Down’s syndrome

A major challenge highlighted in clinical trials for Down’s syndrome is the ability to sensitively distinguish the global intellectual impairment from domain-specific cognitive deficits¹. Our recommended test battery for research of Down’s syndrome is specifically developed to overcome this problem, assessing the key cognitive domains often impaired in individuals with Down’s syndrome, as well as domains likely to be affected by interventions.

The tests in this battery have demonstrated sensitivity to disease-specific cognitive deficits in Down’s syndrome, including those related to hippocampal dysfunction² and frontal lobe dysfunction³, findings that accord well with animal models of the underlying brain pathology^4,5. 

Additionally, in Down’s syndrome, the gametic triplication of chromosome 21 results in the increased production of proteins thought to be pivotal in the development of Alzheimer’s disease (APP and ultimately amyloid β). This leads to an increased likelihood of adults with Down's syndrome developing Alzheimer's disease in comparison to the general population, as they age. Alzheimer's disease in Down's syndrome develops with similar underlying neuropathology and cognitive deficits to those seen in Alzheimer's disease for typically developing adults^6,7, the tests in this battery are also sensitive to the changes in cognitive function seen in Alzheimer's disease.

Measures

• Multitasking
• Episodic memory
• Executive function
• Processing speed

CANTAB Tests

• Motor Screening Task (MOT): 2 minutes
• Paired Associates Learning (PAL): 8 minutes
• Reaction Time (RTI): 3 minutes
• Multitasking Test (MTT): 8 minutes

Research

The tests in this battery have shown episodic memory and recognition memory to be key cognitive domains impaired in people with Down’s syndrome.
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Key References

Haydar TF, Reeves RH. Trisomy 21 and early brain development. Trends Neurosci. 2012 Feb;35(2):81-91.

Edgin JO. Cognition in Down syndrome: a developmental cognitive neuroscience perspective. Wiley Interdiscip Rev Cogn Sci. 2013 May;4(3):307-17.

Das D, Phillips C, Hsieh W, Sumanth K, Dang V, Salehi A. Neurotransmitter-based strategies for the treatment of cognitive dysfunction in Down syndrome. Prog Neuropsychopharmacol Biol Psychiatry. 2014 Oct 3;54:140-8.

Deutsch SI, Burket JA, Benson AD. Targeting the α7 nicotinic acetylcholine receptor to prevent progressive dementia and improve cognition in adults with Down's syndrome. Prog Neuropsychopharmacol Biol Psychiatry. 2014 Oct 3;54:131-9.

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1. _{Deutsch SI, Burket JA, Benson AD. Targeting the α7 nicotinic acetylcholine receptor to prevent progressive dementia and improve cognition in adults with Down's syndrome. Prog Neuropsychopharmacol Biol Psychiatry. 2014 Oct 3;54:131-9.}
2. _{Pennington BF, Moon J, Edgin J, Stedron J, Nadel L. The neuropsychology of Down syndrome: evidence for hippocampal dysfunction. Child Dev. 2003 Jan-Feb;74(1):75-93.}
3. _{Van der Molen MJ, Van der Molen MW, Ridderinkhof KR, Hamel BC, Curfs LM, Ramakers GJ. Attentional set-shifting in fragile X syndrome. Brain Cogn. 2012 Apr;78(3):206-17.}
4. _{De la Torre R, De Sola S, Pons M, Duchon A, de Lagran MM, Farré M, Fitó M, Benejam B, Langohr K, Rodriguez J, Pujadas M, Bizot JC, Cuenca A, Janel N, Catuara S, Covas MI, Blehaut H, Herault Y, Delabar JM, Dierssen M. Epigallocatechin-3-gallate, a DYRK1A inhibitor, rescues cognitive deficits in Down syndrome mouse models and in humans. Mol Nutr Food Res. 2014 Feb;58(2):278-88.}
5. _{Wallace TL, Ballard TM, Pouzet B, Riedel WJ, Wettstein JG. Drug targets for cognitive enhancement in neuropsychiatric disorders. Pharmacol Biochem Behav. 2011 Aug;99(2):130-45.} 
6. _{Oliver, C. and Holland, A.J. (1986). Down’s syndrome and Alzheimer’s disease: A review. Psychological Medicine, 16, 307-322.}
7. _{Oliver, C., Crayton, Holland, A.J., Hall, S., & Bradbury, J. (1998) A four year study of age related cognitive and behavioural change in adults with Down’s syndrome. Psychological Medicine, 28, 1365-1377.}