Interventions Targeting Reversible Dementia in Down Syndrome

A 49-year-old man with high-functioning Down syndrome was admitted to the Behavioural Neurology Unit, Baycrest Centre for Geriatric Care (Toronto, Ontario, Canada), with a 1-year history of cognitive and behavioral decline. Behavioral problems included physical and verbal aggression (kicking, hitting, swearing, screaming), voiding and defecating on the floor, and refusing medication and assistance with hygiene. Hoarding (toilet paper, incontinence briefs) and disrobing were long-standing but had worsened. Although Alzheimer's disease was strongly suspected, he made a remarkable recovery to his premorbid level after a combination of pharmacological and behavioral interventions. Medical history included left eye blindness, hepatitis B, liver cirrhosis, hemochromatosis, irritable bowel syndrome, diverticulitis, hypothyroidism, and hypotension. Psychiatric history was significant for depression and obsessive-compulsive disorder. Signs of sensory defensiveness1 were observed (hypersensitivity to nonnoxious stimuli). Admission medications included zopiclone and mirtazapine. Sertraline was tried but stopped. Zopiclone and mirtazapine were discontinued. Midodrine was prescribed for hypotension. Olanzapine was added to treat his behavioral symptoms. He was noted to be socially withdrawn and unmotivated. His Hospital Anxiety and Depression Scale2 score indicated depression and anxiety. Duloxetine was started to treat depression and anxiety. Behavioral interventions were implemented in conjunction with medication. Reduction of physical and verbal aggression was targeted through differential reinforcement of other behaviors,3 such as pleasant interactions, using verbal praise. Aggressive behaviors were ignored, and staff withdrew from the room when they occurred. A behavior-specific positive reinforcement schedule was implemented within a firm, consistent routine to encourage adherence to care. For example, telephone calls were offered daily after he showered, changed socks, and took medication. Alternative strategies were explored to target inappropriate voiding and defecation, which continued despite this reinforcement schedule. After staff encouraged urination and defecation on an absorbent mat on the bathroom floor, he began adhering. To reduce hoarding, staff provided toilet paper when one-third of the previous roll remained. One incontinence brief was provided every morning to reduce anxiety related to a remote episode of stool incontinence. Sensory defensiveness in multiple sensory modalities was thought to contribute to his behavioral and psychosocial problems, especially in relation to tactile sense (disrobing, refusal to remove socks). To treat sensory defensiveness, a program of deep pressure touch and joint compressions to specific body parts1 was initiated. Food was offered as positive reinforcement after adherence. Only joint compressions were tolerated for the first 2 weeks; for the following 3 weeks, deep pressure touch and joint compressions were administered. This protocol was phased out gradually and discontinued. He was also expected to dress when leaving his room. Verbal reminders were provided, and praise was given for adherence. Nudity in his private room was tolerated. Participation in meaningful leisure activities was encouraged throughout treatment. Frequency of behaviors was documented every shift using an adapted version of the Cohen-Mansfield Agitation Inventory.4 Upon admission and at discharge, neuropsychiatric symptoms and tactile sensory defensiveness were assessed using the Hospital Anxiety and Depression Scale and the Sensory Defensiveness Screening Tool.5 All problem behaviors were significantly reduced 16 weeks after admission, and he was discharged to an assisted living group home. When he was seen as an outpatient 8 weeks later, frequency of most target behaviors and symptoms of tactile sensory defensiveness, anxiety, and depression remained improved, although swearing had occurred several times in the previous 2 weeks in response to a change in routine (Figure 1). Adults with Down syndrome are at risk of developing behavioral, emotional, and psychiatric problems.6 These may be early signs of Alzheimer's disease, because behavioral and psychological symptoms may appear before cognitive decline in these individuals,7 but behavioral and cognitive changes may also be due to depression, anxiety, or other treatable causes.8 Thus, individuals with Down syndrome who present with dementia-like symptoms require thorough assessment and treatment for reversible causes of decline, regardless of how impaired they may seem, before being diagnosed with Alzheimer's disease or other dementia. The aging population worldwide includes an increasing number of individuals with Down syndrome9 who are likely to develop behavioral and psychological symptoms related to Alzheimer's disease, yet information regarding interventions targeting these symptoms in Down syndrome is surprisingly scarce. The present report is an important addition to this sparse literature and provides examples of treatment strategies that may be effective, although because multiple behavioral interventions were implemented simultaneously, in addition to combination pharmacological therapy, the independent contribution of each cannot be ascertained. Additional studies should examine these interventions separately to determine their individual effectiveness in individuals with Down syndrome who have progressive behavioral and cognitive decline. We are grateful to the patient and his family and to the staff of the Baycrest Behavioural Neurology Unit. Conflict of Interest: The authors report no conflict of interest. Dr. Freedman receives support from the Saul A. Silverman Family Foundation as a Canada International Scientific Exchange Program and the Morris Kerzner Memorial Fund. Author Contributions: Callahan, Wong: concept and design, acquisition and interpretation of data, drafting the article, final approval for publication. Ngang: acquisition and interpretation of data, drafting the article, final approval for publication. Wolf: acquisition and interpretation of data, drafting the article, final edits, approval for publication. Freedman: concept and design, acquisition and interpretation of data, final edits, approval for publication. Sponsor's Role: This study was not funded by a sponsoring organization.

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