DANCE checklist of non-seizure Dravet features may aid diagnosis
Tool may help identify other co-occurring conditions in patients: Study
The newly created DANCE checklist, to be completed by caregivers, may help diagnose additional conditions that often occur alongside Dravet syndrome by identifying non-seizure features — such as language, cognitive, and behavioral problems — seen with this type of epilepsy, according to a pilot study that tested the use of this novel tool.
“This pilot study suggests that the DANCE checklist could be a useful screening tool in daily practice for neuropsychiatric comorbidities [co-existing conditions] facilitating their diagnosis and treatment, and empowering both caregivers and patients,” the researchers wrote, adding that “recognizing non-seizure outcomes is crucial for a holistic approach” to treating Dravet.
Details of the DANCE checklist — developed by Dravet Syndrome Foundation Spain researchers — were reported in a study titled “A tool for Dravet syndrome-associated neuropsychiatric comorbidities evaluation (DANCE),” which was published in Epilepsy & Behavior.
“The quantitative feedback results showed good-to-very good scores on usefulness, ease of completion, clarity and comprehensiveness of the checklist,” the team wrote.
DANCE checklist created to help ID Dravet features hidden by seizures
Dravet syndrome is a severe form of epilepsy marked by prolonged and frequent seizures starting in infancy. At the same time, other symptoms, such as developmental delays and behavioral problems, begin to emerge.
To diagnose Dravet, a complete clinical history is needed; this includes documenting when the seizures started, their frequency and duration, possible triggers, and observations of the patient’s ongoing development.
To aid in diagnoses, a team led by researchers at the Dravet Syndrome Foundation Spain in Madrid, developed a checklist called the DS-Associated Neuropsychiatric Comorbidities Evaluation, or DANCE. Its goal was to help identify non-seizure features of Dravet that may remain hidden by patients’ frequent seizures.
“The DANCE project seeks to develop a tool to help both the clinicians and the affected families to easily identify comorbidities,” the researchers wrote, noting that the checklist also can help healthcare providers “to know how these symptoms affect [the quality of life] of patients and caregivers.”
The DANCE project seeks to develop a tool to help both the clinicians and the affected families to easily identify comorbidities [co-occurring conditions]. … [This checklist also aims to help healthcare providers] to know how these symptoms affect [the quality of life] of patients and caregivers.
The team asked 24 caregivers of people with Dravet — from Belgium, France, and Spain — to complete the DANCE questionnaire. The patients’ ages ranged from 6 months to 24 years.
The results showed that two of the 23 caregivers with patients older than age 2 reported normal or fluent language abilities. In contrast, five reported that the patients in their care were nonverbal, and three said the patients for whom they cared said a few single words (less than 20). The majority, 13 caregivers, reported that the patients for whom they cared used “simple language.”
Of the 16 caregivers of patients with an intelligence quotient (IQ) or developmental quotient (DQ) evaluation/estimation, three said those in their care had normal intellectual abilities. All others had a lower IQ/DQ, ranging from mild to profound intellectual disability. A total of 15 of the 24 patients had a formal intelligence evaluation by a professional.
The researchers noted that one-quarter of parents, evenly spread across the three countries involved, reported that “they had received no professional support for treating cognitive symptoms” resulting from Dravet.
Attention and impulsivity problems among key challenges for caregivers
Attention difficulties and multitasking problems were reported by 18 of the 24 caregivers. Further, 17 caregivers reported impulsivity, and 16 reported troubles with repetitive behaviors, inflexibility, and visual and spatial awareness. Other behaviors common among patients included temper tantrums, reported by 14 caregivers, mood swings, as noted by 13, and autism, as reported by 12.
Among the 23 evaluable patients, four had complete mobility, 12 had some motor difficulties, four needed significant support, and three used a wheelchair. A total of 20 received physical therapy.
“Regarding the daily life of the patients, no [Dravet] patient was independent in self-care,” the researchers wrote.
The most common mobility impairments were balance and coordination problems, as reported by 21 caregivers, followed by ataxia, or lack of muscle control, which was reported by 14. Crouch walking was reported by nine caregivers, and parkinsonism, characterized by tremors, slow movements, and rigidity, was noted by seven.
Seven patients could eat independently, nine showed some eating skills, and eight depended on others. Most patients ate a normal amount of food but were very selective with the type of food they ate. Five caregivers reported swallowing difficulties for their patients.
While 16 patients did not have difficulties falling asleep, nine respondents said the patients in their care woke up early, with seven patients waking up because of seizures. One patient was reported to have sleep apnea. However, eight caregivers were unsure about sleep apnea, and just five patients had undergone a sleep study. Seven patients took sleep medication.
Study finds ‘considerable stress’ for family members of Dravet patients
According to the researchers, this study “also highlighted the considerable stress experienced by siblings and parents … of [a] family member with [Dravet].”
Significant relationship difficulties tied to high levels of stress between parents were reported by 18 caregivers, and 14 reported stress within the family. The lives of 19 siblings were reported to have “completely changed.” Among the 24 caregivers, 22 said that family life was significantly altered because of Dravet, marked by changes in parents’ work, leisure, and social time.
On a scale from 0 to 10, caregivers’ most significant concerns comprised the patients’ behavior, cognition, daily life, and family, with mean scores ranging from 8.29 to 8.75. Motor skills, eating, and sleeping were deemed somewhat less important, with mean scores between 6.38 and 7.63.
Overall, the researchers noted that the caregivers’ mean concern scores ranged from 5 to 10, “confirming that [Dravet] imposes a significant burden on caregivers.”
Lastly, caregivers were asked to give feedback on the DANCE checklist, using a scale of 1 to 5. Comprehensiveness had a mean score of 4.25, clarity reached 4.04, ease of completion achieved 4.46, and usefulness scored 4.47.
“We anticipate that the questionnaire will help [in] identifying distinctive [Dravet] comorbidities patterns, facilitating a more objective process of diagnosis,” the researchers concluded. “Additionally, the use of this tool could facilitate prompt interventions, reducing the societal and economic impact of the disease.”
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