1. Gait patterns in Dravet syndrome
Prof. Dr. Alessandra Del Felice
Department of Neuroscience, University of Padova, Italy
Prof. Dr. Ann Hallemans
Department of Rehabilitation Sciences, University of Antwerp, Belgium
Voorstellen
Anekdote vertellen van introductie aan elkaar: Myra en Isabella

2. Outline Gait in children with Dravet syndrome, key problems? Setting
Population
Space-Time parameters
Kinematics of gait
Adult pattern
Child patterns
Main Findings
Suggestions for gait rehabilitation
Next steps

3. Gait in Dravet Syndrome
Onset of walking normal to slightly delayed
Gait ataxia (Dravet, 1978; Ceulemans, 2011)
Described as crouch gait (Rodda et al., 2012; Rilstone et al., 2012)
Possible relation to nonsense mutations or mutations in the pore-forming region? (Rilstone et al., 2012)
BUT
Clinical observation of different patterns and progressions
Parkinsonian features? (Fasano et al. 2014)
Rehabilitative approach?
What is currently known about gait in children with Dravet syndrome
Video gait analysis
Rodda: 26 subjects; Rilstone 10
Different gait patterns? Confirm with objective 3D clinical gait analysis
Parkinsonian features? Also focus on trunk
Insights into gait biomechanics that can give directions for gait rehabilitation?
50 to 80%

4. Setting
Collaborative Italian Network: University of Verona, University of Padova, Istituto C Besta Milano
Dravet clinic from Anwerp University Hospital and Multidisciplinary Motor Centre Antwerp
So there were 2 different research groups interested in this gait problems in children with Dravet symdrome

5. Population Padova Antwerp Gender M/F Age (years) mean (± sd) BMI (m)
Scoliosis
Femoral
Anteversion
Pes
planovalgus
Adults
(n = 8)
7/1
19.7 (5.9)
21.7 (3.9)
4 (50%)
2 (25%)
Children
(n = 10)
6/4
9.7 (2.6)
18.3 (3.5)
4 (40%)
5 (50%)
7 (70%)
Padova
Gender
M/F
Age (years)
mean (± sd)
BMI (m)
Scoliosis
Femoral
Anteversion
Pes
planovalgus
Adults
(n = 5)
3/2
19.7 (2.8)
19.0 (4.2)
1 (20%)
3 (60%)
4 (80%)
Children
(n = 11)
5/6
8.13 (4.17)
17.7 (3.0)
5 (45%)
6 (54%)
Antwerp
Adolescent idiopathic scoliosis is a common disease with an overall prevalence of 0.47–5.2 % in the current literature
Age of 14 as a cut-off
Sample is 28 in total but 10 f them could not do the gait analysis
Adults (n = 13)
10/3
14 – 26 years
38%
62%
Childen (n = 21)
11/10
3 – 13 years
19%
48%
52%

6. Space/ Time parameters
Space parameters

7. Space/Time parameters** * **
Padova **
Only small differences: slower walking speed, shorter strides, slightly increased duration of stance
Children show a larger base of support (broader steps)
Antwerp
* = p< ** = p<0.001

8. Kinematics of gait Objectively describe movement
Davis R.B. III et al. A gait analysis data collection and reduction technique.
Human Movement Science 1991 Oct; 10(5):

9. Analysis workflow Data resampling every 1/100 of gait cycle
Data exportation from BTS format “.mdx”
Data re arrangement and format conversion
Clinical evaluation
95% cross-correlation test
Independent T-Test every 1/100 of gait cycle
Mean and SD of each 1/100 of gait cycle
Subgroups determination
Data output: normative bands, p<0.05 flag
Feedback

10. Pattern in the adult population
Trunk anteflexion
Reduced pendularism

11. Pattern in the adult population
Pelvic anteversion
Reduced hip extension in Isw

12. Pattern in the adult population
Mild flexed knee in stance
Mild reduced knee flexion in swing

13. Pattern in the adult population
Increased dorsiflexion in stance
Reduced plantar flexion at push off

14. Patterns in the pediatric population
2 patterns detected in the PD cohort
«poliflexed»
«smil-adult»
1 pattern in the Antwerp cohort
«mild with reduced push off»
Blu: bambini gruppo1 = De Ciuceis, Villareale, Montesi ('p0074' 'p0093' 'p0134')
- Rosso: bambini gruppo2 = Chtia, Segalini, Kerschakel, Roccato ('p0072' 'p0117' 'p0125' 'p0145')
- Verde: Adulti

15. Mild pattern with reduced push-off (Antwerp cohort)
Reduced Push off force

16. Pattern in pediatric population
Marked trunk anteflexion
Pelvis antiversion

17. Pattern in pediatric population
Increased flexion in LR
Reduced knee extension in St

18. Pattern in the pediatric population1
Increased Dorsiflexion in St
Increased plantiflexion in Sw

19. Pattern in the pediatric population1
Marked foot eversion and abduction

20. Main Findings Age-related patterns? Severity related? Wider steps
Adults
Children
1 pattern
Parkinsonian features
Small steps
Rigid trunk
Flexed position to increase stability?
Different patterns
Almost within normal articular range with limited push off/simil-adult
Polyflexed (psuedo-crouch)
Age-related patterns?
Severity related?
Wider steps
Gait ataxia?

21. Significance of these findings
Assume gait ataxia/ stability issues
Hip, knee and ankle flexion improve stability
External foot rotation increase support base
BUT
Lever arm function is compromised
Trunk anteflexion generates propulsion

22. Rehabilitative approach
Core stability exercises
Strenghtening of the ankle rockers
Orthotic insoles
Ankle-foot orthesis (AFO) for more compromised subjects

23. Next steps….. Longitudinal study
Electrophysiological studies (Gitiaux et al., 2016)
Rehabilitative guidelines
Collaborative European Network
European Registery

24. Dr. F Ragona Dr. T Granata Istituto Neurologico C Besta Milano, Italy Dr. F Darra Dr. E Fontana Prof B Dalla Bernardina Child Neurology, University of Verona, Italy Dr. C Boniver Dr. M Vecchi Child Neurology, University of Padova, Italy Ing. D Pavan Ing Z Sawacha Ing A Guiotto Departmento of Informatic Engineering, University of Padova Prof. MG Bendetti Istituto Ortopedico Rizzoli, Bologna, Italy
Prof. Dr. B. Ceulemans
Dr. A.S. Schoonjans
Ms. K. Verheyen
Ms. N. Op de Beeck
Child Neurology, Antwerp University Hospital
Dr. P. Van de Walle
Department of Rehabilitation Sciences and Physiotherapy, University of Antwerp