Children With Trisomy 21 and Lennox-Gastaut Syndrome With Predominant Myoclonic Seizures
| Autor: | Matthew Macdonald, Jacqueline Crawford, Anita N Datta |
|---|---|
| Rok vydání: | 2021 |
| Předmět: |
0301 basic medicine
Adult Male Pediatrics medicine.medical_specialty Down syndrome genetic structures Atypical absence seizures 03 medical and health sciences 0302 clinical medicine Refractory Myoclonic Seizures Seizures medicine Tonic (music) Humans Child Retrospective Studies business.industry Lennox Gastaut Syndrome Infant Electroencephalography medicine.disease Epileptic spasms 030104 developmental biology Child Preschool Pediatrics Perinatology and Child Health Female Neurology (clinical) Down Syndrome business Trisomy 030217 neurology & neurosurgery Lennox–Gastaut syndrome |
| Zdroj: | Journal of child neurology. 36(11) |
| ISSN: | 1708-8283 |
| Popis: | Introduction: Lennox-Gastaut syndrome is a severe form of pediatric epilepsy that is classically defined by a triad of drug-resistant seizures, including atonic, tonic, and atypical absence seizures; slow spike-and-wave discharges and paroxysmal fast activity on electroencephalography (EEG); and cognitive and behavioral dysfunction. In the vast majority, Lennox-Gastaut syndrome develops in patients with an identified etiology, including genetic or structural brain abnormalities. Long-term prognosis is generally poor with progressive intellectual deterioration and persistent seizures. At present, there are few reported cases of Lennox-Gastaut syndrome and trisomy 21 in the literature. To further delineate the spectrum of epilepsy in trisomy 21, we reviewed children with trisomy 21 and Lennox-Gastaut syndrome at one center over 28 years. Methods: This is a retrospective case series. At our institution, all EEG results are entered into a database, which was queried for patients with trisomy 21 from 1992 to 2019. Pertinent electroclinical data was obtained from medical records. Results: Of 63 patients with trisomy 21 and epilepsy, 6 (10%) had Lennox-Gastaut syndrome and were included in the study. Four of the 6 patients were male and 5 of 6 had neuroimaging, which was normal. Follow-up ranged from 3 to 20 years. Notably, 5 of 6 had predominant myoclonic seizures throughout the course of their epilepsy, associated with generalized spike-wave discharges, Conclusion: We observed myoclonic seizures to be a predominant seizure type in patients with trisomy 21, suggestive that trisomy 21 patients may have a unique pattern of Lennox-Gastaut syndrome. |
| Databáze: | OpenAIRE |
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