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This highlights neurodevelopmental functioning as critical to the disease, but limits the neuropsychological test repertoire. We evaluated the convergent validity of the Vineland Adaptive Behavior Scales as a potential outcome measure. In a prospective observational study of 22 individuals (female:male 11:11; 6\u201320\u2009years\u2010old) with a molecular diagnosis of CLN3, we used generalized linear models and Spearman correlations to quantify the relationship of the adaptive behavior composite (ABC) standard score with established outcomes of verbal IQ (VIQ) and disease severity (Unified Batten Disease Rating Scale, UBDRS) scores. We analyzed ABC changes in 1\u2010year follow\u2010up data in a subset of the same cohort (n\u00a0=\u00a017). The ABC and VIQ, both standard scores, exhibited a strong positive correlation in cross\u2010sectional data (<jats:italic>r<\/jats:italic>\u00a0=\u00a00.81). ABC and UBDRS scores were strongly and positively correlated in cross\u2010sectional data (<jats:italic>r<\/jats:italic><jats:sub>range<\/jats:sub>\u00a0=\u00a00.87\u20130.93). Participants' ABC scores decreased slightly over the 1\u2010year follow\u2010up period (mean change, 95% CI: \u22125.23, \u22122.16). The convergent validity of the Vineland\u20103 for use in CLN3 is supported by its relationships with the established outcomes of VIQ and UBDRS. Future longitudinal research, including replication in other cohorts and evaluation of sensitivity to change, will be important to establish utility of the Vineland\u20103 for monitoring change in CLN3.<\/jats:p>","DOI":"10.1002\/ajmg.a.62607","type":"journal-article","created":{"date-parts":[[2021,12,16]],"date-time":"2021-12-16T13:15:14Z","timestamp":1639660514000},"page":"1056-1064","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":17,"title":["Use of the Vineland\u20103, a measure of adaptive functioning, in <scp>CLN3<\/scp>"],"prefix":"10.1002","volume":"188","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4171-0493","authenticated-orcid":false,"given":"An N.","family":"Dang Do","sequence":"first","affiliation":[{"name":"<i>Eunice Kennedy Shriver<\/i> National Institute of Child Health and Human Development, NIH  Bethesda Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Audrey E.","family":"Thurm","sequence":"additional","affiliation":[{"name":"National Institute of Mental Health, NIH  Bethesda Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cristan A.","family":"Farmer","sequence":"additional","affiliation":[{"name":"National Institute of Mental Health, NIH  Bethesda Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ariane G.","family":"Soldatos","sequence":"additional","affiliation":[{"name":"National Institute of Neurological Disorders and Stroke, NIH  Bethesda Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Colby E.","family":"Chlebowski","sequence":"additional","affiliation":[{"name":"National Institute of Mental Health, NIH  Bethesda Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julie K.","family":"O'Reilly","sequence":"additional","affiliation":[{"name":"National Institute of Mental Health, NIH  Bethesda Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Forbes D.","family":"Porter","sequence":"additional","affiliation":[{"name":"<i>Eunice Kennedy Shriver<\/i> National Institute of Child Health and Human Development, NIH  Bethesda Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2021,12,16]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1017\/S0012162206000570"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1177\/0883073813494813"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbadis.2012.11.014"},{"key":"e_1_2_10_5_1","unstructured":"Assessing Neurocognitive Outcomes in Inborn Errors of Metabolism. 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