For individuals with mild to moderate developmentally disabilities the type, prevalence, and progression of chronic dementia, over the ago of 60, is similar to the general population. Individuals with Down’s syndrome and individuals with severe and profound developmental disabilities may be the exceptions. 

There is very little known about dementia in the severe and profound developmentally disabled adult for two reasons: such individuals usually do not live long enough to experience dementia that is associated with aging, and the symptoms of dementia may be masked by disabilities. 

What is chronic dementia is a clinical syndrome describing a change in behavior/personality and impairment in memory, thinking skills (organization and planning), judgment, and language that is persistent and interferes with levels of social activities, routine activities of daily living, and/or occupational function. Many of the symptoms of acute dementia are similar to those of chronic dementia.

Definition of dementia related symptoms:

Behavior/personality change:

• passivity (withdrawn), apathy, and less responsive

• irritable, easily upset

• suspicious/paranoid

• volatile emotion

Cognition:

• memory - learning or training new information

• higher executive skills- handling complex tasks, abstract thought, planning, problem solving

• reasoning ability - inability to respond to problems, reduced judgment

• spatial and orientation ability- organizing objects, finding way in familiar surroundings

• language - finding words, following conversation

Alzheimer’s Disease:

1. Alzheimer’s disease is the most prevalent form of dementia

2. Risk factors for Alzheimer’s disease:

• Age:  Risk rises exponentially with age, doubling in each decade after age 65

• Family history/genetic disposition: People with relatives with Alzheimer’s disease are 20-40% at risk

• Vascular disease (clot or hemorrhagic mini strokes), high blood pressure, high cholesterol, diabetes 2

• Head injury: AD occurs more often among people who suffered traumatic head injuries earlier in life

• Adults with Down syndrome 50 to 60 years old

3. Step by step increase in symptoms (each step continuously becomes worse):

Mild Symptoms (first set of symptoms, 1-5 years):

• Short term memory impaired, forgetfulness, new learning defective; remote recall impaired

• Confusion

• Problems with routine tasks

• Disorientation to person, place and time, getting lost in familiar surroundings

• Difficulty remembering names of persons and objects

• Personality change: apathy, occasional irritability or sadness

• Reduced judgment

Moderate Symptoms (next set of symptoms, 5 -10 years):

• Difficulty with activities of daily living, such as feeding and bathing

• Anxiety, suspiciousness, agitation

• Sleep disturbances/ Insomnia

• Wandering, pacing, restlessness

• Difficulty recognizing family and friends

• Recent and remote memory severely impaired

• Spatial disorientation impaired

• Marked decrease in expressive language and comprehension abilities

• Personality- indifference, apathy, anxiety, delusion, paranoia

Severe Symptoms (last stage, 10 to 17 or more years):

• Loss of speech

• Loss of appetite; weight loss

• Cannot understand simple directions

• Difficulty in swallowing and eating

• Limbs rigid; posture flexed

• Urinary and fecal incontinence

• Total care – Activities of Daily Living impaired

• Secondary conditions

• Death

Alzheimer’s Disease and Adults with Down Syndrome

Differences between individuals with developmentally disabilities and general population:

•Differences in rate of occurrence - Much higher: 100% of adults with Down’s syndrome will have all the clinical neurological changes associated with Alzheimer’s disease but only 40% of all adults with Down’s syndrome will be diagnosed with Alzheimer’s

•Onset and duration - Earlier onset and shorter duration: Average onset age for adults with Down’s syndrome adult is about 53 years old – late 60s for general population

•Behavioral changes - Early personality change & memory loss

•Neurological signs - Late onset of seizures

Main points:

• Adults with Down’s syndrome are at risk for acute dementia

• Other adults with developmental disabilities are the at same risk as general population

• Assessment is based on comparing the person to him or herself over time

• Community care is similar to other people affected by dementia