretty different from ataxia and Parkinson’s disease. The movements in chorea are involuntary in nature and are not in the patient’s control. The irregular and involuntary movements presented in patients with chorea are neither rhythmic nor repetitive in nature. The choreform movements can be basically described as dance-like motions along with writhing and twisting. Individuals experience great deal of difficulty while walking and exhibit odd leg movements and postures [6].
• The most common symptom of Sydenham’s chorea is “harlequin tongue” characterized by involuntary movements of the tongue that pops in when the individual tries to stick it out.

• Crying

  Other symptoms may include: Changes in handwriting Loss of fine motor control, especially of the fingers and hands Loss of emotional control, with bouts of inappropriate crying or laughing Symptoms of RF may be present. [medlineplus.gov]

  A Cry for Help? Despite its continued claim that it has zero cases of COVID-19, the regime has acknowledged its troubled health care system, and North Korean officials have been quietly requesting help from their international interlocutors. [brookings.edu]

  The next moment she had given a cry of anguish and begun the frantic search Elaine had witnessed during tea. "Oh! My ring!" she wailed. [newspapers.com]

• El Salvador

  Salvador (SV), Guam (GU), Haiti (HT), Marshall Islands (MH), Federated States of Micronesia (FM), Northern Mariana Islands (MP), Palau (PW), Panama (PA) (as well as Panamanian Balboa), Puerto Rico (PR), Timor-Leste (TL), Turks and Caicos Islands (TC) [en.wikipedia.org]

• Jaundice

  Neonatal jaundice[edit] Neonatal jaundice is the other chief complication that leads to the basal ganglia damage associated with this condition. Jaundice is caused by hyperbilirubinemia, or abnormally high levels of bilirubin in the blood. [en.wikipedia.org]

  Causes include asphyxia, neonatal jaundice, Huntington's chorea, cerebrovascular disease and trauma. Management can be difficult but treatment options include medications (eg, diazepam, haloperidol, tetrabenazine), surgery and retraining techniques. [patient.info]

• Muscle Twitch

  Symptoms of chorea may include: Clumsiness Difficulty walking Difficulty writing Involuntary facial expressions Muscle twitching Muscle weakness Poor muscle tone Slurred speech Children with chorea may seem clumsy, restless, or hyperactive. [loyolamedicine.org]

• Fidgeting

  Mild cases of chorea are sometimes mistaken for general restlessness or fidgeting, but severe chorea can involve flailing movements or an inability to walk. [loyolamedicine.org]

  People who are not familiar with chorea also describe the movements as restlessness or fidgeting. Chorea can occur with other abnormal movements, such as athetosis, a slower, writhing movement, referred to as choreoathetosis. [huntingtonsdiseasenews.com]

  Choreic movements can often look like restlessness or fidgeting to those not familiar with them. They can usually be reduced to some degree when the affected person concentrates on suppressing them. [movementdisorders.ufhealth.org]

  Chorea symptoms can range from minor movements, such as fidgeting, to severe uncontrolled arm and leg movements. It can also interfere with: speech swallowing posture gait Chorea symptoms usually depend upon the condition causing it. [healthline.com]

• Aggressive Behavior

  Treatment Medication Medical treatment to correct imbalances in dopamine and other brain chemicals and processes that help regulate movement and emotion can help control abnormal movements and aggressive behavior. [uclahealth.org]

  Medication and non-medical care for depression and aggressive behavior may be required. Prognosis The progression of the disease leads to complete dependency in daily life, which results in patients requiring full-time care, and finally death. [orpha.net]

• Chorea

  […] appear to flow from one muscle to the next hereditary chorea chorea Hereditary benign chorea CHOREA, BENIGN HEREDITARY; BHC Bch Hereditary Progressive Chorea Without Dementia Benign familial chorea BHC Statements Identifiers Sitelinks Wikipedia (27 entries [wikidata.org]

  Chorea as a nonprogressive abnormality is often associated with Sydenham chorea in the pediatric population. Benign hereditary chorea is a condition where chorea presents before age 5 years and runs a very slowly progressive course. [ncbi.nlm.nih.gov]

  Retrieved from https://www.yourdictionary.com/CHOREA Noun (plural choreas or choreae or choreæ) An Ancient Greek circular dance accompanied by a chorus. [yourdictionary.com]

• Seizure

  Patients with chorea-acanthocytosis carrying the described rare mutation can present with focal, treatment-resistant seizures. [ncbi.nlm.nih.gov]

  • Although a rare condition, deficiency of glucose transporter 1 is increasingly recognized as a cause of chorea and different types of seizure disorders in childhood. • PRRT2 gene mutation is a major cause of hereditary paroxysmal chorea, seizures, [medlink.com]

• Agitation

  Psychiatric symptoms such as depression, psychosis and agitation are similarly managed as any other psychiatric condition. [movementdisorders.org]

  Treatment with deutetrabenazine is also associated with akathisia, agitation, and restlessness and may cause parkinsonism in some patients. [neurologyadvisor.com]

  Side effects most often include low blood pressure, nausea, insomnia, agitation, and headache. Tetrabenazine ( Nitoman ® in Canada and some European countries; Xenazine ® in the U.S. and some European countries). [hddrugworks.org]

  Valproic acid, which acts in part via a GABAergic mechanism, has, in a limited number of uncontrolled cases, ameliorated not only the agitation sometimes seen in persons with HD but also the movement problem. [17] However, no systematic studies have been [emedicine.medscape.com]

• Action Tremor

  […] include Wilson's Disease and severe essential tremor (ET is usually postural but when servere can be seen at rest or even with movement) Resting Tremor Postural Tremor Action Tremor Description Tremor when skeletal muscle is at rest. [stanfordmedicine25.stanford.edu]

  Kinetic or action tremor - occurs during voluntary active movement of an upper body part. Intention tremor is one that occurs when a tremor worsens as a goal-directed hand movement nears its intended target. [patient.info]

  The tremor is called an action tremor, meaning that the tremor typically becomes noticeable in the arms when they are being used. Patients often report that the tremor in ET improves significantly with alcohol. [med.umich.edu]

Preliminary examination of the symptoms followed by a past medical history is done. Thereafter, laboratory studies are carried out to analyze the underlying etiology. Genetic testing is carried out for diagnosing Huntington’s disease. Imaging studies such as MRI and Position emission tomography are also indicated.

• Treatment for chorea majorly depends on the type of disease that caused it. Primary medications should be able to treat the disease and control the symptoms. In cases when Parkinson’s disease is the cause, medications are of little use; though they can be helpful for effectively managing the symptoms.
• Antipsychotic medications that mediate dopamine are usually administered for controlling chorea. These include olanzapine, fluphenazine, quetiapine, haloperidol and risperidone. In certain cases, benzodiapines such as klonopin and clonazepam may also be given [9].
• Surgical procedures are employed when other methods of treatment fail to bring about the desired results. In this method, deep brain stimulation by way of implantation of electrodes in the brain helps in appropriate regulation of the nerve impulses. This procedure however, does not cure chorea but only helps in the effective management of symptoms [10].

The prognosis of the condition depends on the underlying etiology. If infections are the cause, then antibiotics can cure chorea; however, if Huntington’s disease is the cause, then the condition cannot be cured. Endocrine conditions that cause chorea eventually improve with treatment of the endocrine disorder.

Chorea is associated with various conditions. These include AIDS (Acquired Immunodeficiency Syndrome), certain drugs such as neuroleptics and levodopa, infections and genetic diseases such as Huntington’s disease. Studies have postulated the fact that individuals with Huntington’s disease are at a 50% increased risk of developing chorea. In addition, immunity related disorders such as Systemic lupus erythematosus and endocrine disorders like hypoglycemia can also increase the likelihood of developing chorea.

Syndenham’s chorea can also accompany as a secondary complication to rheumatic fever. It occurs in only 20% of children as well as adolescents [3].

The exact incidence of chorea is unknown. The prevalence of rate of Huntington’s disease is about 5 to 10 cases per 100,000 individuals [4].

Benign hereditary chorea is a rare disorder with an incidence rate of 1 case per 500,000 individuals. Another condition which gives rise to chorea is Wilson's disease. It is also a rare form of disorder occurring only in 30 cases per 1 million individuals.

Neurophysiological abnormalities that originate in the motor cortex and basal ganglia give rise to hyperkinetic movements. Basal ganglia and motor cortex include neuronal network such as caudate nuclei, globus pallidus externa, globus pallidus interna, thalamus and subthalamus. From the globus pallidus interna arise several GABAergic projections that extend up to the thalamus. Any form of dysfunction in these projections can lead to chorea [5].

It gets difficult to prevent chorea once it has occurred due to a reactive action of certain drugs; therefore, it becomes paramount that the use of such drugs is prevented. Genetic counselling during pregnancy can help in detection of Huntington’s disease so that patients can make informed decisions. Antibiotic prophylaxis should be continued for at least 10 years in the patients who have suffered from rheumatic fever.

Chorea is a type of neurological disorder belonging to the group of dyskinesias. Chorea often accompanies a type of disorder known as athetosis which causes the affected individual to experience writhing and twisting movements. Severe form of chorea characterized by thrashing motions is known as ballism [1]. Development of chorea is often a primary characteristic of Huntington’s disease which is also a movement disorder. The treatment depends on the underlying etiology and severity of the condition [2].

Chorea is a disorder involving involuntary movements of the certain parts of the body. It belongs to the group of neurological disorders known as dyskinesias. Huntington’s disease is the major known causative factor. Other less common factors include infections, drugs, autoimmune diseases, genetic diseases and rheumatic fever. Individuals with chorea experience involuntary movements of legs, hands, feet and tongue. The involuntary movements occur by themselves and are not rhythmic or repetitive in nature.

Treating the causative factor helps in controlling the symptoms and managing the condition. Medications such as antipsychotics are given for controlling the symptoms. When these aren't enough, surgery is carried out to conduct deep brain stimulation.

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