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93 Crouch gait in dravet syndrome: clinical, practical and personal observations
  1. Georgia Raniolo
  1. UK


Background Dravet syndrome (DS) is an aggressive developmental and genetic epileptic encephalopathy. Along with pharmacoresistant epileptic seizures, this rare disease is characterised by cognitive impairment, speech and language disability and, notably, mobility problems. Observational reports have shed some light on the progression of the disease, but the aetiology of DS is still poorly understood. The syndrome is further characterised by a progressive non-specific decline in gait. There is limited literature about the natural history of progressive gait decline in the context of DS.

Objectives In order to explore DS and its significant impact on patients’ and their families, this work will discuss personal experiences caring for a patient with this condition, followed by a systematic literature review focusing on the aetiology and natural history of gait abnormalities in DS, with reflections regarding the aforementioned case.

Methods The search via PubMed produced a total of nine articles. Four articles were excluded from the in-depth literature review as they focused on treatment options for patients with DS rather than the impact on crouch gait in children with DS.

Results The total number of children studied in this systematic review is 131. The final five articles are listed in table 1.

Abstract 93 Table 1

Table showing the key studies utilised for this literature review.

The literature reveals that children with DS show progressive gait deterioration that is noticeable in early childhood but has particular impact in the second decade of life. The literature suggests that children under 6 have a normal or variable gait pattern with possible features of joint hypermobility. Beyond this age, the progressive decline in gait is more heterogenous. The literature indicates that there may be an evolution element to the gait deterioration that requires further investigation.

Over the course of six years caring for Child X, I have witnessed a serious visible decline in their mobility. The change has been gradual but the impact this has had on their independence is massive. Child X follows a classic trajectory of progressive gait deterioration, as observed by the majority of authors in more than half of the population cohort, in the literature review.

Conclusions Caring for a young child with DS, has provided me with a unique insight and first-hand account of the deterioration of their mobility, as well as the impact this has on their quality of life. More consideration is now being directed towards how families cope in their daily lives with the multiple aspects of DS.

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