| Literature DB >> 33074178 |
Agnieszka Stępień1, Katarzyna Maślanko2, Maciej Krawczyk1, Witold Rekowski3, Anna Kostera-Pruszczyk4.
Abstract
BACKGROUND: Alternating hemiplegia of Childhood (AHC) is a rare disease manifested by transient episodes of hemiplegia and other neurological disorders. Delayed motor development has been reported in patients with AHC, but detailed features of the motor impairment have not been described so far. AIM: The aim of the study was to evaluate gross motor function between attacks in a group of Polish patients with AHC.Entities:
Keywords: alternating hemiplegia of childhood; child development; gross motor function disorders; physiotherapy; rare diseases
Mesh:
Year: 2020 PMID: 33074178 PMCID: PMC8518102 DOI: 10.34763/jmotherandchild.2020241.1935.000003
Source DB: PubMed Journal: J Mother Child ISSN: 1428-345X
Gross Motor Function AHC scale – differences between study (AHC) and control groups
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| 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 |
SD, standard deviation; SE, standard error.
p calculated using the Mann–Whitney U–test;
p<0.05.
Characteristics of the study (AHC) and control groups
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| 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 |
Characteristics of the study (AHC) group – signs
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| 1 | + | + | Plegic attacks | 1–2 | 30 min–2 h | Yes |
| 2 | + | + | Plegic attacks | 4–8 | 7 days–several days | No |
| 3 | + | + | Plegic attacks | A few | A few minutes to few hours | Yes |
| 4 | + | + | Plegic attacks | 1–30 | A few minutes to 12 h | No |
| 5 | + | Tonic/dystonic attacks | 5–30 | 30 s–1 min | Yes | |
| 6 | + | + | Plegic attacks | 10 | A few hours–2 days | Yes |
| 7 | + | + | Plegic attacks | 1–20 | A few minutes to a few hours | Yes |
| 8 | + | + | Plegic attacks | One to a few | A few hours to a few days | No |
| 9 | + | + | Plegic attacks | 17–20 | A few minutes to a few days | No |
| 10 | + | + | Tonic/dystonic attacks | Few – 20 | Several minutes to a few hours | Yes |
Gross Motor Function AHC
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| 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 | 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 | 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 | 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 |
Tasks based on the Hammersmith Functional Motor Scale Expanded (HFMSE);
the Gross Motor Function Measure (GMFM) and
other motor tasks.