Table 2

Neurocognitive characterisation, cognitive domains, neuropsychological assessments and cut-offs for cognitive dysfunction (CD) classification in SLE cohorts

First author, yearNeurocognitive characterisation (clinical NPSLE diagnosis and CD status)(Cognitive domains)
neuropsychological assessments (scoring)
CD classification cut-offs
Appenzeller, 2006Baseline: 64/107 NPSLE, 30/64—active CNS disease,
CD in 35/107 SLE (5 mild, 10 moderate, 20 severe CD)
Follow-up: 40/60—active CNS disease,
CD in 40/60 SLE (6 mild, 12 moderate, 22 severe CD)
(Attention, executive skills, memory, visual-spatial processing, language, reasoning, psychomotor speed)
CVLT, MMSE, WAIS-4 (z-scores)
CD: z-score ≤ −2SD in any domain (CD: mild<3, moderate 3–4, severe>5 domains)
Appenzeller, 2007Baseline: 36/75 NPSLE, 15/36—active CNS disease
Follow-up: NR
(Attention, executive skills, memory, visual-spatial processing, language, reasoning, psychomotor speed)
CVLT, MMSE, WAIS-4 (z-scores)
CD: z-score ≤ −2SD in any domain (CD: mild<3, moderate 3–4, severe>5 domains)
Jung, 2012
  • 16/31 NPSLE

  • NPSLE vs Non-NPSLE: ↓ psychomotor speed

(Overall cognitive function, attention, executive skills, psychomotor speed, memory)
COWAT, DS, HVLT, RCFT, TMT, WAIS-3, WRAT-3 (individual and total z-scores)
No CD cut-off
Gitelman, 2013
  • cSLE w/ CD: 6/8 NPSLE

  • cSLE w/o CD: 1/14 NPSLE

  • cSLE w/ CD vs cSLE w/o CD and HC: ↓ memory, psychomotor speed, visual-spatial processing and overall cognitive function

(General cognition, attention, executive skills, psychomotor speed, memory, visual-spatial processing)
DKEFST, CPT-II, WASI, WISC-4, WRAML-2, Woodcock-Johnson III Tests of Achievement
CD: z-scores ≤ −1 SD in at least two domains or z-scores ≤ −2SD in at least one domain
Cesar, 2015
  • NPSLE: 6/23—migraine, mood disorder and CD; 5/23—migraine and CD; 2/23—mood disorder & CD

  • NPSLE vs HC: ↓ memory

  • NPSLE vs MS: no differences in cognitive function

(Attention, executive skills, psychomotor speed, memory)
BVMT-R, CVLT, DKEFST, PASAT, SDMT (raw scores)
No CD cut-off
Bizzo, 2016
  • SLE w/o EM deficits: 14/34 NPSLE

  • SLE w/ EM deficits: 10/17 NPSLE

(Memory—learning/recall)
RAVLT (individual and mean z-scores)
CD: Mean z-scores≤−1.5 SD, or 2 or more individual z-scores≤−1.5 SD
Bódi, 2017
  • SLE: No NPSLE diagnoses

  • SLE vs HC: SLE patients showed a mild but significant CD on ACE scale

(Overall cognitive function)
MMSE, ACE (raw scores)
No CD cut-off
Zimmerman, 2017
  • SLE w/o CD: 11/20 NPSLE

  • SLE w/ CD: 11/20 NPSLE, CD (15 mild, 4 moderate, 1 severe), ↑ CD in reasoning (57%), visual-spatial processing (38%), psychomotor speed (33%), executive skills (25%), language (24%)

(Attention, executive skills, language, reasoning, memory, visual-spatial processing)
BCT, BBNAB, HT, RAVLT, SCWT, TMT, verbal fluency tasks, WAIS-3, WCST (z-scores)
CD: z-scores ≤ −2SD in any domain
Wiseman, 2017
  • SLE: 4/51 NPSLE, overall CD (MoCA—19/50, ACER—13/50, MMSE—5/50 SLE)

  • SLE vs HC: ↓ premorbid intelligence (NART)

(Overall cognitive function)
MoCA, ACER, MMSE, NART (raw scores)
No CD cut-off
Cannerfelt, 2018
  • SLE: 43/70 NPSLE (38/43—migraine, 26/43—CD, 25/43—depression, 19/43—anxiety, 18/43—neuropathy)

(Memory, executive skills, psychomotor speed, attention, reasoning)
CNS-VS
No CD cut-off for group classification
Corrêa, 2018
  • SLE: NPSLE diagnosis—NR, no group differences in any domain except memory

(Overall cognitive function, attention, executive skills, psychomotor speed, memory, visual-spatial processing)
MMSE, RAVLT, BBNAB, WAIS-3, TMT, HT, SCWT (z-scores)
CD: RAVLT (mem)—total z-scores≤−1.5 SD, or z-scores≤−1.5 SD in≥2 RAVLT domains
Nystedt, 2018
  • SLE: 39/54 NPSLE

  • NPSLE vs non-NPSLE: ↓ psychomotor speed, attention, cognitive flexibility (executive skills)

  • NPSLE vs HC: ↓ all cognitive domains

(memory, executive skills, psychomotor speed, attention, reasoning)
CNS-VS
No CD cut-off
Wiseman, 2018
  • SLE: 3/47 active NPSLE, NART correlated w/ ↑ g (overall cognitive function)

(Overall cognitive function)
MoCA, ACER, MMSE, NART (raw scores)
No CD cut-off
MacKay, 2019
  • SLE-2 (Baseline): No NPSLE diagnoses, match-to-sample, matching grids, CPT scores ≤ −2SD in 3/20 (15%), 8/20 (40%), 1/20 (5%), respectively

  • SLE-2 (follow-up): ↑ Match-to-Sample & Matching Grids scores (still remained ≤ −2SD for same patients)

  • SLE-2 vs HC: ↓ scores on all ANAM tests

(Attention, memory, visual-spatial processing)
Matching Grids, Match-to-Sample, CPT (ANAM battery throughput scores—combined RT & accuracy)
No CD cut-off
DiFrancesco, 2020
  • cSLE: No NPSLE or CD diagnoses

(Attention, psychomotor speed, memory, visual-spatial processing)
CPT-II, KABC, SEBC, WASI, WISC-4 (z-scores)
CD: z-scores ≤ −2SD in one domain, or z-scores ≤ −1SD in two domains
Mårtensson, 2021
  • SLE: 41/69 NPSLE

(memory, executive skills, psychomotor speed, attention, reasoning)
CNS-VS
No CD cut-off
Qian, 2022
  • SLE: NPSLE diagnosis—NR

  • SLE vs HC: ↓ mean IQ, ↓ CPT

(Attention, memory, visual-spatial processing)
WAIS-4, ANAM battery (throughput scores)
No CD cut-off
Julio, 2023
  • cSLE: 53/71 NPSLE, 31/71 CD

  • aSLE: 36/49 NPSLE, 16/49 CD

(Overall cognitive function)
MoCA
No CD cut-off
  • ACE, Addenbrooke’s Cognitive Examination; ACER, ACE-Revised; ANAM, Automated Neuropsychological Assessment Metric; BBNAB, Brazilian Brief Neuropsychological Assessment Battery NEUPSILIN; BCT, Bells Cancellation Test; BVMT, Brief Visuospatial Memory Test–Revised; CARRA, Childhood Arthritis & Rheumatology Research Alliance; CNS-VS, Central Nervous System Vital-Signs; COWAT, Controlled Oral Word Association Test; CPT, Continuous Performance Test; cSLE, childhood-onset SLE; CVLT, California Verbal Learning Test; DKEFST, Delis-Kaplan Executive Function System Test; DS, digit symbol; HC, healthy controls; HT, Hayling Test; HVLT, Hopkins Verbal Learning Test; IQ, intelligence quotient; KABC, Kaufman Assessment Battery for Children; MMSE, Mini Mental State Examination; MoCA, Montreal Cognitive Assessment; NART, National Adult Reading Test; NPSLE, neuropsychiatric SLE; NR, not reported; PASAT, Paced Auditory Serial Addition Test; RAVLT, Rey Auditory Verbal Learning Test; RCFT, Rey Complex Figure Test; RT, reaction time; SCWT, Stroop Color-Word Test; SDMT, Symbol Digit Modalities Test; SEBC, Second Edition Block Counting; TMT, Trail Making Test; WAIS-3, Wechsler Adult Intelligence-III; WAIS-4, Wechsler Adult Intelligence Scale–Revised; WASI, Wechsler Abbreviated Scale of Intelligence; WCS, Wisconsin Card Sort; WISC-4, Wechsler Intelligence Scale for Children-IV; WRAML-2, Wide Range Assessment of Memory and Learning 2; WRAT-3, Wide Range Achievement Test–III.