The newly created DANCE checklist, designed to be completed by caregivers, may help diagnose additional conditions that often co-occur with Dravet syndrome by identifying non-seizure features – such as language, cognitive and behavioral problems – that occur in this type of epilepsy, according to a pilot study testing the use of this new tool.

“This pilot study suggests that the DANCE checklist could be a useful screening tool for neuropsychiatric comorbidities (comorbidities) in daily practice, facilitating their diagnosis and treatment and allowing more freedom of action for both caregivers and patients,” the researchers wrote, adding that “detecting non-seizure sequelae is crucial for a holistic approach” to treating Dravet.

Details of the DANCE checklist – developed by researchers at the Spanish Dravet Syndrome Foundation – were published in a study entitled “A tool for assessing neuropsychiatric comorbidities associated with Dravet syndrome (DANCE)”, published in Epilepsy and behavior.

“The results of the quantitative feedback showed good to very good ratings regarding usefulness, ease of completion, clarity and completeness of the checklist,” the team wrote.

DANCE checklist is designed to help identify features of Dravet syndrome hidden by seizures

Dravet syndrome is a severe form of epilepsy characterized by prolonged and frequent seizures that begin in childhood, along with other symptoms such as developmental delays and behavioral problems.

A complete medical history is required to diagnose Dravet. This includes documenting when the seizures began, their frequency and duration, possible triggers, and monitoring the patient’s further development.

To facilitate diagnosis, a team led by researchers at the Dravet Syndrome Foundation Spain in Madrid developed a checklist called DS-Associated Neuropsychiatric Comorbidities Evaluation (DANCE). The aim was to identify non-seizure features of Dravet syndrome that may be hidden by patients’ frequent seizures.

“The DANCE project aims to develop a tool that will help both clinicians and affected families to easily identify comorbidities,” the researchers write, noting that the checklist can also help healthcare providers “understand how these symptoms affect the quality of life of patients and caregivers.”

The aim of the DANCE project is to develop a tool that will help both clinicians and affected families to more easily identify comorbidities (accompanying diseases). … (This checklist is also intended to help healthcare providers recognize how these symptoms affect the quality of life of patients and caregivers.

The team asked 24 caregivers of people with Dravet syndrome – from Belgium, France and Spain – to complete the DANCE questionnaire. The patients’ ages ranged from 6 months to 24 years.

Results showed that two of the 23 caregivers with patients over 2 years of age reported normal or fluent language skills. In contrast, five reported that the patients in their care could not speak, and three said that the patients they cared for said few single words (less than 20). The majority, 13 caregivers, reported that the patients they cared for used “simple language.”

Of the 16 caregivers of patients with an assessment/estimate of intelligence quotient (IQ) or developmental quotient (DQ), three said the individuals they cared for had normal intellectual abilities. All others had lower IQ/DQ, ranging from mild to severe intellectual disability. A total of 15 of the 24 patients underwent a formal intelligence assessment by a professional.

The researchers found that a quarter of parents, evenly distributed across the three countries involved, reported receiving “no professional support in managing cognitive symptoms” as a result of Dravet.

Attention and impulsivity problems are among the biggest challenges for caregivers

18 of the 24 caregivers reported attention difficulties and multitasking problems. In addition, 17 caregivers reported impulsivity and 16 reported problems with repetitive behavior, lack of flexibility, and visual and spatial perception. Other common patient behaviors included temper tantrums, reported by 14 caregivers, mood swings, reported by 13, and autism, reported by 12 caregivers.

Of the 23 evaluable patients, four were fully mobile, 12 had some motor difficulties, four required significant assistance, and three were wheelchair-bound. A total of 20 received physical therapy.

“In terms of patients’ daily routine, no (Dravet) patient was independent in self-care,” the researchers wrote.

The most common mobility impairments were balance and coordination problems, reported by 21 caregivers, followed by ataxia or lack of muscle control, reported by 14. Nine caregivers reported crouched walking and seven reported parkinsonism, which is characterized by tremors, slow movements and rigidity.

Seven patients were able to eat independently, nine demonstrated some eating skills, and eight were dependent on others. Most patients ate a normal amount but were very selective about the type of food they ate. Five caregivers reported difficulty swallowing in their patients.

While 16 patients had no difficulty falling asleep, nine respondents reported that patients in their care woke up early, and seven patients woke up due to seizures. Sleep apnea was reported in one patient, but eight caregivers were unsure if it was sleep apnea, and only five patients had undergone a sleep study. Seven patients were taking sleeping medication.

Study finds “significant stress” for family members of Dravet patients

According to the researchers, this study also highlighted the significant stress faced by siblings and parents … of a family member with (Dravet).”

18 caregivers reported significant relationship difficulties due to high levels of stress between parents, and 14 reported stress within the family. The lives of 19 siblings were said to have been “completely changed.” Of the 24 caregivers, 22 said family life had changed significantly due to Dravet, characterized by changes in parents’ work, leisure and free time.

On a scale of 0 to 10, the most important concerns of the caregivers included the behavior, cognitive abilities, daily life and family of the patients, with average scores ranging from 8.29 to 8.75. Motor skills, eating and sleeping were considered slightly less important, with average scores ranging from 6.38 to 7.63.

Overall, the researchers found that caregivers’ average concern scores ranged from 5 to 10, “confirming that (Dravet) places a significant burden on caregivers.”

Finally, the caregivers were asked to provide feedback on the DANCE checklist on a scale of 1 to 5. The average score for completeness was 4.25, for understandability 4.04, for ease of completion 4.46, and for usefulness 4.47.

“We expect that the questionnaire will help identify characteristic (Dravet) comorbidity patterns and thus enable a more objective diagnostic process,” the researchers conclude. “In addition, the use of this tool could enable rapid intervention and thus reduce the social and economic impact of the disease.”