Dementia in People with Severe/Profound Intellectual (and Multiple) Disabilities: Practice-Based Observations of Symptoms

Figures & data

Table 1. Characteristics of respondents.

Characteristics	All respondents N = 100	Care professionals N = 87	Family members N = 13
Age (years [median (IQR), min-max])	46 (22), 21–83	42 (19), 21–68	63 (20), 44–83
Sex (% female)	91	94	69
Level of education: primary school, high school, mbo, hbo, wo (%)	2, 2, 37, 39, 20	0, 0, 39, 38, 23	15, 15, 23, 46, 0
Care institution: Ipse de Bruggen, ’s Heeren Loo, Alliade, Visio, other (%)		38, 21, 24, 9, 8	N/A
Role: physician, nurse specialist, DSP, psychologist, psychologic assistant, occupational therapist, speech therapist, physiotherapist, dietician (%)		6, 6, 51, 16, 2, 5, 8, 6, 1	N/A
Experience working with SPI(M)D (years [median (IQR), min-max])		15 (13), 0–44	N/A
Working with SPI(M)D: D, W, M, other (%)		39, 43, 13, 6	N/A
Experience working with SPI(M)D + (suspected) dementia (years [median (IQR), min-max])		10 (12), 0–33	N/A
Working with SPI(M)D + (suspected) dementia: D, W, M, other (%)		35, 38, 15, 13	N/A
Family relationship: parent, sibling, no family member but legal representative (%)		N/A	8, 77, 15
Years knowing relative ([median (IQR), min-max])		N/A	57 (13), 1–67
Frequency of visits (% W, M, Q)		N/A	31, 62, 8
Characteristics of relative with SPI(M)D + (suspected) dementia
- Age (years [median (IQR), min-max])		N/A	60 (9), 47–73
- Level of intellectual disability: severe, profound, not determined but probably severe/profound (%)		N/A	62, 31, 8
- Presence of Down syndrome (%)		N/A	54
- Presence of multiple disabilities (%)		N/A	62
- Living situation: care institution, at home (%)		N/A	92, 8

Percentages (rounded off to the nearest whole number without decimals) are calculated based on the total number of respondents per group (column). The group of psychologists is composed of behavioral therapists who studied psychology or special needs education (in Dutch: orthopedagogiek). Abbreviations: D, daily; DSP, direct support professional/caregiver; hbo, higher vocational education; M, monthly; mbo, intermediate vocational education; N/A, not applicable; Q, quarterly; SPI(M)D, severe/profound intellectual (and multiple) disabilities; W, weekly; wo, higher education.

Figure 1. Schematic overview of included and excluded survey respondents.

Figure 2. Respondents’ observations of cognitive and activities of daily living (ADL) changes in people with SPI(M)D since the onset of (suspected) dementia. Per item, the proportion (%) of decrease, unaltered, unaltered/never shown (i.e., unaltered for some persons, never shown for others), never shown and unknown are presented within each bar. From left to right, items are ordered from highest to lowest percentage of respondents observing a decrease (increase for losing objects) since (suspected) dementia. References: 1, (Alzheimer’s Association, 2021); 2, (Dekker, Wissing et al., 2021).

Figure 3. Respondents’ observations of behavioral and psychological changes in people with SPI(M)D since the onset of (suspected) dementia. Per item, the proportion (%) of decrease, decrease/increase (i.e., decrease for some persons, increase for others), increase, unaltered, unaltered/never shown (i.e., unaltered for some persons, never shown for others), never shown and unknown are presented within each bar. From left to right, changes are depicted from most frequently reported (either a decrease, an increase or a combination of both) to least frequently reported. Behavioral and psychological categories are provided in accordance with the sections of the BPSD-DS II (Dekker, Ulgiati et al., 2021).

Figure 4. Respondents’ observations of motor changes in people with SPI(M)D since the onset of (suspected) dementia. Per item, the proportions (%) of decrease, decrease/increase (i.e., decrease for some persons, increase for others), increase, unaltered, unaltered/never shown (i.e., unaltered for some persons, never shown for others), never shown and unknown are presented within each bar. From left to right, motor changes are presented from most frequently reported (either a decrease, an increase or a combination of both) to least frequently reported. References: 2, (Dekker, Wissing et al., 2021); 3, (Ries, 2018).

Figure 5. Respondents’ observations of changes in medical comorbidities in people with SPI(M)D since the onset of (suspected) dementia. Per item, the proportion (%) of decrease, decrease/increase (i.e., decrease for some persons, increase for others), increase, unaltered, unaltered/never shown (i.e., unaltered for some persons, never shown for others), never shown and unknown are shown within each bar. From left to right, motor changes are presented from most frequently reported (either a decrease, an increase or a combination of both) to least frequently reported. Reference: (Strydom et al., 2010).

Table 2. Characteristics of interviewees.

Characteristics	Care professionals N = 16
Age (years [median (IQR), min-max])	50 (21), 29–64
Sex (% female)	88
Level of education: mbo, hbo, wo (%)	13, 38, 50
Care institution: Ipse de Bruggen, ’s Heeren Loo, Alliade, Visio, other (%)	25, 19, 19, 31, 6
Role: physician, nurse specialist, DSP, psychologist, occupational therapist, speech therapist, physiotherapist (%)	19, 6, 19, 25, 13, 13, 6
Experience working with SPI(M)D (years [median (IQR), min-max])	22 (16), 5–33
Experience working with SPI(M)D + (suspected) dementia (number of people [median (IQR), min-max])	127 (186), 2–500

Percentages (rounded off to the nearest whole number without decimals) are calculated based on the total number of interviewees. The group of psychologists is composed of behavioral therapists who studied psychology or special needs education (in Dutch: orthopedagogiek). Abbreviations: DSP, direct support professional/caregiver; hbo, higher vocational education; mbo, intermediate vocational education; SPI(M)D, severe/profound intellectual (and multiple) disabilities; wo, higher education.

Table 3. Symptom matrix structuring dementia symptoms in people with SPI(M)D observed by interviewees.

			Verbal communication skills	Walking skills	Category-independent
Cognitive changes^1,2,3	Memory	P	↓ remembering names, ↑ saying incorrect things, ↑repeatedly asking something	↓ remembering: how to walk/dress up/set table, that you went to toilet, were to hang coat, that you were asked to sit on chair.	↓ understanding: what is (about to) happen, expectations, activities, changes, jokes, communication (verbal, augmentative and alternative). ↓ remembering: new information, that you were going to eat/drink, how to use cutlery, how to chew/swallow/brush teeth, what to do with food in mouth, that dining chairs belong to table.
		A
	Orientation in time	P	Saying “good morning” in afternoon	Put on pajamas in morning, regular clothes in evening
		A
	Orientation in place	P		↓ remembering: where you/rooms are, direction/destination, new routes. ↑ getting lost, ↑ suddenly standing still in rooms
		A		↑ stretching arms when passing doorways, ↑ agitation when wheelchair is turned
	Understanding visual images/ spatial relationships	P		↑ bumping into things, difficulty with floor transitions
		A		↑ bumping into things with wheelchair
	Language skills	P	↓ talking, ↓ speech intelligibility, ↓ number of words used, ↓ expression with words, ↓ language comprehension, stop talking mid-sentence, speaking confusedly
		A	↓ frequency of producing sounds
	Losing objects	P		↓ remembering where you put toys
		A
	Person recognition	P			↓ recognizing DSP/family members
		A
	Object recognition	P		↓ recognizing walking lines on floor	↓ recognizing food/cutlery/table/chair/doll
		A
	Sound recognition	P			↓ recognizing sounds/songs
		A
	Preference for (favorite) objects	P		Not going to preferred seat	Liking things that were previously disliked, no longer touching cup/toys that someone used to hold
		A
	Responsiveness	P			No response while previously full of expectations, ↓ quickness of response, ↓ contact, ↓ alertness, ↑ alertness, ↓ keeping up with pace, ↓ reacting on the environment, ↑ staring ahead, ↑ not making eye contact, ↑ not responding to songs/activities
		A
	Awareness of proper order	P		↓ understanding sequence of showering before dressing up, ↑ dependency on structure, put underwear over pants, start activities at wrong moment	↓ remembering: daily routines, consecutive steps. Shift daily routines, skip steps, ↓ understanding daily activity icons
		A
	Sensory sensitivities	P			Sensory overload, negative reactions to stimuli, cannot bear sounds/songs/light, ↓ toleration of other residents, perceiving being touched as unpleasant, clothes feel uncomfortable, ↑ seeking proprioceptive input
		A
	Concentration	P			↓ concentration
		A
ADL changes^1,2,3	ADL functioning	P		↓ ability to: (un)dress, put on socks/coat, go to toilet, stair climbing.	↓ ability to: eat/drink, take food from spoon, open mouth, use cutlery/cup, washing vegetables, fold laundry, pick up puzzle pieces.
		A		↓ ability to propel wheelchair
Behavioral and psychological changes^4,5	Anxious behavior	P		↑ hesitant to walk, sliding across floor	↑ screaming, ↑ crying, ↑ whining, ↑ nervous, ↑ tension, ↑ panic, ↑ stress, ↑ freezing, inability to relax, anxious facial expression, fear in someone’s eyes, eyes wide open, forehead sweating, ↑ feeling unsafe, ↑ fear of being alone/hoist/going outside/hard noises/at night/ when eating, ↑ frightened when being touched/ball is thrown, clinging to table, avoiding things, ↑ contact seeking, ↑ seeking security, hesitant to let DSP go
		A		↓ anxiety for hoist
	Sleeping problems	P		↑ wandering at night, crawl out of bed	Day-night rhythm disturbance, ↑ insomnia, ↑ prowling about/restlessness/waking up/screaming at night, ↑ difficulty getting up, ↑ sleeping during day, ↑ being tired during day, falling asleep earlier
		A
	Irritable behavior	P			↑ touchy, ↑ irritability, ↑ frustration, ↑ anger, ↑ yelling, ↑ screaming during ADL, ↑ sounds of discomfort
		A
	Obstinate behavior	P		↓ willingness to walk	↑ resistance against eating/dressing up/showering/ activities. ↑ being uncooperative, ↑ being self-willed, no longer accepting aids, ↑ turning head away
		A
	Restless/stereotypic behavior	P	↑ repeating words/questions, excessive talking	↑ walking, ↑ wandering	↑ restlessness, ↑ compulsive acts, ↑ stereotypical acts, ↑ picking behavior, ↑ fecal smearing, ↑ rituals preventing to sleep
		A
	Aggressive behavior	P		↑ slam doors	↑ verbal/physical aggression against self and/or others, ↑ biting/beating, ↑ throwing objects
		A
	Apathetic behavior	P		↓ motivation to walk, not getting of sofa	↑ withdrawn, ↑ being passive, ↑ laziness, ↓ initiative, ↓ motivation, ↓ jovial, ↓ enjoying food/music, ↓ interest in activities they used to like, ↓ focus on eating, ↓ (eye) contact, ↓ noticing things, ↑ staring ahead, ↑ letting go daily structure
		A		↑ sitting still in wheelchair
	Depressive behavior	P			↓ emotion regulation, ↓ smiling, ↑ crying, ↑ discourages, ↑ mood changes, very unhappy, hollow eyes, head down
		A
	Psychotic behavior	P	↑ mentioning things that are not there		↑ suddenly looking at something/noticing things
		A
	Eating/drinkingbehavior	P			↓ appetite/eating/drinking, ↓ preference favorite food, eating slowly
		A
Motor changes^5	Motor skills	P		↓ walking distance/speed, gait changes, ↓ lower limb coordination, ↓ standing up, ↑ bottom shuffle, ↑ wheelchair use	↓ movement speed, ↑ stiffness, ↑ clumsiness, ↓ muscle strength, ↑ muscle tension, ↑ cramps, ↑ overstretching muscles, ↑ fetal sleep position, ↑ sitting cross-legged
		A
	Balance	P		↑ gait clumsiness/unsteadiness, ↑ insecure walking, ↑ tripping, ↑ falling, ↓ body awareness
		A		↓ maintaining body posture
	Chewing/ swallowing	P			↓ chewing, ↓ swallowing, ↑ unsafe swallowing, ↑ swallowing without chewing,↑ choking
		A
Medicalcomorbidities^6		P		↑ incontinence, ↑ weight, ↓ bowel movements, ↑ bedridden	↑ epilepsy, ↓ weight
		A

Dementia symptoms reported by interviewees were categorized based on symptom domains and items addressed in the survey (rows) and verbal communication/walking skills at baseline, i.e., highest level of functioning before dementia-related decline occurred (columns). Legend: ↓ = decrease compared to baseline level of functioning, ↑ = increase compared to baseline level of functioning. Abbreviations: ADL, activities of daily living; A, skill/behavior absent at baseline; DSP, direct support professional/caregiver; P, skill/behavior present at baseline. References: 1, (American Psychiatric Association, 2013); 2, (McKhann et al., 2011); 3, (World Health Organization, 2018); 4, (Dekker, Ulgiati et al., 2021); 5, (Ries, 2018); 6, (Strydom et al., 2010).

Table 4. Comparison of dementia symptoms in people with SPI(M)D obtained with different research methods.

	Symptoms	Survey	Interviews	Focus groups^1	Literature review^2
Cognitive changes	↓ Memory	✔	✔	✔	✔
	↓ Orientation in place	✔	✔	✔	✔
	↓ Language skills	✔	✔	✔	✔
	↓ Responsiveness	✔	✔	✔
	↓ Person recognition	✔	✔	✔
	↓ Awareness of proper order	✔	✔	✔
	↓ Object recognition	✔	✔	✔
	↓ Orientation in time	✔	✔	✔
	↓ Preference for (favorite) objects	✔	✔	✔
	↓ Understanding visual images/spatial relationships	✔	✔	✔
	↓ Concentration	✔	✔
	↑ Losing objects	✔	✔
	↑ Sensory sensitivities	✔	✔
	↓ Planning	✔
	↓ Problem solving	✔
	↓ Judgment	✔
	↓ Sound recognition		✔
ADL	↓ ADL	✔	✔	✔*	✔
Behavioral and psychological changes	↑ Irritable behavior	✔	✔	✔	✔
	↓ Eating/drinking behavior	✔	✔	✔	✔
	↑ Apathetic behavior	✔	✔	✔	✔
	↑ Sleeping problems	✔	✔	✔	✔
	↑ Restless/stereotypic behavior	✔	✔	✔	✔
	↑ Aggressive behavior	✔	✔	✔	✔
	↑ Anxious behavior	✔	✔	✔
	↑ Obstinate behavior	✔	✔	✔
	↑ Disinhibited behavior	✔		✔	✔
	↑ Depressive behavior	✔	✔	✔
	↑ Psychotic behavior	✔	✔	✔
Motor changes	↓ Walking	✔	✔	✔	✔
	↑ Wheelchair use	✔	✔	✔
	↓ Balance	✔	✔	✔
	↑ Fall frequency	✔	✔	✔
	↑ Swallowing problems	✔	✔	✔
	↑ Stiffness	✔	✔	✔
	↑ Cramps	✔	✔	✔
	↓ Body awareness	✔	✔	✔
	↓ Muscle strength	✔	✔	✔
	↓ Motor skills	✔	✔	✔
	↓ Movement speed	✔	✔
	↑ Fetal sitting/laying position	✔	✔
	↑ Tremor	✔
Medical comorbidities	↓ Weight	✔	✔	✔	✔
	↑ Incontinence	✔	✔	✔	✔
	↑ Epilepsy	✔	✔	✔	✔
	↑ Bedridden	✔	✔		✔
	↑ Pain	✔		✔
	↓ Taste sensation	✔		✔
	↓ Bowel movements		✔

This table provides a comparison of dementia symptoms reported in the survey, interviews with previously published findings using two other research methods, namely focus groups (Dekker, Wissing et al., 2021) and systematic literature review (Wissing et al., 2021). Symptoms are categorized in five symptom domains, which is in line with dementia criteria (American Psychiatric Association, 2013; McKhann et al., 2011; World Health Organization, 2018) and literature (Dekker, Ulgiati et al., 2021; Ries, 2018; Strydom et al., 2010). ✔ indicates that a symptom was reported in a research method. For behavioral and psychological changes, motor changes and medical comorbidities only the most prominently reported symptoms are presented. Legend: ↓ = decrease compared to baseline level of functioning, ↑ = increase compared to baseline level of functioning. Baseline level of functioning is the highest level of functioning before dementia-related decline occurred. *Symptoms reported in focus groups were categorized based on the daily contexts in which they were often observed in practice. Therefore, a decline in activities of daily living (ADL) functioning was addressed in various contexts and symptoms.

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