Impact Beyond Seizures
Most patients have medically intractable seizures and follow a course of developmental delay with lifelong cognitive impairment and motor function deficits.1-4
DEVELOPMENTAL DELAYS
Correlation between ongoing seizure activity and cognitive impairment underscores the importance of optimizing seizure control to improve developmental outcomes.5
Despite significant interpatient variability, the course of the disease has been described as generally progressing through 3 phases1,2,6
LEARNING DISABILITY
The magnitude of cognitive impairment is related to seizure frequency.3 Virtually all patients experience moderate to severe learning disability by the time they reach adulthood.3,7
- 6 in 10 patients had mild to severe learning disability in the second year of life
- Nearly 9 in 10 had mild to severe learning disability by the third to fourth year of life
- Severe to profound learning disability was seen in the majority of patients by the time they reached adolescence
Results from a study of 355 patients with Dravet syndrome in which clinicians rated the progression of learning disability.7
PERCENTAGE OF PATIENTS WITH LEARNING DIFFICULTIES3
There is an urgent need for continued research to help provide:
Improvements to reduce cognitive decline and long-term disability in patients
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References
- Dravet C. The core Dravet syndrome phenotype. Epilepsia. 2011;52(suppl 2):3-9. https://www.ncbi.nlm.nih.gov/pubmed/21463272
- Gataullina S, Dulac O. From genotype to phenotype in Dravet disease. Seizure. 2017;44:58-64. https://www.ncbi.nlm.nih.gov/pubmed/27817982
- Lagae L, Brambilla I, Mingorance A, Gibson E, Battersby A. Quality of life and comorbidities associated with Dravet syndrome severity: a multinational cohort survey. Dev Med Child Neurol. 2018;60(1):63-72. https://www.ncbi.nlm.nih.gov/pubmed/28984349
- Jensen MP, Brunklaus A, Dorris L, etc. The humanistic and economic burden of Dravet syndrome on caregivers and families: implications for future research. Epilepsy Behav. 2017;70(pt A):104-109. https://www.ncbi.nlm.nih.gov/pubmed/28431364
- O’Reilly H, Eltze C, Bennett K, et al. Cognitive outcomes following epilepsy in infancy: a longitudinal community-based study. Epilepsia. 2018;59(12):2240-2248. https://www.ncbi.nlm.nih.gov/pubmed/30395354
- Nolan KJ, Camfield CS, Camfield PR. Coping with Dravet syndrome: parental experiences with a catastrophic epilepsy. Dev Med Child Neurol. 2006;48(9):761-765. https://www.ncbi.nlm.nih.gov/pubmed/16904024
- Brunklaus A, Ellis R, Reavey E, Forbes GH, Zuberi SM. Prognostic, clinical and demographic features in SCN1A mutation-positive Dravet syndrome. Brain. 2012;135(pt 8):2329-2336. https://www.ncbi.nlm.nih.gov/pubmed/22719002