Differential Diagnosis for Chorea
- Single most likely diagnosis
- Huntington's disease: This is a common cause of chorea, especially in adults, and is characterized by an expansion of a CAG repeat in the huntingtin gene, leading to progressive damage to the basal ganglia.
- Other Likely diagnoses
- Sydenham's chorea: This is a major criterion for acute rheumatic fever and typically occurs in children, often following a group A streptococcal infection.
- Benign hereditary chorea: A rare, autosomal dominant disorder that presents with non-progressive or slowly progressive chorea, often without other significant neurological symptoms.
- Chorea associated with autoimmune disorders (e.g., systemic lupus erythematosus, antiphospholipid syndrome): These conditions can cause chorea due to inflammation and autoantibody production affecting the basal ganglia.
- Do Not Miss (ddxs that may not be likely, but would be deadly if missed.)
- Wilson's disease: A genetic disorder leading to copper accumulation in the brain and liver, which can cause chorea, among other symptoms. If untreated, it can lead to severe neurological damage and death.
- Neuroacanthocytosis: A group of rare genetic disorders characterized by the presence of acanthocytes (abnormal red blood cells) and can lead to chorea, among other neurological symptoms.
- Chorea due to vascular causes (e.g., stroke, vasculitis): Vascular events can cause chorea by damaging the basal ganglia, and prompt recognition and treatment are crucial to prevent further damage.
- Rare diagnoses
- Dentatorubral-pallidoluysian atrophy (DRPLA): A rare, autosomal dominant disorder that can cause chorea, ataxia, and other neurological symptoms due to an expansion of a CAG repeat.
- Neurodegeneration with brain iron accumulation (NBIA): A group of rare genetic disorders characterized by iron accumulation in the brain, leading to various neurological symptoms, including chorea.
- Chorea associated with mitochondrial disorders: Certain mitochondrial disorders, such as MELAS syndrome, can cause chorea due to impaired energy production in the brain.