A long‐term follow‐up study of Dravet syndrome up to adulthood
Open accessAbstract
Summary: Purpose: We intended to elucidate the whole clinical course of Dravet syndrome (DS) comprehensively, from infancy through adulthood. Methods: Subjects were 31 patients with DS (14 with typical DS, and 17 with borderline DS) who were followed from childhood to at least 18 years of age. Their seizures, abilities, and electroencephalography (EEG) findings were investigated and statistically analyzed. Results: The clinical findings of the patients with typical DS and those with borderline DS became largely similar in adolescence and adulthood. Seizures were intractable in childhood in all patients, but suppressed in five (16.1%) during follow‐up. Thirty‐five (87.5%) of the 40 apparently generalized convulsive seizures that were captured by ictal EEG recording at 7 years of age or later were of focal origin. The seizure‐free outcomes were significantly correlated with the experience of <3 episodes of convulsive status epilepticus, and also with disappearance of spikes on the follow‐up EEGs. Mental outcomes involving less severe intellectual disability were correlated with the presence of occipital alpha rhythms in the background activity of the follow‐up EEGs. Mean age at the recording of the follow‐up EEGs was 23.8 years. Discussion: Prevention of the occurrence of convulsive status epilepticus was indicated to be critically important for the improvement of seizure prognosis in DS.
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The established mortality rate for Dravet syndrome is that 17% of patients die by age 20 years.
“The established mortality rate for Dravet syndrome is that 17% of patients die by age 20 years 20 .”
From Developmental and epileptic encephalopathies (2024) · cites this paper as [20]
The ‘Patients who died before 18 years of age’ section reports that 6 of 37 patients (16.2%) died in childhood, all before age 13. This supports a mortality figure near 17% by age 20 for this cohort, but not an established rate for all patients with Dravet syndrome. The copy provides no page number for this passage. (LLM, conf=0.88)
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- Authors
- Akiyama, M., Kobayashi, K., Yoshinaga, H., & Ohtsuka, Y.
- Affiliations
- Okayama University (JP)
- Corresponding author
- Kobayashi, Katsuhiro
- Venue
- Epilepsia
- Publisher
- Wiley
- Published
- 2010-06
- Volume
- 51
- Issue
- 6
- Pages
- 1043-1052
- ISSN
- 0013-9580 (ISSN-L)
- 1528-1167
- OpenAlex
- W1602228332
- Cited by
- 192
- References
- 39
- Citations by year
- 2026: 1 · 2025: 2 · 2024: 8 · 2023: 8 · 2022: 14 · 2021: 20 · 2020: 38 · 2019: 11 · 2018: 7 · 2017: 13 · 2016: 11 · 2015: 8 · 2014: 12 · 2013: 11 · 2012: 10
- FWCI
- 5.75
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Epilepsia (publishedVersion)
- PubMed (publishedVersion)
- Okayama University Scientific Achievement Repository (Okayama University) (submittedVersion)
- Topics
- Health Sciences › Medicine › Psychiatry and Mental health › Epilepsy research and treatment
- Health Sciences › Medicine › Pediatrics, Perinatology and Child Health › Pharmacological Effects and Toxicity Studies
- Health Sciences › Medicine › Physiology › Diet and metabolism studies
- Keywords
- Dravet syndrome, clinical course, seizure outcome, convulsive status epilepticus, seizure prognosis, EEG background activity, occipital alpha rhythm, intellectual disability, refractory seizures, focal seizures, long-term follow-up
- MeSH terms
- Adolescent, Adult, Age Factors, Female, Follow-Up Studies, Humans, Male, Seizures, Syndrome, Time Factors, Treatment Outcome, Young Adult
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