Unlocking Behaviors: Cognitive Changes
by Risley “Ley” Linder, MA, MED, BCBA and Craig Escudé, MD, FAAFP, FAADM
Jamie is a 48-year-old dynamic, energetic, fun-loving person who happens to have Down syndrome. She lives in a small community-based group home with two other people. She’s known as the jokester in the group and always has a way of making her friends laugh. She is an impeccable dresser and fancies cool hairstyles, which she highlights with fashionable hair accessories.
Jamie experienced a pretty big traumatic event in her life when she lost her mother two years ago. It had quite an impact on her. She became a bit withdrawn. She was less interested in many of her usual activities, and as her friend said, “She just wasn’t acting like Jamie anymore.” Her friends noted that she was less concerned about what she wore, sometimes wearing the same clothing two days in a row. Her hairstyle became much more straightforward and even appeared a bit messy at times. Her usual witty jokes seemed more challenging to come by for her, and often, she took long pauses before answering questions.
“Over time, Jamie seemed to need more assistance with usual activities such as preparing meals, and later, she even seemed to have difficulties feeding herself. She occasionally chose a short-sleeved shirt to wear when it was 30 degrees outside, and more than once, she’d put her shirt on backward.”
Behavioral Discussion
In the behavioral realm, one of the most challenging things is delineating between psychiatric symptoms (e.g., Jamie’s possible depression symptoms), dementia symptoms, and normal aging in a person with intellectual disabilities. Over time, I have developed an approach of “prove to me it is dementia.” I have adopted this approach because, far too often, dementia is a diagnosis that interdisciplinary teams, including physicians and other medical professionals make too quickly and easily. There are many reasons a person can exhibit changes in cognitive status, mood, affect, or preferences. As behavior analysts, it’s critically important for us to keep an eye on all the possible reasons a person could be exhibiting changes in their behavior as we work to delineate changing behavioral presentations as a person ages.
One of my most commonly used tools for making this delineation is a dementia screening tool developed specifically for people with intellectual disabilities: The National Task Group on Intellectual Disabilities and Dementia Practices Early Detection Screen for Dementia (NTG-EDSD). The NTG-EDSD can be used for the early detection screening of those adults with an intellectual disability who are suspected of or may be showing early signs of mild cognitive impairment or dementia. The NTG-EDSD is not an assessment or diagnostic instrument, but an administrative screen staff and family caregivers can use to note functional decline and health problems and record valuable information for further assessment (NTG, 2013).
“Jamie would likely benefit from the completion of a dementia screening tool, such as the NTG-EDSD, which is recommended on an annual basis for individuals with Down syndrome at the age of 40.”
This would allow her staff, with a distal and working knowledge of Jamie, to provide insight into the specific areas of her life in which changes have occurred, such as language and communication, activities of daily living, behavior and affect, and potential medical issues, to name a few. These areas can help guide the interdisciplinary team’s decision-making as they work to “prove that it is dementia.”
Medical Discussion
Jamie has many classic signs of depression and had a good reason for being depressed with the loss of her mother. However, because Jamie has Down syndrome, she is also at a much greater risk for Alzheimer’s dementia, and the signs and symptoms can overlap. People with Down syndrome have an extra copy of chromosome 21, which also carries a gene responsible for producing a protein called amyloid precursor protein. Too much buildup of this protein in the brain leads to beta-amyloid plaques, which are implicated as a major cause of Alzheimer’s disease. It is estimated that 50% or more of people with Down Syndrome will develop Alzheimer’s, and they are more likely to develop it at an earlier age than those without Down Syndrome.
Ruling out other causes for symptoms that are consistent with dementia is also very important. Mental health conditions, brain tumors, vitamin deficiencies, medication side effects, and thyroid, kidney, and liver disease can all present with signs resembling dementia.
There is new and promising research in Alzheimer’s disease, including blood tests that can help diagnose Alzheimer’s dementia. While these tests are currently not available everywhere, soon, we should have widespread availability of blood testing to diagnose the condition. There have also been advances in medication treatment options for dementia, and current trials are promising for the availability of effective treatment options in the near future. Learn more about Alzheimer’s and other dementias at https://www.alzheimers.gov/.
Outcome
Jamie was initially evaluated by a neuropsychiatrist and was diagnosed initially with depression with secondary concern for Alzheimer’s. Medication treatment for depression did improve some of her symptoms, but after testing, talking with her support team, and reviewing her NTG-EDSD screen, she was diagnosed with Alzheimer’s dementia. She is currently receiving support from her team and friends, who have been educated on best practices for supporting people with dementia.
About the authors:
Ley is a Board-Certified Behavior Analyst with an academic and professional background in gerontology and applied behavior analysis. Ley’s specialties include behavioral gerontology and the behavioral presentations of neurocognitive disorders, in addition to working with high-management behavioral needs for dually diagnosed persons with intellectual disabilities and mental illness. He is an officer on the Board of Directors for the National Task Group on Intellectual Disabilities and Dementia Practices, works closely with national organizations such as the National Down Syndrome Society, and is the owner/operator of Crescent Behavioral Health Services based in Columbia, SC.
Dr. Craig Escudé is a board-certified Fellow of the American Academy of Family Physicians and the American Academy of Developmental Medicine and President of IntellectAbility. He has over 20 years of clinical experience providing medical care for people with IDD and complex medical and mental health conditions. He is the author of “Clinical Pearls in IDD Healthcare” and developer of the “Curriculum in IDD Healthcare,” an eLearning course used to train clinicians on the fundamentals of healthcare for people with IDD. He is also the host of the “IDD Health Matters” podcast.