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Published on: August 25, 2014
A tool for Dravet syndrome-associated neuropsychiatric comorbidities evaluation (DANCE)
Simona Giorgi1, Stéphane Auvin2, An-Sofie Schoonjans3
1Dravet Syndrome Foundation Spain, Madrid, Spain.
Insights
A new checklist helps identify neuropsychiatric issues in Dravet syndrome (DS) patients. This tool aids in early diagnosis and treatment of cognitive, behavioral, and motor challenges, improving care for affected families.
Area of Science:
- Neurology
- Genetics
- Psychiatry
Background:
- Dravet syndrome (DS) is a severe infant-onset epilepsy linked to SCN1A gene variants.
- DS involves drug-resistant seizures, developmental delays, and behavioral issues.
- Current diagnosis of comorbidities relies on clinical interviews and healthcare professional (HCP) experience.
Purpose of the Study:
- To develop and pilot a screening checklist for DS-Associated Neuropsychiatric Comorbidities (DANC).
- To assess the utility and refine the DANC checklist with input from HCPs and caregivers.
Main Methods:
- Expert HCPs and caregivers collaborated to create the DANC checklist.
- The checklist covers cognitive, psychiatric, motor domains, and family impact.
- 24 caregivers of DS patients completed the checklist; feedback was gathered for refinement.
Main Results:
- DS patients exhibit diverse neuropsychiatric symptoms, including attention deficits, impulsivity, temper tantrums, mood swings, and autism spectrum disorder.
- Motor impairments (balance, coordination) are prevalent, with most patients requiring assistance with self-care and exhibiting sleep disturbances.
- Caregivers reported significant stress, with behavior and cognition being primary concerns; the DANC checklist received positive feedback for usability.
Conclusions:
- The DANC checklist shows promise as a practical screening tool for DS neuropsychiatric comorbidities.
- It can facilitate earlier diagnosis and treatment, empowering patients and caregivers.
- Further validation could enhance clinical management of Dravet syndrome.
Background:
Dravet syndrome (DS) is a rare and severe form of epilepsy that begins in infancy, which is primarily caused by pathogenic variants in the SCN1A gene. DS is characterized by prolonged and frequent drug-resistant seizures, as well as developmental delays and behavioral problems. The identification of these comorbidities is based on clinical interview and relies on healthcare professionals (HCPs) experience.
Methods:
We assembled a group of expert HCPs and caregivers to create a screening checklist for assessing DS-Associated Neuropsychiatric Comorbidities (DANC). The checklist includes questions related to cognitive and psychiatric domains, motor skills, and the impact of DS on families' daily lives. We administered the checklist to 24 caregivers of DS patients from Belgium, France, and Spain. After piloting, we obtained feedback from expert HCPs and caregivers to refine the checklist.
Results:
DS patients showed a wide array of neuropsychiatric symptoms related to DS. The most common cognitive domains reported were attention difficulties and multitasking problems (18/24 caregivers), and impulsivity (17/24), while the most common psychiatric symptoms were temper tantrums (14/24), mood swings (13/24) and autism spectrum disorder (12/24). Balance and coordination problem have been reported in almost all patients with a statement of only 4/23 with complete mobility. Most patients were dependent on others for self-care and eating, and presented sleeping disturbances. Caregivers reported high levels of stress in the family unit, both between siblings and parents. Results show that the main concerns of parents were the behavior and the cognition of the person with DS. The quantitative feedback results showed good-to-very good scores on usefulness, ease of completion, clarity and comprehensiveness of the checklist.
Conclusions:
This pilot study suggests that the DANCE checklist could be a useful screening tool in daily practice for neuropsychiatric comorbidities facilitating their diagnosis and treatment, and empowering both caregivers and patients.
