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. 2022 Jul;198(2):382-390.
doi: 10.1111/bjh.18188. Epub 2022 Apr 6.

The Montreal cognitive assessment as a cognitive screening tool in sickle cell disease: Associations with clinically significant cognitive domains

Macy L Early  1 , Elizabeth Linton  2 , Allison Bosch  3 , Timothy Campbell  4 , Felicia Hill-Briggs  5 , Lydia H Pecker  1 , Eboni I Lance  6   7 , Sophie Lanzkron  1
Affiliations

The Montreal cognitive assessment as a cognitive screening tool in sickle cell disease: Associations with clinically significant cognitive domains

Macy L Early et al. Br J Haematol. 2022 Jul.

Abstract

Adults with sickle cell disease (SCD) are at risk for cognitive impairment, which causes significant morbidity. Guidelines support routine cognitive screening, but no screening test is validated in this population. We explored the Montreal Cognitive Assessment (MoCA) as a possible screening test in SCD. We administered the MoCA; a literacy test, the Wide Range Achievement Test, fourth edition (WRAT-4); and a health literacy test, the Shortened Test of Functional Health Literacy in Adults (S-TOFHLA) to adults with SCD and gathered clinical variables through chart review. Spearman's rho, Mann-Whitney, and Kruskal-Wallis tests and quantile regression models were used. Among our sample of 49 adults with SCD, the median MoCA score was 25.0 [interquartile range (IQR) 22.0-28.0]. Higher educational attainment was associated with MoCA scores (p = 0.001). In multivariable models, MoCA scores were associated with S-TOFHLA (p = 0.001) and WRAT-4 Reading (p = 0.002) scores, and overt stroke (p = 0.03) at the median. This pilot study adds to the limited literature of cognitive screening tests in adults with SCD and demonstrates a relationship between MoCA scores and measures of literacy and health literacy. The MoCA is a promising option for briefly screening for cognitive impairment in adults with SCD, though further study is needed to confirm its validity.

Keywords: Montreal Cognitive Assessment; cognitive performance; cognitive screening; health literacy; sickle cell disease.

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Conflict of interest statement

Sophie Lanzkron receives funding from Global Blood Therapeutics, Imara, Shire, Novartis, and the NIH; is a consultant for Bluebird Bio and Novo Nordisk; and holds stock in Pfizer and Teva. The other authors have nothing to disclose.

Figures

FIGURE 1
FIGURE 1
Stroke, other ischaemic neurologic event, and baseline haemoglobin are associated with some cognitive test scores in quantile models at the 25th percentile and median when controlling for educational attainment and age. (A) Effect of clinical variables on Montreal Cognitive Assessment (MoCA) scores; (B) Effect of clinical variables on the Shortened Test of Functional Health Literacy in Adults (S‐TOFHLA) scores; (C) Effect of clinical variables on Wide Range Achievement Test, fourth edition (WRAT‐4): Reading standardized scores; (D) Effect of clinical variables on WRAT‐4: Sentence Comprehension standardized scores. Neurologic events include overt stroke (four patients), hypoxic ischaemic encephalopathy (one patient), and posterior reversible encephalopathy syndrome (one patient). CI, confidence interval
FIGURE 2
FIGURE 2
Scores on the Montreal Cognitive Assessment (MoCA), which measures general cognitive performance, is related to scores on the (A) Shortened Test of Functional Health Literacy in Adults (S‐TOFHLA), which measures health literacy, and (B/C) Wide Range Achievement Test, fourth edition (WRAT‐4): Reading and Sentence Comprehension subtests, which measure literacy. CI, confidence interval
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