HD Neuropsychological Assessments
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The present research project aims to generate a normative database for cognitive performance in the neuropsychological test battery in Huntington Disease (HD). It is essential as it allows clinicians to evaluate results obtained by HD patients in neuropsychological tests and be informed about the degree of current cognitive dysfunction (i.e. mild, moderate, severe) and can help evaluate monitoring of their quality of life.

In this research project, we have analyzed data collected from Enroll-HD and its precursor Registry, the world-wide observational study for research into Huntington disease.

Neuropsychological Assessments

The cognitive battery used in this project consists of five neuropsychological tests measuring different cognitive domains known to be affected by Huntington Disease (HD). The used neuropsychological assessments:
  • Symbol Digit Modalities Test (SDMT) [1, 2] 
  • Categorical Fluency [3]
  • Stroop Color Naming Test (SCNT), Stroop Word Reading Test (SWRT) and Stroop Interference Test (SIT) [4]
  • Trail Making Test Part A (TMT-A) and Part B (TMT-B) [5]
  • Letter Fluency Test [3]
The SDMT is used as a measure of cognitive processing speed and working memory [6]. It is a time-dependent test in which participants are required to write down as many symbols–number assignments as possible in a given period of 90 seconds.
The Stroop test comprises three subtests, each of which must be completed within a predetermined period of 45 seconds. The first two subtests, the SWRT and SCNT, are used to provide a measure of attention. In these tests, participants must name the colour of ink patches (SCNT) and read the names of colours printed in black ink (SWRT). The third subtest is used as a measure for response inhibition. In this test, the participants see colour words, each written in a particular colour of ink. They are required to inhibit reading of the words and instead name the colour of ink in which incongruent colour names are printed (e.g., if the word red is printed in blue ink, the correct response is "blue"). For all subtests, the total score indicates the correct number of items in 45 s.
Both Categorical and Letter Fluency are used as measures of mental flexibility and semantic knowledge. In Letter fluency [7], participants are required to produce as many words as possible, starting with a particular letter within one minute. This test consists of three sub-tests, each testing a different letter; the Categorical fluency [8, 9] resembles the Letter fluency and requires participants to reproduce as many words as possible within the animal semantic category in one minute.
The TMT [10, 11] consists of two parts with psychomotor tasks in which participants have to connect given numbers in ascending order (TMT-A) and to connect given numbers and letters in alternating order (i.e., 1-A-2-B-3-C; TMT-B). Thereby, the TMT provides measures for a wide range of psychomotor speed, executive and visual-perceptual functions [6], such as psychomotor speed (TMT-A), cognitive flexibility.

References: 
  1. Smith, A., Symbol Digits Modalities Test. 1982, Los Angeles: Western Psychological Services.
  2. Smith, A., The symbol-digit modalities test: a neuropsychologic test of learning and other cerebral disorders, in Learning disorders, J. Helmuth, Editor. 1968, Special Child Publications: Seattle. p. 83-91.
  3. Benton, A., K. Hamsher, and A. Sivan, Multilingual Aphasia Examination 3rd Edition ed. 1994, San Antonio, TX: The Psychological Corporation.
  4. Treisman, A. and S. Fearnley, The Stroop test: selective attention to colours and words. Nature, 1969. 222(5192): p. 437-9.
  5. Partington, J.E. and R.G. Leiter, Partington's Pathways Test. Psychological Service Center Journal, 1949. 1: p. 11-20.
  6. Stout, J.C., et al., Neurocognitive Signs in Prodromal Huntington Disease. Neuropsychology, 2011. 25: p. 1-14.
  7. Benton, A.L. and K. Hamsher, Multilingual aphasia examination. 1989.
  8. Benton, A.L., Neuropsychological assessment. Annual Review of Psychology, 1994. 45: p. 1-23.
  9. Morris, J.C., et al., The consortium to establish a registry for Alzheimer's disease (CERAD). Part I. Clinical and neuropsychological assessment of Alzheimer's disease. Neurology, 1989. 39: p. 1159-1165.
  10. Reitan, R.M., Validity of the Trail Making Test as an Indicator of Organic Brain Damage. Perceptual and Motor Skills, 1958. 8: p. 271-276.
  11. Kelland, DZ and R.F. Lewis, Evaluation of the reliability and validity of the Repeatable Cognitive-Perceptual-Motor Battery. Clinical Neuropsychologist, 1994. 8: p. 295-308.