Huntington’s disease

Cognitive deficits are common in individuals with Huntington’s disease, some of which are detectable up to 10 years before formal diagnosis¹. Our recommended test battery for Huntington’s disease is highly sensitive to these cognitive deficits in patients and also to cognitive deterioration over time, even in early disease².

The tests in this battery capture the key cognitive domains often impaired at different stages of Huntington’s disease, as well as the cognitive domains likely to be affected by medications. The tests in this battery can also detect unexpected and adverse effects of exploratory novel interventions on cognition³, as well as sensitively detect cognitive enhancements. This test battery is also clinically relevant, with performance correlating with functional impairment in Huntington’s disease patients⁴.

The One Touch Stockings of Cambridge (OTS) task was shown to correlate with loss of striatal dopamine receptors in the striatum of Huntington’s patients, when assessed over time⁵, demonstrating the sensitivity of this test to the underlying pathology of Huntington’s disease/HD.

Measures

• Motor Skills
• Processing speed
• Episodic memory
• Executive function

CANTAB Tests

• Motor Screening Task (MOT): 2 minutes
• Reaction Time (RTI): 3 minutes
• Paired Associates Learning (PAL): 8 minutes
• One Touch Stockings of Cambridge (OTS): 10 minutes
• Spatial Span (SSP): 5 minutes

Research 

When completing the One Touch Stockings of Cambridge (OTS), Huntington’s disease patients took longer to solve the problems and therefore have deficits in executive function.¹

_{Watkins et al., (2000)}

Key References

Novak MJ, Tabrizi SJ. Huntington's disease. BMJ. 2010 Jun 30;340:c3109.

Papoutsi M, Labuschagne I, Tabrizi SJ, Stout JC. The cognitive burden in Huntington's disease: pathology, phenotype, and mechanisms of compensation. Mov Disord. 2014 Apr 15;29(5):673-83.

Mason S, Barker RA. Progress in Huntington's disease: the search for markers of disease onset and progression. J Neurol. 2015 Mar 21. 262: 1990. doi:10.1007/s00415-015-7700-0

Ho AK, Sahakian BJ, Brown RG, Barker RA, Hodges JR, Ané MN, Snowden J, Thompson J, Esmonde T, Gentry R, Moore JW, Bodner T; NEST-HD Consortium. Profile of cognitive progression in early Huntington's disease. Neurology. 2003 Dec 23;61(12):1702-6.

Pavese N, Andrews TC, Brooks DJ, Ho AK, Rosser AE, Barker RA, Robbins TW, Sahakian BJ, Dunnett SB, Piccini P.. Progressive striatal and cortical dopamine receptor dysfunction in Huntington's disease: a PET study. Brain. 2003 May;126(Pt 5):1127-35.  

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1. _{Papoutsi M, Labuschagne I, Tabrizi SJ, Stout JC. The cognitive burden in Huntington's disease: pathology, phenotype, and mechanisms of compensation. Mov Disord. 2014 Apr 15;29(5):673-83.}
2. _{Ho AK, Sahakian BJ, Brown RG, Barker RA, Hodges JR, Ané MN, Snowden J, Thompson J, Esmonde T, Gentry R, Moore JW, Bodner T; NEST-HD Consortium. Profile of cognitive progression in early Huntington's disease. Neurology. 2003 Dec 23;61(12):1702-6.}
3. _{Blackwell AD, Paterson NS, Barker RA, Robbins TW, Sahakian BJ. The effects of modafinil on mood and cognition in Huntington's disease. Psychopharmacology (Berl). 2008 Jul;199(1):29-36.}
4. _{Hamilton JM, Salmon DP, Corey-Bloom J, Gamst A, Paulsen JS, Jerkins S, Jacobson MW, Peavy G. Behavioural abnormalities contribute to functional decline in Huntington's disease. J Neurol Neurosurg Psychiatry. 2003 Jan;74(1):120-2.}
5. _{Pavese N, Andrews TC, Brooks DJ, Ho AK, Rosser AE, Barker RA, Robbins TW, Sahakian BJ, Dunnett SB, Piccini P. Progressive striatal and cortical dopamine receptor dysfunction in Huntington's disease: a PET study. Brain. 2003 May;126(Pt 5):1127-35}