Aided language modelling, responsive communication and eye-gaze technology as communication intervention for adults with Rett syndrome: three experimental single case studies

Figures & data

Figure 1. Study phases. See Intervention section for details.

Table 1. Participant characteristics.

	Diana	Karen	Sarah
Age	31 years	27 years	29
Seizures	A few seizures per month	No seizures	Daily seizures
Antiepileptic medication	Yes	Yes	Yes
MECP2 mutation	Identified	Not tested	Identified
Ambulatory status^a	Level IV	Level IV	Level III
Hand function^b	Level 1	Level 1	Level 6
Yes/no signal	Blink/move head to side	Symbols for yes/no	Symbols for yes/no
Current aided AAC	Photos, objects, low tech communication book, gaze-controlled device	Gaze-controlled device	Photos, small set of abstract symbols, gaze-controlled device

^aThe Modified Hoffer Scale (Vogel et al., [40]) was used to assess ambulatory status. Level IV = Non ambulant/stander. The person needs a wheelchair for all daily mobility activities but is able to stand. The person is capable of standing on the floor with or without physical assistance or in a standing frame. Level III = Therapeutic ambulator. The person walks with therapists or parents in the home or at school/day centre. The gait is not functional.

^b The Rett Syndrome Hand Function Scale (Downs et al., [41]) was used to assess hand function. Level 1= no grasping ability present, Level 6 = Able to grasp, pick up, and hold at least one large object >2 s AND use the radial side of the hand to grasp, pick up, and hold a small object >2 s. Can be a scissors, inferior pincher or superior pincer grasp.

Figure 2. Synthesised words per minute expressed by Diana. Between session 16 and 17 there was a ten-day break. The dotted line = median score.

Figure 3. Unique synthesised words per minute expressed by Diana. Between session 16 and 17 there was a ten-day break. The dotted line = median score.

Figure 4. Rate of Diana’s focused gazes. Between session 16 and 17 there was a ten-day break. The dotted line = median score.

Figure 5. Synthesised words per minute expressed by Karen. Between session 16 and 17 there was a ten-day break. The dotted line = median score.

Figure 6. Unique synthesised words per minute expressed by Karen. Between session 16 and 17 there was a ten-day break. The dotted line = median score.

Figure 7. Rate of Karen’s focused gazes. Between session 16 and 17 there was a ten-day break. The dotted line = median score.

Figure 8. Synthesised words per minute expressed by Sarah. Between session 10 and 11 there was a ten-day break. The dotted line = median score.

Figure 9. Unique synthesised words per minute expressed by Sarah. Between session 10 and 11 there was a ten-day break. The dotted line = median score.

Figure 10. Rate of Sarah’s focused gazes. Between session 10 and 11 there was a ten-day break. The dotted line = median score.

Table 2. Tau-U across all phases and Tau-U comparing phase A and B1.

Participants	Dependent measure	Tau-U^a	P^a	CI^a (85%)	Tau-U^b	P^b	CI^b (85%)
Diana	Words	0.49	.01	0.206< >0.764	0.65	.05	0.181< >1
	Unique words	0.74	.0001	0.4581< >1	0.85	.008	0.389< >1
	Focused gazes	0.74	.0002	0.4408< >0.9985	0.88	.007	0.410< >1
Karen	Words	0.25	.3	−0.0987< >0.5904	1	.04	0.311< >1
	Unique words	0.69	.001	0.3871< >0.9868	0.77	.02	0.306< >1
	Focused gazes	0.07	.7	−0.2366< >0.3831	−0.62	.06	−1< >−0.139
Sarah	Words	0.21	.3	−0.0787< >0.4943	0.21	.5	−0.256< >0.673
	Unique words	0.68	.0007	0.3909< >0.9639	0.88	.007	0.410< >1
	Focused gazes	0.26	.2	−0.0305< >0.5481	−0.36	.3	−0.837< >0.123

^aAcross all phases and ^bcomparing phase A and B1. Benchmarks for effectiveness: small <.20; moderate .21–.60; large .61–.80; very large >.80.

Vogel LC, Mendoza MM, C SJ, et al. Ambulation in children and youth with spinal cord injuries. J Spinal Cord Med. 2007;30(Suppl 1):S158–S164.

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Downs J, Bebbington A, Jacoby P, et al. Level of purposeful hand function as a marker of clinical severity in Rett syndrome. Dev Med Child Neurol. 2010;52(9):817–823.

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