Progressive Gait Deterioration in Adolescents With Dravet Syndrome

Abstract

To characterize changes in gait by age in patients with Dravet syndrome.

The humanistic and economic burden of Dravet syndrome on caregivers and families: Implications for future research

“…Children with DS have a high frequency of comorbid diagnoses including autism [27], attention deficit disorder [10], and developmental delay [11,28]. They also evidence significant motor problems including deterioration in gait [29], behavioral/conduct problems [10,30], and communication problems [28]. In essence, caring for a child with DS requires that carer's contend with a range of cognitive, communication, and behavior problems beyond managing seizures [4].…”

Perception of impact of Dravet syndrome on children and caregivers in multiple countries: looking beyond seizures

“…[6][7][8] Adolescents and adults with Dravet syndrome may struggle to integrate with peers due to impaired cognition and communication difficulties, causing social isolation. Issues arise with fine motor skills and gross motor function, including abnormal gait that have an impact on physical function.…”

Antecollis and levodopa-responsive parkinsonism are late features of Dravet syndrome

“…"Crouch gait" has also been described in older patients. 2,3 Given the poor knowledge of the clinical features of patients with DS surviving until adulthood, we prospectively investigated the motor abnormalities in a consecutive sample of adults with genetically proven DS.…”

“…2 In keeping with an involvement of the basal ganglia, a previous adult series reported (1) lingual dyskinesias (unknown whether drug-induced), rigidity, and dystonic gait in 4 of 14 adult patients with DS 5 ; (2) "extrapyramidal signs" without additional description in 3 of 24 patients with DS 6 ; and (3) "kyphosis" only in patients 13 years old or older. 3,6 The etiology of these postural abnormalities is difficult to determine and is likely caused by multifactorial processes. 4 Given the lack of full cooperation, we could not systematically assess the strength of neck extensor muscles.…”