Huntington disease vs Wilson disease and Sydenham chorea
How to tell Huntington disease and Wilson disease and Sydenham chorea apart when a board question makes them look alike.
Huntington is adult AD chorea-dementia; Wilson is copper/liver/KF rings; Sydenham is post-strep child chorea.
Side by side
Huntington disease causes adult-onset progressive chorea, psychiatric symptoms, dementia, caudate atrophy, autosomal dominant inheritance, and CAG repeat expansion with anticipation.
Wilson disease causes movement/psychiatric symptoms with liver disease, Kayser-Fleischer rings, low ceruloplasmin, and copper accumulation; Sydenham chorea is post-streptococcal childhood chorea linked to rheumatic fever.
Mutant huntingtin causes degeneration of caudate and striatal indirect pathway neurons, with loss of GABA and acetylcholine signaling in medium spiny neurons.
Wilson disease results from ATP7B copper transport failure; Sydenham chorea is autoimmune basal ganglia involvement after group A streptococcal infection.
Family history, caudate atrophy, genetic CAG expansion, chorea, and psychiatric decline support Huntington disease.
Low ceruloplasmin, liver disease, KF rings, and high copper support Wilson; recent strep/rheumatic fever signs support Sydenham chorea.
The trap
Chorea overlaps, so age, inheritance, liver/copper clues, and post-strep timing decide the cause.
The repair move
For chorea, sort by adult inherited degeneration, treatable copper disease, or post-strep autoimmune disease.
Try a free question
CoreAn adult has progressive chorea, depression, cognitive decline, family history of similar symptoms, caudate atrophy, and expanded CAG repeats. Which diagnosis is most likely?
Show answer and explanation
Answer: A. Huntington disease. Adult-onset progressive chorea with caudate atrophy and autosomal dominant CAG expansion supports Huntington disease.
Why the other choices are tempting
- B. Wilson disease adds liver disease, copper abnormalities, and Kayser-Fleischer rings.
- C. Sydenham chorea is post-streptococcal childhood chorea.
- D. Parkinson disease causes bradykinesia, rigidity, and resting tremor.
- E. Tourette syndrome causes motor and vocal tics, not caudate atrophy dementia.
Flashcards
Tap a card to flip it.
What inheritance pattern supports Huntington disease?
Autosomal dominant inheritance with anticipation supports Huntington disease.
What brain structure atrophies in Huntington disease?
The caudate nucleus atrophies in Huntington disease.
Quick answers
What is the fastest way to tell Huntington disease from Wilson disease and Sydenham chorea?
Huntington is adult AD chorea-dementia; Wilson is copper/liver/KF rings; Sydenham is post-strep child chorea.
What is the key clue for Huntington disease?
Huntington disease causes adult-onset progressive chorea, psychiatric symptoms, dementia, caudate atrophy, autosomal dominant inheritance, and CAG repeat expansion with anticipation.
What is the key clue for Wilson disease and Sydenham chorea?
Wilson disease causes movement/psychiatric symptoms with liver disease, Kayser-Fleischer rings, low ceruloplasmin, and copper accumulation; Sydenham chorea is post-streptococcal childhood chorea linked to rheumatic fever.
What is the common trap with Huntington disease vs Wilson disease and Sydenham chorea?
Chorea overlaps, so age, inheritance, liver/copper clues, and post-strep timing decide the cause.