Chorea is a type of movement disorder, characterized by involuntary movements of the hands and feet. The word “chorea” is derived from Greek language, meaning a form of dance.
Presentation
- 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.
Entire Body System
- Fever
Abstract Despite improvements in socio-economic status and the standard of health care services, rheumatic fever continuous to occur in Saudi Arabia, although with decreasing frequency. [ncbi.nlm.nih.gov]
What Causes Rheumatic Fever? Rheumatic fever results from an inflammatory reaction to certain group A streptococcusbacteria. [webmd.com]
The incidence of acute rheumatic fever and rheumatic heart disease is not declining. [en.wikipedia.org]
Anti-inflammatory treatment for carditis in acute rheumatic fever. [hippoed.com]
- Disability
The patient was referred to our hospital for disabling chorea 16 years after PTE. Neurological examination revealed choreatic movements in the four extremities. [ncbi.nlm.nih.gov]
Presents in childhood or adolescence with mild disability. Helped by alcohol and beta-blockers. Progressive myoclonic encephalopathies: appear as part of a range of other neurological disorders. [patient.info]
- Camping
The identification of PDE10A mutations as a cause of chorea further motivates the study of cAMP signaling in MSNs and highlights the crucial role of striatal cAMP signaling in the regulation of basal ganglia circuitry. [ncbi.nlm.nih.gov]
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- Chills
When a self-righteous majority levels undiluted hatred at a small group of refugees, and thinks itself ordinary, it's downright chilling. [koreaexpose.com]
(as of Mar 11) a) anyone who has symptoms of fever, coughing, difficulty of breathing or chills b) anyone who has been in close contact or provided care to a person known to be infected with COVID-19 within 14 days c) anyone who has visited, worked in [koreanair.com]
458,198 kL (16,181,100 cu ft) 2012 448,046 kL (15,822,600 cu ft) 2013 426,216 kL (15,051,700 cu ft) 2014 430,896 kL (15,216,900 cu ft) 2015 416,046 kL (14,692,500 cu ft) Consumption[edit] Makgeolli in a bowl with a ladle Makgeolli is usually served chilled [en.wikipedia.org]
A cold, hostile voice spoke from inside the house that sent chills down my spine. A blond haired beauty slowly emerged from the house with predatory eyes and a malicious smile. “Well Lavizo?” [royalroad.com]
- Surgical Procedure
Surgical procedures are available for medication-refractory dystonia, markedly affecting quality of life [ 4 ]. Primary pure dystonia is inherited in a mainly autosomal dominant pattern [ 5 ]. Two genes have been identified: DYT1 and DYT6. [patient.info]
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. [symptoma.com]
On Monday, a Seoul-based news website, the Daily NK, reported that Kim had undergone a “cardiovascular surgical procedure” and had largely recovered. [buzzfeednews.com]
These patients have continuous jerky, random, and uncontrollable movements, and so when they are posted for any surgical procedure, they are of special concern to anesthesiologists. [karnatakaanaesthj.org]
Gastrointestinal
- Diarrhea
Although the most common adverse effects are somnolence, diarrhea, dry mouth, and fatigue, the drug also comes with a black box warning for increased risk for depression and suicidality. [neurologyadvisor.com]
Skin
- Erythema
The patient did not have erythema marginatum, subcutaneous nodules or arthritis. Review of systems revealed no alopecia, photosensivity, rash, joint symptoms, chest pain or Raynaud's phenomenon. [academic.oup.com]
In 1889 Cheadle described the full rheumatic syndrome of carditis, polyarthritis, chorea, subcutaneous nodules, and erythema marginatum. [medlink.com]
In about 50 % of the cases symptoms of rheumatic fever precede the choreatic movement disorders, including polyarthritis, carditis, erythema annulare and subcutaneous papules. School age children are particularly affected by this. [lecturio.com]
Major criteria Minor criteria Polyarthritis Fever Carditis Arthralgia Subcutaneous nodules Elevated acute phase reactants Erythema marginatum Prolonged P-R interval on ECG Sydenham chorea Previous episode of rheumatic fever Cardiac involvement, especially [clinicaladvisor.com]
[…] tongue fasciculations ("bag of worms") and a "milk sign", which is a relapsing grip demonstrated by alternate increases and decreases in tension, as if hand milking.[5] Non-neurologic manifestations of acute rheumatic fever are carditis, arthritis, erythema [en.wikipedia.org]
- Skin Rash
[…] tending to spare the nasolabial folds Discoid rash Erythematosus raised patches with adherent keratotic scaling and follicular plugging; atrophic scarring may occur in older lesions Photosensitivity Skin rash as a result of unusual reaction to sunlight [academic.oup.com]
Systemic Lupus Erythematosus (SLE): Butterfly skin rash, purpura and arthropathy 4. Polycythemia Rubra Vera: Facial plethora and splenomegaly 5. Thyrotoxicosis: Tremor, tachycardia, lidlag and goitre (may be present) 6. [epomedicine.com]
These may include high fever, heart problem, joint pain or swelling, skin lumps or skin rashes, and nosebleeds. The health care provider will perform a physical exam. Detailed questions will be asked about the symptoms. [medlineplus.gov]
Penicillamine is probably the most potent copper chelating agent available and has been mostly used as the first line therapy for initial and long term management, although chronic treatment is associated with various side effects, mainly skin rash and [pmj.bmj.com]
Musculoskeletal
- 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]
Psychiatrical
- Fear
NORTH KOREA Kim Jong-un 'Flees Pyongyang over Coronavirus' North Korean leader Kim Jong-un appears to have fled Pyongyang for fear of the coronavirus epidemic there. [english.chosun.com]
News BTS tell fans to avoid their shows over coronavirus fears World Donald Kirk Coronavirus: Fear and anger in South Korea as president and secretive Donald Kirk News BTS cancel South Korea tour dates due to coronavirus fears Americas Trump appoints [independent.co.uk]
The alternative, they feared, would be a wider war with Russia and China–or even, as some warned, World War III. Finally, in July 1953, the Korean War came to an end. [history.com]
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The stare had been succeeded, incredibly, by a look of fear, and then hadTcome the hurried stumble round the corner and out of sight. Out of sight, but the corridor was long. [newspapers.com]
- Distractibility
Saccades in Huntington’s disease: initiation defects and distractibility. Neurology. 1987;37(3):364-70. doi:10.1212/WNL.37.3.364 [ Links ] 35. Rubin AJ, King WM, Reinbold KA, Shoulson I. [scielo.br]
Variability of movements (often increasing with attention) and distractibility from movements (often decreasing when patient is asked to perform another task). [stanfordmedicine25.stanford.edu]
Things began to change in the 1970s, when the US was distracted by the war in Vietnam and the Cold War with the Soviet Union. [edition.cnn.com]
Neurologic
- 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]
- Tremor
Tremor See also the separate Tremor article. This is a rhythmic movement of part of the body. Essential tremor and Parkinsonian tremor are the most common forms of tremo. [ 16 ]. [patient.info]
Objectives After completing this article, readers should be able to: Identify the important distinguishing features of chorea, dystonia, myoclonus, and tremor. Recognize important causes of chorea, dystonia, myoclonus, and tremor. [pedsinreview.aappublications.org]
[…] 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]
However, when tremors become persistent, it may be a sign of something else. Essential tremor (ET), also known as benign essential tremor, or familial tremor, is the most common movement disorder. [med.umich.edu]
Tremor and Other Hyperkinetic Movements. 5: 314. doi:10.7916/D8RJ4HM5. PMC 4502401. PMID 26196025. External links[edit] Classification D OMIM: 118700 MeSH: C565851 External resources GeneReviews: NKX2-1-Related Disorders Orphanet: 1429 [en.wikipedia.org]
- 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]
Workup
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
- 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].
Prognosis
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.
Etiology
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].
Epidemiology
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.
Pathophysiology
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].
Prevention
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.
Summary
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].
Patient Information
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.
References
- Bhidayasiri R, Truong DD. Chorea and related disorders. Postgrad Med J 2004; 80:527.
- Shannon KM. Treatment of chorea. Continuum 2007; 13:72.
- Zomorrodi A, Wald ER. Sydenham's chorea in western Pennsylvania. Pediatrics 2006; 117:e675.
- Berman SA. Chorea. In: Joseph AB, Young RR, eds. Movement Disorders in Neurology and Neuropsychiatry. 2nd ed. Malden, Mass: Blackwell Science; 1999:481-94.
- Li JY, Yong TY, Sebben R, Khoo E, Disney AP. Bilateral basal ganglia lesions in patients with end-stage diabetic nephropathy. Nephrology 2008; 13(1): 68-72
- Cardoso F, Seppi K, Mair KJ, et al. Seminar on choreas. Lancet Neurol 2006; 5:589.
- Klein C. The Wilson films--Huntington's chorea. Mov Disord. Dec 2011;26(14):2464-6.
- Dubinsky RM, Greenberg M, Di Chiro G, et al. Hemiballismus: study of a case using positron emission tomography with 18fluoro-2-deoxyglucose. Mov Disord. 1989;4(4):310-9.
- Grove VE Jr, Quintanilla J, DeVaney GT. Improvement of Huntingtons disease with olanzapine and valproate. N Engl J Med. Sep 28 2000;343(13):973-4.
- Edwards TC, Zrinzo L, Limousin P, Foltynie T. Deep brain stimulation in the treatment of chorea. Mov Disord 2012; 27:357.