Gait deviations in patients with dravet syndrome: A systematic review

Wyers, Lore; Walle, Patricia Van de; Hoornweg, Aurélie; Bobescu, I. Tepes; Verheyen, Karen; Ceulemans, Berten; Schoonjans, An‐Sofie; Desloovere, Kaat; Hallemans, Ann

doi:10.1016/j.ejpn.2019.03.003

Cited by 24 publications

(28 citation statements)

References 37 publications

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“…Around adolescence, walking problems become evident, making many of the patients lean on others or use a wheelchair for longer distances [5]. Literature on gait deviations in patients with DS is still scarce and mainly observational [6].…”

Section: Introductionmentioning

confidence: 99%

“…Quantitative analysis of gait in this population is only recently reported [7]. Crouch gait is often described in about half of the population, next to a variety of other deviations [6,7]. Crouch gait is defined by excessive knee flexion in stance and was originally described in populations with cerebral palsy, where it is caused by a complex of muscle weakness, spasticity and contractures [8,9].…”

Section: Introductionmentioning

confidence: 99%

“…Crouch gait is defined by excessive knee flexion in stance and was originally described in populations with cerebral palsy, where it is caused by a complex of muscle weakness, spasticity and contractures [8,9]. However, these symptoms are rarely seen in patients with DS [6,10], hence the nature of their gait problems remains unclear.…”

Section: Introductionmentioning

confidence: 99%

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The mechanics behind gait problems in patients with Dravet Syndrome

et al. 2021

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BackgroundDravet Syndrome (DS) is a developmental and epileptic encephalopathy starting in infancy and characterised by treatment resistant epilepsy with cognitive impairment and progressive motor dysfunction. Walking becomes markedly impaired with age, but the mechanical nature of gait problems remains unclear. Research questionWhat are the kinetic strategies characterised in gait of patients with DS? MethodsThis case-control study compared 41 patients with DS aged 5.2 to 26.1 years (19 female, 22 male) to 41 typically developing (TD) peers. Three dimensional gait analysis (VICON) was performed to obtain spatiotemporal parameters, kinematics and kinetics during barefoot, level walking at self-selected walking velocity. The sagittal plane support moment was analysed using Statistical Parametric Mapping (SPM). Three DS subgroups were identified based on differences in kinetic strategies characterised by the net internal knee joint moments and trunk lean. Kinematic and kinetic time profiles of the subgroups were compared to the TD group (SPM t-test).Clinical characteristics from physical examination and parental anamnesis were compared between DS (sub)groups using non-parametric tests (Kruskal-Wallis, Wilcoxon rank-sum, Fisher's exact). ResultsSupport moments in stance were significantly increased in the DS group compared to TD and strongly related to minimum knee flexion in midstance. Persistent internal knee extension moments during stance were detected in a subgroup of 27% of the patients. A second subgroup of 34% showed forward trunk lean and attained internal knee flexion moments. The remaining 39% had neutral or backward trunk lean with internal knee flexion moments. Subgroups differed significantly in age and functional mobility. SignificanceInefficient kinetic patterns suggested that increased muscle effort was needed to control lower limb stability.Three distinct kinetic strategies that underly kinematic deviations were identified. Clinical evaluation of gait should pay attention to knee angles, trunk lean and support moments.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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The mechanics behind gait problems in patients with Dravet Syndrome

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“…Treatment of DS and LGS focuses on alleviation of seizure burden, but some patients may have treatment-resistant seizures. In addition, DS and LGS are associated with multiple comorbidities, which present further management challenges [4,10,[15][16][17]. Despite their different etiologies, DS and LGS share several comorbid features, including developmental delays in several domains: social-emotional, cognitive, motor, adaptive, and communicative [18][19][20][21][22].…”

Section: Introductionmentioning

confidence: 99%

Comorbidities in Dravet Syndrome and Lennox–Gastaut Syndrome

Marchese

Cappelletti

Filippini³

et al. 2021

SN Compr. Clin. Med.

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This study aims to describe the main cognitive and behavioral comorbidities of Dravet syndrome (DS) and Lennox–Gastaut syndrome (LGS), their impact on the health-related quality of life (QOL) of patients and their caregivers, and provide a summary of the neuropsychological tools available for the evaluation of these comorbidities. The cognitive and behavioral comorbidities in patients with DS and LGS have a profound effect on the QOL of affected individuals and their caregivers and, as patients grow, tend to surpass the impact of the seizures. DS is a genetic condition associated with loss-of-function mutations in the SCNA1 sodium channel gene; LGS is an etiologically heterogeneous condition that is often secondary to structural brain abnormalities. The first seizures associated with DS typically present in the first year of life, and developmental delay becomes progressively evident thereafter. LGS usually starts between the ages of 3 and 8 years, with cognitive impairment becoming clinically evident in most patients within 5 years from the onset. In both DS and LGS, cognitive impairment is generally moderate to severe and is often accompanied by behavioral problems such as hyperactivity and inattention. In addition to optimal seizure control, regular assessment and active management of cognitive and behavioral comorbidities are required to meet the complex needs of patients with DS or LGS.

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“…Children with DS show a delay in motor development before the age of two, often with a delayed achievement of independent walking [5][6][7]. Diverse gait alterations have been described in DS, with about half of the investigated cases presenting a gait pattern which resembles crouch gait [6,8,9]. Although gait problems are a major concern for children and adults with DS and their caregivers [10,11], scarce literature has addressed this topic and especially quantitative assessments of gait deviations are lacking [8].…”

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confidence: 99%

Foot-floor contact pattern in children and adults with Dravet Syndrome

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Background: Dravet Syndrome (DS) is a developmental and epileptic encephalopathy characterized by severe drug-resistant seizures and associated with cognitive and motor impairments. Walking problems are frequently observed. As the foot plays a key role during walking, compromised foot function can be a feature of deviant gait. p.2Aim: To investigate foot function in DS by characterizing foot-floor contact patterns using pedobarography.Methods: A total of 31 children and adults were included in the DS group (aged 5.2-32.8 years, 17 female, 174 steps) and 30 in the control group (aged 6.0-32.9, 16 female, 180 steps). The foot-floor contact pattern was evaluated based on progression, length and smoothness (spectral arc length) of the center of pressure (CoP). Linear mixed models were used to identify differences between non-heel strikes and heel strikes and between the DS and control group. Results: Fifteen participants with DS showed inconsistency in the type of foot-floor contact (heel strikes and non-heel strikes). Heel strikes of participants with DS had significantly reduced time of CoP under the hindfoot and increased time under the midfoot region compared to the control group. Significant time and age effects were detected. Conclusions and Implications: Deviant foot-floor contact patterns were observed in DS. Possible gait immaturity and instability as well as implications for interventions are discussed.

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Gait deviations in patients with dravet syndrome: A systematic review

Cited by 24 publications

References 37 publications

The mechanics behind gait problems in patients with Dravet Syndrome

The mechanics behind gait problems in patients with Dravet Syndrome

Comorbidities in Dravet Syndrome and Lennox–Gastaut Syndrome

Foot-floor contact pattern in children and adults with Dravet Syndrome

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