In the previous articles of the “Approach to Pediatric Neurology Signs and Symptoms” series, I explored the evaluation of children presenting with movement disorders. To ensure a systematic and rational approach, the “CELE” framework was applied, encompassing:
- C – Confirmation of signs and symptoms by identifying Constellations, Characteristic features, and Clues
- E – Emergency considerations
- L – Localization of the site of the lesion
- E – Evaluation for Etiology
This article focuses on the approach to children presenting with chorea, a common type of movement disorder. The “CELE” framework will be used again to systematically assess and manage these cases.
Chorea is a movement disorder characterized by irregular, arrhythmic, and rapid movements that are typically involuntary and occur in a jerky or dancing manner. These movements are often unpredictable and can affect various parts of the body, including the limbs, face, and trunk, both proximal and distal. Chorea can present with random movements that integrate into voluntary actions, resulting in a migratory pattern of abnormal movements. It can interfere with normal motor function and may be accompanied by other symptoms such as changes in muscle tone and difficulties with voluntary movements.
Ballismus or ballism, is a severe form of chorea characterized by sudden, violent, and flinging movements of the limbs, often involving the proximal muscles. These movements are typically described as forceful and throwing motions. Ballismus usually affects one side of the body (hemiballismus) but can also occur bilaterally.
In the previous article, I emphasized the importance of phenomenology in the approach to children with movement disorders. The following table illustrates the application of phenomenology in the description of chorea and ballism:
Table 1. Phenomenology of chorea and ballism
The overall approach to chorea, based on the CELE framework, is outlined in Algorithm 1. Next, I will discuss how to approach a child with chorea using the CELE Framework.
C- Confirmation
Abnormal movements with the phenomenology described above are suggestive of chorea. To confirm a diagnosis of chorea, a thorough clinical examination is conducted as follows.
Tests and clinical confirmation for chorea involves several clinical assessments to evaluate the presence and extent of involuntary, irregular movements. Motor impersistence is tested by asking the patient to maintain a specific posture or movement, such as holding their arm outstretched, and noting their ability to sustain this position despite involuntary movements. St. Vitus dance, a term historically used to describe chorea, is observed by looking for rapid, unintentional, and erratic movements, often affecting the limbs and face. The darting tongue test involves asking the patient to protrude their tongue and observing for rapid, irregular movements or involuntary deviations. Finally, the milk maid’s grip test assesses for the presence of chorea by asking the patient to squeeze the examiner’s fingers, with the presence of a characteristic, irregular grip suggesting chorea. Together, these tests help clinicians diagnose and evaluate the severity of chorea.
On the other hand, chorea can mimic seizures, especially if the movements are intermittent and paroxysmal. However, children with seizures often have impaired awareness, and their abnormal movements are typically more patterned and stereotypic. Chorea can be wrongly diagnosed as muscle weakness, especially if it involves the legs and is severe. However, choreiform movements should also be present in the rest of the body. Chorea should be differentiated from other movement disorders, such as tics and athetosis; therefore, the phenomenology is important. Athetosis involves slower, writhing movements predominantly affecting the distal extremities. Tics are repetitive, patterned movements without impaired awareness and are partially suppressible.”
Again, a constellation of signs and symptoms can help physicians make a specific diagnosis. For example, if chorea is associated with joint and cardiac symptoms and is of short duration, it may suggest rheumatic or Sydenham’s chorea. Chorea associated with a history of severe neonatal jaundice and developmental delay is suggestive of dyskinetic cerebral palsy.
E – Emergency
Recognizing danger signs and symptoms, such as sudden or subacute onset, altered mental status, and other neurological abnormalities, is crucial for excluding serious conditions like viral encephalitis, autoimmune encephalitis, and neurodegenerative diseases. Persistent focal or unilateral chorea may indicate the presence of a structural lesion. Identifying these associated features helps clinicians differentiate between chorea and other potentially serious and life-threatening conditions, ensuring accurate diagnosis and prompt, effective management.
L- Localization
When a child presents with chorea, several areas of the brain may be affected, primarily involving the basal ganglia, which is crucial for coordinating movement. Key regions include:
1. Caudate Nucleus: This part of the basal ganglia is often involved in chorea, leading to the irregular and involuntary movements characteristic of the disorder.
2. Putamen: Also part of the basal ganglia, the putamen plays a role in regulating movement, and its involvement can contribute to chorea symptoms.
3. Globus Pallidus: This structure, another component of the basal ganglia, helps control voluntary movements. Dysfunction here can also be associated with chorea.
4. Subthalamic Nucleus: Located in the diencephalon, this nucleus can influence motor control and may be involved in the pathophysiology of chorea.
In principle, if chorea involves both sides of the body, the pathology typically affects the basal ganglia structures bilaterally, suggesting a more generalized issue within these areas. Conversely, if the chorea is unilateral or focal, it may indicate a localized lesion within the basal ganglia, affecting only one side of the brain
E- Evaluation for Etiology
Evaluation of the etiology of chorea depends on several factors. These include the age of onset—whether it occurs early (1-2 years) or later in life; the mode of onset and time course, which can be acute, insidious, or paroxysmal; and the distribution of movements, which may be focal, unilateral, or bilateral. Additionally, associated signs and symptoms play a crucial role in narrowing down the cause. It is important to recognize potentially serious or treatable underlying conditions, such as emergency conditions, structural lesions, rheumatic fever, Wilson’s disease, or medication-related effects. A thorough evaluation based on these parameters helps in accurately diagnosing the underlying cause and guiding appropriate management. Individual diseases causing chorea are detailed in Algorithm 2.
Summary
In summary, this article discusses the approach to diagnosing and managing pediatric chorea using the CELE Framework, which stands for Confirmation, Emergency considerations, Localization, and Evaluation for Etiology. The CELE Framework provides a systematic method for assessing chorea by confirming symptoms, considering emergency conditions, localizing brain lesions, and evaluating the etiology. Chorea is characterized by irregular, involuntary movements and can be confused with other movement disorders or conditions like seizures and muscle weakness. Confirmatory tests include assessing motor impersistence, St. Vitus dance, darting tongue movements, and the milk maid’s grip. Emergency considerations involve identifying signs of serious conditions such as viral or autoimmune encephalitis and structural lesions. Localization focuses on brain structures like the caudate nucleus, putamen, globus pallidus, and subthalamic nucleus, noting that bilateral chorea suggests generalized basal ganglia involvement, while unilateral or focal chorea points to localized lesions. Evaluation for etiology considers factors like age of onset, mode of onset, movement distribution, and associated symptoms to identify underlying causes such as rheumatic fever, Wilson’s disease, or medication effects.
Algorithm 1. The overall approach to chorea
Algorithm 2. Individual diseases causing chorea
References:
1. Albin, R. L. (1995). The pathophysiology of chorea/ballism and Parkinsonism. Parkinsonism & Related Disorders, 1(1), 3-11. https://doi.org/10.1016/1353-8020(95)00011-t. PMID: 18590996.
2. Baizabal-Carvallo, J. F., & Cardoso, F. (2020). Chorea in children: etiology, diagnostic approach, and management. Journal of Neural Transmission (Vienna), 127(10), 1323-1342. https://doi.org/10.1007/s00702-020-02238-3. Epub 2020 Aug 9. PMID: 32776155.
3. Beier, K., Lui, F., & Pratt, D. P. (2024). Sydenham Chorea. In StatPearls (pp. 1-11). Treasure Island (FL): StatPearls Publishing. PMID: 28613588.
4. Jankovic, J., & Hallett, M. (2020). Ballismus and Chorea. In Principles and Practice of Movement Disorders (pp. 267-278). Elsevier.
Author Information
Kyaw Linn
Professor, Paediatric Neurology
Senior Consultant Paediatrician
