Huntington’s disease HD: differential diagnosis
Evidence-based neurology checklist on huntington’s disease hd: differential diagnosis: Huntington’s disease-like 1 (HDL1) This is caused by a prion protein gene mutation It presents with personality change in early to mid-childhood It may also manifest with chorea, myoclonus, and seizures…
Huntington’s disease-like 1 (HDL1)
- This is caused by a prion protein gene mutation
- It presents with personality change in early to mid-childhood
- It may also manifest with chorea, myoclonus, and seizures
Huntington’s disease-like 2 (HDL2)
Huntington’s disease-like 3 (HDL3)
Huntington’s disease-like 4 (HDL4, SCA17)
Spinocerebellar ataxia (SCA)
Other neurodegenerative causes of chorea
Other causes of HD phenotype
Other causes of chorea
References
- Schneider SA, Walker RH, Bhatia KP. The Huntington's disease-like syndromes: what to consider in patients with a negative Huntington's disease gene test. Nat Clin Pract Neurol 2007; 3:517-525.
- Martino D, Stamelou M, Bhatia K. The differential diagnosis of Huntington's disease-like syndromes: 'red flags' for the clinician. JNNP 2013; 84:650-656.
- Schneider S, Bird T. Huntington's disease, Huntington's disease look-alikes, and benign hereditary chorea: what's new? Mov Disord Clin Pract 2016; doi: 10.1002/mdc3.12312 (Epub ahead of issue).
- Walker RH. Update on the non-Huntington's disease choreas with comments on the current nomenclature. Tremor Other Hyperkinet Mov (N Y) 2012; 2. pii: tre-02-49-211-1.
- Anderson DG, Haagensen M, Ferreira-Correia A, et al. Emerging differences between Huntington's disease-like 2 and Huntington's disease: a comparison using MRI brain volumetry. Neuroimage Clin 2019; 21:101666.
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