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Gross Motor Function Disorders in Patients with Alternating Hemiplegia of Childhood

Agnieszka Stępień
Agnieszka Stępień
, 
Katarzyna Maślanko
Katarzyna Maślanko
, 
Maciej Krawczyk
Maciej Krawczyk
, 
Witold Rekowski
Witold Rekowski
 y 
Anna Kostera-Pruszczyk
Anna Kostera-Pruszczyk
   | 29 jul 2020
Journal of Mother and Child's Cover Image
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Article Category: Original Research Paper
Publicado en línea: 29 jul 2020
Páginas: 24 - 32
DOI: https://doi.org/10.34763/jmotherandchild.2020241.1935.000003
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© 2020 Agnieszka Stępień et al., published by Sciendo
This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

Gross Motor Function AHC

Item No. Item Points: 0 Points: 1 Points: 2
1 Lifts head from supine position† Unable Head is lifted, but through side flexion or with no neck flexion. Held for a count of 3 In supine position, head must be lifted in midline. Chin moves towards chest. Held for a count of 3
2 Lying to sitting position† Unable Turns into prone position or towards floor Able to do by lying on side
3 Sitting† 2 hands for support 1 hand for support, for a count of 3 No hand support, for a count of 3
4 Long sitting † 2 hands for support 1 hand for support, for a count of 3 No hand support, for a count of 3
5 Right hand to head in sitting position† Hand touches head above level of ears Unable Can only bring right hand to head by flexing head Able to bring right hand to head. Head and trunk remain stable
6 Left hand to head in sitting position† Hand touches head above level of ears Unable Can only bring left hand to head by flexing head Able to bring right hand to head. Head and trunk remain stable
7 Two hands to head in sitting position† Hands touch head above level of ears Unable Able to place hands on head but only using head flexion or side tilt Able to place both hands on head, arms free from side. Head and trunk remain stable
8 Sitting to lying position† Unable or falls over Able to lie down by flopping forwards and rolling sideways Able to lie down in a controlled fashion through lying on side or using clothes
9 Supine position to side-lying position† Unable Can ½ roll only one way: either right or left Able to ½ roll from supine position both ways
10 Rolls from supine position to prone position over right † Unable Turns to prone position by pulling/pushing on arms Turns to prone position with free arms to the right
11 Rolls from prone position to supine position over right † Unable Turns to supine position using arms to push/pull with Turns to supine position with free arms to the right
12 Rolls from supine position to prone position over left† Unable Turns to prone position by pulling/pushing on arms Turns to prone position with free arms to the left
13 Rolls from prone position to supine position over left† Unable Turns to supine position using arms to push/pull with Turns to supine position with free arms to the left
14 Lifts head from prone position† Unable Lifts head with arms in a forward position for a count of 3 Able to lift head up in prone position, arms by side for a count of 3
15 Props on forearms† Unable Holds position when placed for a count of 3 Able to achieve prop on elbows with head up for a count of 3
16 Props on extended arms† Unable Holds position when placed Achieves hands support in prone position
17 Four-point kneeling † Unable Holds position when placed for a count of 3 Achieves four-point kneeling – head up for a count of 3
18 Crawling† Unable Moves all four points only once Able to crawl forwards – moves all four points twice or more
19 Gets to standing position from a supine position§ Unable Uses furniture Achieves standing without furniture
20 Supported standing† Can stand with hand support but needs knee/hip support in addition for a count of 3, unable Able to stand with minimal trunk support (not hip) for a count of 3 Can stand using one hand support for a count of 3
21 Stands unsupported† Stands only momentarily, unable Stands independently for a count of 3 Can stand independently for more than a count of 3
22 Squats† Unable Initiates squat (>10%), uses arm support, or static <3 seconds Squats with arms free (at least 90° of hip and knee flexion), static > 3 seconds
23 Gets off a chair without using arms§ Unable Uses arms Gets off independently without using arms
24 Walks forward‡ Unable Walks <10 steps Walks >10 steps
25 Stands on toes§ Unable Stands <5 seconds Stands >5 seconds
26 Walks on toes§ Unable Walks with assistance, <3 m Walks independently, >3 m
27 Walks on heels§ Unable Walks with assistance, <3 m Walks independently, >3 m
28 Runs‡ Unable Runs <4.5 m Runs >4.5 m, stops and returns
29 Hops on both legs§ Unable <5 consecutive hops >5 consecutive hops
30 Hops on right foot§ Unable <5 consecutive hops >5 consecutive hops
31 Hops on left foot§ Unable <5 consecutive hops >5 consecutive hops
32 Hops on alternate legs§ Unable Does not hop on alternate legs and arms, <10 consecutive hops Hops on alternate legs and arms, >10 consecutive hops
33 Ascends stairs without rail† Unable Ascends <4 stairs, arms free, any pattern or with arm support Ascends 4 stairs, arms free, alternating feet
34 Descends stairs without rail† Unable Descends <4 stairs, arms free, any pattern or with arm support Descends 4 stairs, arms free, alternating feet
35 Catches a ball with both hands, stands, ball 25 cm, 5 attempts§ Unable Catches a ball with both hands, throws to a distance >1.5 m Catches a ball with both hands >4 times
36 Throws a tennis ball, stands, dominant hand, 5 attempts§ Unable Throws distance <1.5 m or throws to a distance >1.5 m < 4 times Throws to a distance > 1.5 m, 4 or 5 times
37 Kicks ball – dominant leg, stands, ball 20 cm, 3 m, 5 attempts § Unable Kicks 3 m< 4 times Kicks 3 m> 4 times
38 Stands on right leg with eyes open, hands crossed on chest § Timer
39 Stands on left leg with eyes open, hands crossed on chest § Timer
40 Stands on right leg with eyes closed, hands crossed on chest § Timer
41 Stands on left leg with eyes closed, hands crossed on chest § Timer

Characteristics of the study (AHC) group – signs

No. Unilateral Attacks Bilateral attacks Signs Frequency (number of attacks per month) Average duration of episode Medicines
1 + + Plegic attacksTonic/dystonic attacksNystagmusDeviation of the eyesEpisodic respiratory difficultiesEpisodic autonomic disturbances 1–2 30 min–2 h Yes
2 + + Plegic attacksTonic/dystonic attacksNystagmusDeviation of the eyesEpisodic respiratory difficultiesEpisodic autonomic disturbances 4–8 7 days–several days No
3 + + Plegic attacksTonic/dystonic attacksAtaxia A few A few minutes to few hours Yes
4 + + Plegic attacksTonic/dystonic attacksNystagmusDeviation of the eyes 1–30 A few minutes to 12 h No
5 + Tonic/dystonic attacksNystagmusEpisodic autonomic disturbances 5–30 30 s–1 min Yes
6 + + Plegic attacksTonic/dystonic attacksDeviation of the eyesEpisodic respiratory difficulties 10 A few hours–2 days Yes
7 + + Plegic attacksTonic/dystonic attacksNystagmus 1–20 A few minutes to a few hours Yes
8 + + Plegic attacksTonic/dystonic attacksDeviation of the eyesEpisodic respiratory difficultiesEpisodic autonomic disturbances One to a few A few hours to a few days No
9 + + Plegic attacksTonic/dystonic attacksNystagmusEpisodic respiratory difficultiesPlegic attacks 17–20 A few minutes to a few days No
10 + + Tonic/dystonic attacksDeviation of the eyesEpisodic respiratory difficulties Few – 20 Several minutes to a few hours Yes

Characteristics of the study (AHC) and control groups

Study group (AHC) Control group
No. Gender Height (cm) Weight (kg) Age (years) Mutation of ATP1A3 No. Gender Mean height (cm) Mean weight (kg) Mean age (years)
1 F 83 11.5 2.5 + 1.1; 1.2; 1.3 F 89.8 13.6 2.5
2 M 104 14 3.5 + 2.1; 2.2; 2.3 M 100.3 15 3.5
3 M 104 19 3.5 3.1; 3.2; 3.3 M 103 19 3.5
4 F 124 23 6.2 + 4.1; 4.2; 4.3 F 121.3 22 6.6
5 F 128 22 9.1 5.1; 5.2. 5.3 F 141 31 8.9
6 M 132 26 9.1 + 6.1;6.2; 6.3 M 142.7 37 9.2
7 M 164 60 12.8 7.1; 7.2;7.3 M 159.7 53 12.9
8 M 188 50 20.3 + 8.1; 8.2; 8.3 M 181 74 20.5
9 F 154 52 24.3 + 9.1; 9.2; 9.3 F 169 62.3 24.3
10 M 184 70 30.1 + 10.1;10.2;10.3 M 182.5 82.5 30.3

Gross Motor Function AHC scale – differences between study (AHC) and control groups

Item Gross Motor Function AHC Study (AHC) group Gross Motor Function AHC Control group p
Mean ± SD SE Mean ± SD SE
1. Lifts head from supine position 1.90 ± 0.32 0.10 1.93 ± 0.25 0.05 0.73
2. Lying to sitting position 1.90 ± 0.32 0.10 2.00 ± 0.00 0.00 0.08
3. Sitting position 2.00 ± 0.00 0.00 2.00 ± 0.00 0.00 1.00
4. Long sitting position 2.00 ± 0.00 0.00 2.00 ± 0.00 0.00 1.00
5. Right hand to head in sitting position 1.90 ± 0.32 0.10 2.00 ± 0.00 0.00 0.08
6. Left hand to head in sitting position 1.90 ± 0.32 0.10 2.00 ± 0.00 0.00 0.08
7. Two hands to head in sitting position 1.90 ± 0.32 0.10 2.00 ± 0.00 0.00 0.08
8. Sitting to lying position 2.00 ± 0.00 0.00 2.00 ± 0.00 0.00 1.00
9. Supine position to lying on side 2.00 ± 0.00 0.00 2.00 ± 0.00 0.00 1.00
10. Rolls from supine position to prone position over right 1.90 ± 0.32 0.10 2.00 ± 0.00 0.00 0.08
11. Rolls from prone position to supine position over right 1.90 ± 0.32 0.10 2.00 ± 0.00 0.00 0.08
12. Rolls from supine position to prone position over left 1.90 ± 0.32 0.10 2.00 ± 0.00 0.00 0.08
13. Rolls from prone position to supine position over left 1.90 ± 0.32 0.10 2.00 ± 0.00 0.00 0.08
14. Lifts head from prone position 2.00 ± 0.00 0.00 2.00 ± 0.00 0.00 1.00
15. Props on forearms 2.00 ± 0.00 0.00 2.00 ± 0.00 0.00 1.00
16. Props on extended arms 2.00 ± 0.00 0.00 2.00 ± 0.00 0.00 1.00
17. Achieves four-point kneeling 1.90 ± 0.32 0.10 2.00 ± 0.00 0.00 0.08
18. Crawls 2.00 ± 0.00 0.00 2.00 ± 0.00 0.00 1.00
19. Gets to standing from a supine position 1.60 ± 0.84 0.27 2.00 ± 0.00 0.00 0.01*
20. Executes supported standing 1.80 ± 0.63 0.20 2.00 ± 0.00 0.00 0.08
21. Stands unsupported 1.60 ± 0.84 0.27 2.00 ± 0.00 0.00 0.01*
22. Squats 0.96 ± 0.61 0.19 1.79 ± 0.46 0.08 0.00*
23. Gets off a chair without using arms 1.60 ± 0.84 0.27 2.00 ± 0.00 0.00 0.01*
24. Walks forward 1.60 ± 0.84 0.27 2.00 ± 0.00 0.00 0.01*
25. Stands on toes 0.50 ± 0.53 0.17 1.90 ± 0.31 0.06 0.00*
26. Walks on toes 1.18 ± 0.90 0.28 2.00 ± 0.00 0.00 0.00*
27. Walks on heels 0.70 ± 0.82 0.26 2.00 ± 0.00 0.00 0.00*
28. Runs 0.94 ± 0.88 0.28 1.94 ± 0.13 0.02 0.00*
29. Hops on both legs 1.30 ± 0.82 0.26 2.00 ± 0.00 0.00 0.00*
30. Hops on right foot 0.40 ± 0.84 0.27 1.90 ± 0.31 0.06 0.00*
31. Hops on left foot 0.40 ± 0.84 0.27 1.83 ± 0.38 0.07 0.00*
32. Hops on alternate legs 0.43 ± 0.56 0.18 1.56 ± 0.48 0.09 0.00*
33. Ascends stairs without rail 1.25 ± 0.78 0.25 1.92 ± 0.20 0.04 0.00*
34. Descends stairs without rail 1.25 ± 0.78 0.25 1.92 ± 0.20 0.04 0.00*
35. Catches a ball with both hands 1.20 ± 0.79 0.25 1.93 ± 0.25 0.05 0.00*
36. Throws a tennis ball 1.60 ± 0.84 0.27 2.00 ± 0.00 0.00 0.01*
37. Kicks ball – dominant leg 1.60 ± 0.84 0.27 2.00 ± 0.00 0.00 0.01*
38. Stands on right leg with eyes open 3.58 ± 5.01 1.59 11.92 ± 4.59 0.84 0.00*
39. Stands on left leg with eyes open 3.78 ± 5.07 1.60 11.81 ± 4.43 0.81 0.00*
40. Stands on right leg with eyes closed 2.22 ± 3.52 1.11 8.82 ± 5.97 1.09 0.00*
41. Stands on left leg with eyes closed 1.93 ± 2.48 0.79 9.03 ± 6.03 1.10 0.00*
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