Neuropsychiatric Symptoms of Alzheimer's Disease in Down Syndrome and Its Impact on Caregiver Distress

Luciana Mascarenhas Fonseca; Guilherme Prado Mattar; Glenda Guerra Haddad; Ekaterina Burduli; Sterling M McPherson; Laura Maria de Figueiredo Ferreira Guilhoto; Mônica Sanches Yassuda; Geraldo Filho Busatto; Cassio Machado de Campos Bottino; Marcelo Queiroz Hoexter; Naomi Sage Chaytor

doi:10.3233/JAD-201009

Neuropsychiatric Symptoms of Alzheimer's Disease in Down Syndrome and Its Impact on Caregiver Distress

J Alzheimers Dis. 2021;81(1):137-154. doi: 10.3233/JAD-201009.

Affiliations

¹ Department of Medical Education and Clinical Science, Washington State University, Spokane, WA, USA.
² Programa Terceira Idade PROTER, Old Age Research Group, Department and Institute of Psychiatry, University of São Paulo School of Medicine, São Paulo, Brazil.
³ College of Nursing, Washington State University, Spokane, WA, USA.
⁴ Institute Jo Clemente, São Paulo, Brazil.
⁵ School of Arts, Sciences and Humanities, University of São Paulo, Brazil.
⁶ Laboratorio de Neuroimagem em Psiquiatria (LIM21, Laboratory of Psychiatric Neuroimaging), Department and Institute of Psychiatry, University of São Paulo School of Medicine, São Paulo, Brazil.
⁷ Projeto Transtornos do Espectro Obsessivo-Compulsivo PROTOC, Obsessive-Compulsive Spectrum Disorders Program, Department and Institute of Psychiatry, University of São Paulo School of Medicine, São Paulo, Brazil.

Abstract

Background: Neuropsychiatric symptoms (NPS) are non-cognitive manifestations common to dementia and other medical conditions, with important consequences for the patient, caregivers, and society. Studies investigating NPS in individuals with Down syndrome (DS) and dementia are scarce.

Objective: Characterize NPS and caregiver distress among adults with DS using the Neuropsychiatric Inventory (NPI).

Methods: We evaluated 92 individuals with DS (≥30 years of age), divided by clinical diagnosis: stable cognition, prodromal dementia, and AD. Diagnosis was determined by a psychiatrist using the Cambridge Examination for Mental Disorders of Older People with Down's Syndrome and Others with Intellectual Disabilities (CAMDEX-DS). NPS and caregiver distress were evaluated by an independent psychiatrist using the NPI, and participants underwent a neuropsychological assessment with Cambridge Cognitive Examination (CAMCOG-DS).

Results: Symptom severity differed between-groups for delusion, agitation, apathy, aberrant motor behavior, nighttime behavior disturbance, and total NPI scores, with NPS total score being found to be a predictor of AD in comparison to stable cognition (OR for one-point increase in the NPI = 1.342, p = 0.012). Agitation, apathy, nighttime behavior disturbances, and total NPI were associated with CAMCOG-DS, and 62% of caregivers of individuals with AD reported severe distress related to NPS. Caregiver distress was most impacted by symptoms of apathy followed by nighttime behavior, appetite/eating abnormalities, anxiety, irritability, disinhibition, and depression (R2 = 0.627, F(15,76) = 8.510, p < 0.001).

Conclusion: NPS are frequent and severe in individuals with DS and AD, contributing to caregiver distress. NPS in DS must be considered of critical relevance demanding management and treatment. Further studies are warranted to understand the biological underpinnings of such symptoms.

Keywords: Alzheimer’s disease; Down syndrome; Neuropsychiatric Inventory; dementia; intellectual disability; neuropsychiatric symptoms.

Publication types

Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't

MeSH terms

Adult
Alzheimer Disease / diagnosis*
Alzheimer Disease / etiology
Alzheimer Disease / psychology
Caregivers / psychology*
Down Syndrome / complications*
Down Syndrome / psychology
Female
Humans
Male
Middle Aged
Neuropsychological Tests
Psychological Distress
Severity of Illness Index
Symptom Assessment