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Chorea

Choreas

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.
Down Syndrome
  • Rejecting the diagnosis too soon may lead to unnecessary treatment for other possible underlying aetiologies, especially in patients with Down Syndrome, known to be vulnerable for autoimmune disorders.[ncbi.nlm.nih.gov]
Hiccup
  • Chlorpromazine and haloperidol are used to relieve intractable hiccup. Propranolol or another beta-adrenoceptor blocking drug may be used to treat essential tremor or tremors associated with anxiety or thyrotoxicosis.[patient.info]
Jaundice
  • 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]
Systolic Murmur
  • Grade II ejection systolic murmur was noticed on cardiovascular examination. Echocardiography evaluation showed thickened aortic and mitral valve leaflets with mild to moderate degree of mitral regurgitation.[ncbi.nlm.nih.gov]
Hyperhidrosis
  • Myokymia, insomnia and hyperhidrosis are invariably present. There may be abnormalities on cerebrospinal fluid testing and VGKC-Abs can occasionally be absent.[ncbi.nlm.nih.gov]
Myokymia
  • ADCY5 genetic analysis should be performed in cases with a benign choreiform movement disorder even in the absence of facial myokymia.[ncbi.nlm.nih.gov]

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.

Neurophysiologic Abnormalities
  • Neurophysiological abnormalities that originate in the motor cortex and basal ganglia give rise to hyperkinetic movements.[symptoma.com]
Ischemic Changes
  • Magnetic resonance imaging (MRI) showed no significant abnormality on admission, but an ischemic change in her left pallidum appeared on day 7.[ncbi.nlm.nih.gov]

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.

Sex distribution
Age distribution

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 dyskinesiasHuntington’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

Article

  1. Bhidayasiri R, Truong DD. Chorea and related disorders. Postgrad Med J 2004; 80:527.
  2. Shannon KM. Treatment of chorea. Continuum 2007; 13:72.
  3. Zomorrodi A, Wald ER. Sydenham's chorea in western Pennsylvania. Pediatrics 2006; 117:e675.
  4. Berman SA. Chorea. In: Joseph AB, Young RR, eds. Movement Disorders in Neurology and Neuropsychiatry. 2nd ed. Malden, Mass: Blackwell Science; 1999:481-94.
  5. 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
  6. Cardoso F, Seppi K, Mair KJ, et al. Seminar on choreas. Lancet Neurol 2006; 5:589.
  7. Klein C. The Wilson films--Huntington's chorea. Mov Disord. Dec 2011;26(14):2464-6.
  8. 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.
  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.
  10. Edwards TC, Zrinzo L, Limousin P, Foltynie T. Deep brain stimulation in the treatment of chorea. Mov Disord 2012; 27:357.

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Last updated: 2019-07-11 22:54