Table 7.
Relationship between Behavioral Profile, Developmental and Clinical Features, and Treatments for Participants with Classic RTT.
| Category | Total % (n=687) |
Internalizing- only Behavioral Cohort % (n=368) |
Mixed Behavioral Cohort % (n=319) |
χ2 | Fisher’s Exact p-value |
|
|---|---|---|---|---|---|---|
| Developmental Features | ||||||
| Holds cup/bottle to drink | 43.2 | 35.4 | 52.4 | 17.205* | 0.000 | |
| Walking unsupported | 52.4 | 45.5 | 60.5 | 13.326* | 0.000 | |
| Hand use to feed self | 46.6 | 40.1 | 54.2 | 11.736* | 0.001 | |
| Standing unsupported | 55.1 | 48.9 | 62.4 | 10.727* | 0.001 | |
| Walking supported | 73.6 | 68.7 | 79.3 | 8.599 | 0.004 | |
| Pincer grasp | 21.0 | 16.9 | 25.8 | 6.995 | 0.009 | |
| Verbalize with meaning | 23.7 | 20.4 | 27.7 | 4.313 | 0.042 | |
| Standing supported | 88.3 | 85.9 | 91.1 | 3.911 | 0.054 | |
| Manipulates objects with hands | 44.4 | 44.2 | 44.6 | 0.012 | 0.934 | |
| Clinical Features | ||||||
| General | Self-Abuse | 35.4 | 25.4 | 47.2 | 30.554* | 0.000 |
| Seizures | 60.0 | 65.2 | 53.9 | 7.836 | 0.005 | |
| Cool hands | 49.3 | 48.9 | 49.8 | 0.049 | 0.869 | |
| Cool feet | 80.0 | 82.4 | 77.1 | 2.595 | 0.121 | |
| Gastrointestinal | Constipation | 82.0 | 86.2 | 77.1 | 8.206 | 0.005 |
| Gastroesophageal reflux |
44.2 | 41.1 | 48.0 | 2.832 | 0.097 | |
| Gallbladder dysfunction |
1.9 | 2.5 | 1.1 | 1.571 | 0.210 | |
| Diarrhea | 9.0 | 8.2 | 10.0 | 0.589 | 0.472 | |
| Sleep | Frequent daytime naps |
37.1 | 42.3 | 31.0 | 8.049 | 0.005 |
| Difficulty arousing in the morning |
13.6 | 12.2 | 15.1 | 1.054 | 0.335 | |
| Difficulty going to sleep |
23.9 | 22.3 | 25.8 | 1.029 | 0.333 | |
| Wakes frequently during night |
41.5 | 39.8 | 43.5 | 0.840 | 0.402 | |
| Breathing | Hyperventilation | 54.9 | 53.3 | 56.8 | 0.740 | 0.407 |
| Breath holding | 72.7 | 71.5 | 74.2 | 0.537 | 0.516 | |
| Puffing air or saliva |
51.4 | 50.2 | 52.8 | 0.400 | 0.563 | |
| Aerophagia | 48.5 | 48.3 | 48.7 | 0.011 | 0.934 | |
| Oromotor | Drooling | 76.9 | 77.7 | 76.0 | 0.247 | 0.625 |
| Chokes easily | 42.4 | 41.7 | 43.2 | 0.132 | 0.739 | |
| Effective chewing |
55.9 | 56.1 | 55.7 | 0.009 | 0.934 | |
| Bruxism | 78.0 | 78.1 | 77.9 | 0.003 | 1.000 | |
| Treatments | ||||||
| Occupational therapy | 77.5 | 74.3 | 81.2 | 3.979 | 0.048 | |
| Attends school/adult program | 90.3 | 88.4 | 92.6 | 2.988 | 0.094 | |
| Music therapy | 16.1 | 14.4 | 18.1 | 1.454 | 0.261 | |
| Hippotherapy | 15.9 | 17.6 | 14.0 | 1.365 | 0.260 | |
| Speech therapy | 76.1 | 74.3 | 78.2 | 1.247 | 0.287 | |
| Any SSRI Use | 15.6 | 14.7 | 16.6 | 0.390 | 0.570 | |
| Anxiety – on any medication | 17.3 | 16.0 | 18.8 | 0.822 | 0.383 | |
| Physical therapy | 81.5 | 82.4 | 80.4 | 0.390 | 0.595 | |
| Swimming therapy | 16.3 | 15.7 | 17.0 | 0.182 | 0.737 | |
| Other therapy | 13.1 | 12.5 | 13.7 | 0.160 | 0.714 | |
| Anxiety – on an SSRI | 11.9 | 12.2 | 11.4. | 0.870 | 0.799 | |
| No therapy | 8.8 | 9.1 | 8.5 | 0.066 | 0.884 | |
*
Note. p <.00125 after Bonferroni corrections for multiple comparisons. RTT = Rett syndrome.