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Table 1 Classification and evaluation of participants

From: Endophenotypical drift in Huntington’s disease: a 5-year follow-up study

Clinical evaluation

UHDRS total motor score (TMS)

Pre-manifest: TMS ≤ 5

Manifest: TMS > 5

Neuropsychiatric evaluation

Allocation to neuropsychiatric group if at least one criterion is met

1. Usage of psychotropic medication on neuropsychiatric indication

2. A SCL-90-R GSI T-score ≥ 63 or a T-score ≥ 63 in more than two of the nine primary symptom dimensions. The SCL-90-R cut-offs are based on SCL-90-R guide

3. A HAM-17 score ≥ 13 (moderate to severe depression)

Cognitive evaluation

Cognitively impaired if

Pre-morbid intellectual level:

Wechsler Adult Intelligence Scale (WAIS)

Danish adult reading test (DART)

A. four or more test performances were impaired

Memory:

Selective reminding test

Rey complex figure text

B. all tests in a domain (except psychomotor speed/attention) were impaired

Psychomotor speed/attention:

Trail making test A & B

SDMT

Executive functions:

Stroop test (100 items − incongruent))

Verbal fluency tests (category fluency (animals, 1 min), lexical fluency (s-words and a-words, 1 min))

C. performances on all tests in the psychomotor speed/attention domain, and at least one other test was below cut-off

Visiospatial functions:

Rey complex figure

Ravens progressive matrices (set 1)

Block design test (modified version)

  1. Classification of participants into groups of premanifest and manifest HD gene expansion carriers and evaluation of neuropsychiatric symptoms and cognitive impairment
  2. SCL-90-R Symptom Checklist-90-Revised, HAM-17 Hamilton Rating Scale for Depression-17, SDMT Symbol digit modality test