Electroclinical Patterns and Evolution of Epilepsy in the 4p- Syndrome
| العنوان: | Electroclinical Patterns and Evolution of Epilepsy in the 4p- Syndrome |
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| المؤلفون: | Celeste Acquafondata, Domenica Battaglia, Isabella Vasta, Francesco Guzzetta, Charlotte Dravet, Donatella Lettori, Paolo Mariotti, Marcella Zollino, Giovanni Neri, Marika Pane, Giuseppe Zampino |
| المصدر: | Epilepsia. 44:1183-1190 |
| بيانات النشر: | Wiley, 2003. |
| سنة النشر: | 2003 |
| مصطلحات موضوعية: | Pediatrics, medicine.medical_specialty, Longitudinal study, medicine.diagnostic_test, Myoclonic Jerk, Electroencephalography, Neonatal age, medicine.disease, Surgery, Central nervous system disease, Epilepsy, Neurology, El Niño, medicine, Neurology (clinical), Psychology, Wolf–Hirschhorn syndrome |
| الوصف: | Summary: Background: Wolf–Hirschhorn syndrome (WHS) is a well-known clinical entity caused by partial deletion of the short arm of one chromosome 4 (4p– syndrome). Seizures occur in almost all the cases, but studies on the electroclinical disorder and its evolution are still scarce. We present a longitudinal study of the electroclinical features in 10 children with WHS. Methods: Ten patients (five boys and five girls) underwent a detailed clinical assessment and a prolonged EEG study. Six of the 10 also had video-polygraphy. Results: Nine of the 10 patients had seizures; they were generalized or unilateral clonic and tonic–clonic, and atypical absences associated with myoclonic jerks. Age at onset of seizures varied from 1 day to 2.5 years. In all the patients, including the only one without seizures, two stereotyped EEG patterns were observed, consisting of (a) bursts of rhythmic (3–5 Hz), high-voltage slow waves located in the posterior regions and increased by sleep, or bursts of rapid spike–wave complexes in the centroparietal and parietooccipital regions; and (b) repetitive rapid posterior spikes. Sleep organization was constantly absent or very poor. The evolution of epilepsy was frequently good, with four seizure-free cases at the end of follow-up, two of them weaned from antiepileptic drugs (AEDs). Conclusions: Seizure onset in WHS also can occur at neonatal age. At least two electrical stereotyped patterns of the epileptic disorder are associated with a relevant disorganization of the sleep states. Prognosis of epilepsy is generally good both for the seizure control and for its evolution. |
| تدمد: | 1528-1167 0013-9580 |
| URL الوصول: | https://explore.openaire.eu/search/publication?articleId=doi_________::9f0d7f956952ebffe70cc8a344a4720b https://doi.org/10.1046/j.1528-1157.2003.63502.x |
| حقوق: | OPEN |
| رقم الأكسشن: | edsair.doi...........9f0d7f956952ebffe70cc8a344a4720b |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 15281167 00139580 |
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