The Behavioral and Psychological Symptoms of Dementia in Down Syndrome (BPSD-DS) Scale: Comprehensive Assessment of Psychopathology in Down Syndrome

doi:10.3233/JAD-170920

Multicenter Study

. 2018;63(2):797-819.

doi: 10.3233/JAD-170920.

The Behavioral and Psychological Symptoms of Dementia in Down Syndrome (BPSD-DS) Scale: Comprehensive Assessment of Psychopathology in Down Syndrome

Alain D Dekker^{1

2}, Silvia Sacco³, Angelo Carfi⁴, Bessy Benejam⁵, Yannick Vermeiren^{1

2}, Gonny Beugelsdijk⁶, Mieke Schippers⁶, Lyanne Hassefras⁶, José Eleveld⁷, Sharina Grefelman⁷, Roelie Fopma⁸, Monique Bomer-Veenboer⁹, Mariángeles Boti⁵, G Danielle E Oosterling¹⁰, Esther Scholten¹¹, Marleen Tollenaere^{2

12}, Laura Checkley¹³, André Strydom¹³, Gert Van Goethem^{14

15}, Graziano Onder⁴, Rafael Blesa¹⁶, Christine Zu Eulenburg¹⁷, Antonia M W Coppus^{18

19}, Anne-Sophie Rebillat³, Juan Fortea^{5

16}, Peter P De Deyn^{1

2

12}

Affiliations

¹ Department of Neurology and Alzheimer Research Center, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands.
² Laboratory of Neurochemistry and Behaviour, Institute Born-Bunge, University of Antwerp, Wilrijk, Belgium.
³ Institut Jérôme Lejeune, Paris, France.
⁴ Department of Geriatrics, Policlinico Gemelli, Universitá Cattolica del Sacro Cuore, Rome, Italy.
⁵ Down Medical Center, Catalan Down Syndrome Foundation, Barcelona, Spain.
⁶ Ipse de Bruggen, Center for Intellectual Disabilities, Nieuwveen/Zwammerdam, The Netherlands.
⁷ Cosis, Center for Intellectual Disabilities, Groningen, The Netherlands.
⁸ Talant, Center for Intellectual Disabilities, Heerenveen, The Netherlands.
⁹ Pameijer, Center for Intellectual Disabilities, Rotterdam, The Netherlands.
¹⁰ Aveleijn, Center for Intellectual Disabilities, Borne, The Netherlands.
¹¹ Elver, Center for Intellectual Disabilities, Nieuw-Wehl, The Netherlands.
¹² Department of Neurology and Memory Clinic, Hospital Network Antwerp (ZNA) Middelheim and Hoge Beuken, Antwerp, Belgium.
¹³ Division of Psychiatry, University College London, London, UK.
¹⁴ Het GielsBos, Center for Intellectual Disabilities, Gierle, Belgium.
¹⁵ Department of Neurology, University Hospital Antwerp, Antwerp, Belgium.
¹⁶ Department of Neurology, Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau, Universitat Autònoma de Barcelona, Barcelona, Spain.
¹⁷ Department of Epidemiology, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands.
¹⁸ Dichterbij, Center for Intellectual Disabilities, Gennep, The Netherlands.
¹⁹ Department of Primary and Community Care, Radboud University Medical Center, Nijmegen, The Netherlands.

PMID: 29689719
PMCID: PMC5929348
DOI: 10.3233/JAD-170920

Multicenter Study

The Behavioral and Psychological Symptoms of Dementia in Down Syndrome (BPSD-DS) Scale: Comprehensive Assessment of Psychopathology in Down Syndrome

Alain D Dekker et al. J Alzheimers Dis. 2018.

. 2018;63(2):797-819.

doi: 10.3233/JAD-170920.

Authors

Affiliations

¹ Department of Neurology and Alzheimer Research Center, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands.
² Laboratory of Neurochemistry and Behaviour, Institute Born-Bunge, University of Antwerp, Wilrijk, Belgium.
³ Institut Jérôme Lejeune, Paris, France.
⁴ Department of Geriatrics, Policlinico Gemelli, Universitá Cattolica del Sacro Cuore, Rome, Italy.
⁵ Down Medical Center, Catalan Down Syndrome Foundation, Barcelona, Spain.
⁶ Ipse de Bruggen, Center for Intellectual Disabilities, Nieuwveen/Zwammerdam, The Netherlands.
⁷ Cosis, Center for Intellectual Disabilities, Groningen, The Netherlands.
⁸ Talant, Center for Intellectual Disabilities, Heerenveen, The Netherlands.
⁹ Pameijer, Center for Intellectual Disabilities, Rotterdam, The Netherlands.
¹⁰ Aveleijn, Center for Intellectual Disabilities, Borne, The Netherlands.
¹¹ Elver, Center for Intellectual Disabilities, Nieuw-Wehl, The Netherlands.
¹² Department of Neurology and Memory Clinic, Hospital Network Antwerp (ZNA) Middelheim and Hoge Beuken, Antwerp, Belgium.
¹³ Division of Psychiatry, University College London, London, UK.
¹⁴ Het GielsBos, Center for Intellectual Disabilities, Gierle, Belgium.
¹⁵ Department of Neurology, University Hospital Antwerp, Antwerp, Belgium.
¹⁶ Department of Neurology, Hospital de la Santa Creu i Sant Pau, Biomedical Research Institute Sant Pau, Universitat Autònoma de Barcelona, Barcelona, Spain.
¹⁷ Department of Epidemiology, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands.
¹⁸ Dichterbij, Center for Intellectual Disabilities, Gennep, The Netherlands.
¹⁹ Department of Primary and Community Care, Radboud University Medical Center, Nijmegen, The Netherlands.

PMID: 29689719
PMCID: PMC5929348
DOI: 10.3233/JAD-170920

Abstract

People with Down syndrome (DS) are prone to develop Alzheimer's disease (AD). Behavioral and psychological symptoms of dementia (BPSD) are core features, but have not been comprehensively evaluated in DS. In a European multidisciplinary study, the novel Behavioral and Psychological Symptoms of Dementia in Down Syndrome (BPSD-DS) scale was developed to identify frequency and severity of behavioral changes taking account of life-long characteristic behavior. 83 behavioral items in 12 clinically defined sections were evaluated. The central aim was to identify items that change in relation to the dementia status, and thus may differentiate between diagnostic groups. Structured interviews were conducted with informants of persons with DS without dementia (DS, n = 149), with questionable dementia (DS+Q, n = 65), and with diagnosed dementia (DS+AD, n = 67). First exploratory data suggest promising interrater, test-retest, and internal consistency reliability measures. Concerning item relevance, group comparisons revealed pronounced increases in frequency and severity in items of anxiety, sleep disturbances, agitation & stereotypical behavior, aggression, apathy, depressive symptoms, and eating/drinking behavior. The proportion of individuals presenting an increase was highest in DS+AD, intermediate in DS+Q, and lowest in DS. Interestingly, among DS+Q individuals, a substantial proportion already presented increased anxiety, sleep disturbances, apathy, and depressive symptoms, suggesting that these changes occur early in the course of AD. Future efforts should optimize the scale based on current results and clinical experiences, and further study applicability, reliability, and validity. Future application of the scale in daily care may aid caregivers to understand changes, and contribute to timely interventions and adaptation of caregiving.

Keywords: Alzheimer’s disease; BPSD; Down syndrome; behavior; dementia; neuropsychiatric symptoms; trisomy 21.

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Figures

**Fig.1**
Schematic overview of included and excluded BPSD-DS informant interviews, subdivided in the three diagnostic groups. BPSD-DS, Behavioral and Psychological Symptoms of Dementia in Down Syndrome scale; CVA, cerebrovascular accident.

**Fig.2**
Significant frequency changes and severity changes for items in section 1 (anxiety & nervousness). The proportion of individuals (%) showing decreased, unaltered or increased scores is depicted per study group. Specifically, the black sections and corresponding percentage (top), the pale grey sections and corresponding percentage (middle) and the grey sections and corresponding percentage (bottom) respectively indicate the proportion of each study group showing a decreased, unaltered or increased frequency/severity. Statistics (Kruskal-Wallis group comparisons) and further item descriptions, including items that did not significantly differ between groups, are provided in the text. DS, Down syndrome without signs of dementia; DS+Q, Down syndrome with questionable dementia; DS+AD, Down syndrome with diagnosed AD.

**Fig.3**
Significant frequency changes and severity changes for items in sections 2 (sleep disturbances) and 3 (irritability). The proportion of individuals showing decreased, unaltered or increased scores is depicted per study group. Specifically, the black sections and corresponding percentage (top), the pale grey sections and corresponding percentage (middle) and the grey sections and corresponding percentage (bottom) respectively indicate the proportion of each study group showing a decreased, unaltered or increased frequency/severity. Statistics (Kruskal-Wallis group comparisons) and further item descriptions, including items that did not significantly differ between groups, are provided in the text. DS, Down syndrome without signs of dementia; DS+Q, Down syndrome with questionable dementia; DS+AD, Down syndrome with diagnosed AD.

**Fig.4**
Significant frequency changes and severity changes for items in sections 4 (obstinacy), 5 (agitation & stereotypical behavior) and 6 (aggression). The proportion of individuals showing decreased, unaltered or increased scores is depicted per study group. Specifically, the black sections and corresponding percentage (top), the pale grey sections and corresponding percentage (middle) and the grey sections and corresponding percentage (bottom) respectively indicate the proportion of each study group showing a decreased, unaltered or increased frequency/severity. Statistics (Kruskal-Wallis group comparisons) and further item descriptions, including items that did not significantly differ between groups, are provided in the text. DS, Down syndrome without signs of dementia; DS+Q, Down syndrome with questionable dementia; DS+AD, Down syndrome with diagnosed AD.

**Fig.5**
Significant frequency changes and severity changes for items in section 7 (apathy & aspontaneity). The proportion of individuals showing decreased, unaltered or increased scores is depicted per study group. Specifically, the black sections and corresponding percentage (top), the pale grey sections and corresponding percentage (middle) and the grey sections and corresponding percentage (bottom) respectively indicate the proportion of each study group showing a decreased, unaltered or increased frequency/severity. Statistics (Kruskal-Wallis group comparisons) and further item descriptions, including items that did not significantly differ between groups, are provided in the text. DS, Down syndrome without signs of dementia; DS+Q, Down syndrome with questionable dementia; DS+AD, Down syndrome with diagnosed AD.

**Fig.6**
Significant frequency changes and severity changes for items in section 8 (depressive symptoms). The proportion of individuals showing decreased, unaltered or increased scores is depicted per study group. Specifically, the black sections and corresponding percentage (top), the pale grey sections and corresponding percentage (middle) and the grey sections and corresponding percentage (bottom) respectively indicate the proportion of each study group showing a decreased, unaltered or increased frequency/severity. Statistics (Kruskal-Wallis group comparisons) and further item descriptions, including items that did not significantly differ between groups, are provided in the text. DS, Down syndrome without signs of dementia; DS+Q, Down syndrome with questionable dementia; DS+AD, Down syndrome with diagnosed AD.

**Fig.7**
Significant frequency changes and severity changes for items in sections 9 (delusions), 10 (hallucinations) and 11 (disinhibition & sexual behavior). The proportion of individuals showing decreased, unaltered or increased scores is depicted per study group. Specifically, the black sections and corresponding percentage (top), the pale grey sections and corresponding percentage (middle) and the grey sections and corresponding percentage (bottom) respectively indicate the proportion of each study group showing a decreased, unaltered or increased frequency/severity. Statistics (Kruskal-Wallis group comparisons) and further item descriptions, including items that did not significantly differ between groups, are provided in the text. DS, Down syndrome without signs of dementia; DS+Q, Down syndrome with questionable dementia; DS+AD, Down syndrome with diagnosed AD.

**Fig.8**
Significant frequency changes and severity changes for items in section 12 (eating/drinking behavior). The proportion of individuals showing decreased, unaltered or increased scores is depicted per study group. Specifically, the black sections and corresponding percentage (top), the pale grey sections and corresponding percentage (middle) and the grey sections and corresponding percentage (bottom) respectively indicate the proportion of each study group showing a decreased, unaltered or increased frequency/severity. Statistics (Kruskal-Wallis group comparisons) and further item descriptions, including items that did not significantly differ between groups, are provided in the text. DS, Down syndrome without signs of dementia; DS+Q, Down syndrome with questionable dementia; DS+AD, Down syndrome with diagnosed AD.

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References

1. Ballard C, Mobley WC, Hardy J, Williams G, Corbett A (2016) Dementia in Down’s syndrome. Lancet Neurol 15, 622–636. - PubMed
1. Alzheimer’s Assocation (2016) 2016 Alzheimer’s disease facts and figures. Alzheimers Dement 12, 459–509. - PubMed
1. Wiseman FK, Al-Janabi T, Hardy J, Karmiloff-Smith A, Nizetic D, Tybulewicz VLJ, Fisher EMC, Strydom A (2015) A genetic cause of Alzheimer disease: Mechanistic insights from Down syndrome. Nat Rev Neurosci 16, 564–574. - PMC - PubMed
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[1] Ballard C, Mobley WC, Hardy J, Williams G, Corbett A (2016) Dementia in Down’s syndrome. Lancet Neurol 15, 622–636. - PubMed

[2] Ballard C, Mobley WC, Hardy J, Williams G, Corbett A (2016) Dementia in Down’s syndrome. Lancet Neurol 15, 622–636. - PubMed

[3] Alzheimer’s Assocation (2016) 2016 Alzheimer’s disease facts and figures. Alzheimers Dement 12, 459–509. - PubMed

[4] Alzheimer’s Assocation (2016) 2016 Alzheimer’s disease facts and figures. Alzheimers Dement 12, 459–509. - PubMed

[5] Wiseman FK, Al-Janabi T, Hardy J, Karmiloff-Smith A, Nizetic D, Tybulewicz VLJ, Fisher EMC, Strydom A (2015) A genetic cause of Alzheimer disease: Mechanistic insights from Down syndrome. Nat Rev Neurosci 16, 564–574. - PMC - PubMed

[6] Wiseman FK, Al-Janabi T, Hardy J, Karmiloff-Smith A, Nizetic D, Tybulewicz VLJ, Fisher EMC, Strydom A (2015) A genetic cause of Alzheimer disease: Mechanistic insights from Down syndrome. Nat Rev Neurosci 16, 564–574. - PMC - PubMed

[7] Mann DMA (1988) Alzheimer’s disease and Down’s syndrome. Histopathology 13, 125–137. - PubMed

[8] Mann DMA (1988) Alzheimer’s disease and Down’s syndrome. Histopathology 13, 125–137. - PubMed

[9] Zigman WB, Lott IT (2007) Alzheimer’s disease in Down syndrome: Neurobiology and risk. Ment Retard Dev Disabil Res Rev 13, 237–246. - PubMed

[10] Zigman WB, Lott IT (2007) Alzheimer’s disease in Down syndrome: Neurobiology and risk. Ment Retard Dev Disabil Res Rev 13, 237–246. - PubMed

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The Behavioral and Psychological Symptoms of Dementia in Down Syndrome (BPSD-DS) Scale: Comprehensive Assessment of Psychopathology in Down Syndrome

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The Behavioral and Psychological Symptoms of Dementia in Down Syndrome (BPSD-DS) Scale: Comprehensive Assessment of Psychopathology in Down Syndrome

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