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Published on: November 21, 2013
[Update on chorea]
F Vázquez-Sánchez1, E Rodríguez-Martínez, A Arés-Luque
1Servicio de Neurología, Complejo Asistencial de León, León, España.
Insights
Chorea is an involuntary movement disorder with diverse causes, including genetics and medications. Early diagnosis and targeted treatment are crucial for managing choreic syndromes.
Area of Science:
- Neurology
- Movement Disorders
Context:
- Chorea is characterized by involuntary, unpredictable movements affecting any muscle group.
- The differential diagnosis is broad, encompassing genetic, acquired, infectious, toxic, metabolic, and pharmaceutical causes.
Purpose:
- To review the phenomenology, pathophysiology, causes, diagnosis, and recent treatment findings for choreic movements.
- To highlight key aspects of diagnosing and managing choreic syndromes.
Summary:
- Huntington's disease is the most common hereditary cause; diagnosis is challenging with negative genetic studies.
- Medication-induced chorea is frequent; hemichorea necessitates ruling out basal ganglia structural lesions.
- Treatment focuses on addressing the underlying cause and providing symptomatic relief.
Impact:
- Provides a comprehensive overview for clinicians managing patients with chorea.
- Emphasizes the importance of etiological diagnosis and tailored therapeutic strategies.
- Informs genetic counseling for hereditary choreas.
Introduction:
Chorea is a brief, random, involuntary movement that can affect any muscle group and flows in an unpredictable manner from one part of the body to another. Choreic movements are present at rest, increase with distracting manoeuvres, can be partially suppressed and often disappear during sleep. The differential diagnosis of choreic syndromes is very wide and includes both genetic and acquired causes (cerebrovascular disease and space-occupying lesions, immune-based choreas, choreas caused by infection, toxic and metabolic encephalopathies, and choreas induced by pharmaceuticals).
Development:
We review the phenomenology and pathophysiology of choreic movements, the most frequent causes, the diagnostic process and the most important recent findings in its treatment.
Conclusions:
Huntington's disease is the most frequent cause of hereditary chorea. In patients with a familial picture suggesting Huntington's disease, but with a negative genetic study, the chances of reaching a definitive diagnosis are, today, very low. Medication is the most frequent cause of acquired chorea. When dealing with a case of hemichorea, it is essential to rule out structural insult of the basal ganglia or their connections. Treatment of choreas must be aimed at correcting their cause if it is reversible, although the patient must always be given symptomatic treatment if the intensity and functional repercussions of the chorea require it. In cases of hereditary choreas, prevention could involve genetic counselling aimed at affected patients and their relatives.
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