Long-term evolution of EEG in Unverricht-Lundborg disease

Summary Objectives To describe the EEG characteristics of patients with Unverricht-Lundborg disease (ULD) and their changes during the long-term evolution of the disease. Methods A retrospective study including all patients with ULD confirmed by molecular biology and more than 15 years’ duration of...

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Bibliographic Details
Published in:Neurophysiologie clinique 2016-04, Vol.46 (2), p.119-124
Main Authors: Gargouri-Berrechid, Amina, Nasri, Amina, Kacem, Imen, Sidhom, Youssef, Abdelkefi, Istabrak, Hizem, Yosr, Ben Djebrara, Mouna, Gouider, Riadh
Format: Article
Language:English
Subjects:
Adult
Anticonvulsants - therapeutic use
Cerebral Cortex - physiopathology
Disease Progression
Electroencephalography
Epilepsy
Female
Humans
Long-term evolution
Maladie d’Unverricht-Lundborg
Male
Middle Aged
Myoclonie
Myoclonus
Neurology
Retrospective Studies
Unverricht-Lundborg disease
Unverricht-Lundborg Syndrome - diagnosis
Unverricht-Lundborg Syndrome - drug therapy
Unverricht-Lundborg Syndrome - physiopathology
Électroencéphalographie
Épilepsie
Évolution à long terme
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Summary:Summary Objectives To describe the EEG characteristics of patients with Unverricht-Lundborg disease (ULD) and their changes during the long-term evolution of the disease. Methods A retrospective study including all patients with ULD confirmed by molecular biology and more than 15 years’ duration of disease progression at the time of inclusion. EEGs were recorded at inclusion, 2 years and 5 years of follow-up. Patients who discontinued treatment during follow-up had an EEG monitoring 1 year after reintroduction of therapy. Results Forty-seven EEGs were performed in 17 patients. The mean age at onset was 12.0 ± 5.5 years. The mean duration of follow-up was 26.5 ± 6.9 years. The average background rhythm was 8.2 c/s, and was normal in 30 EEGs (64%), slow in 17 (36%) and disorganized in 11 (23%). Epileptic abnormalities were found in 22 EEGs (47%). Myoclonic jerks were found in 13 EEGs (28%). After re-adaptation of antiepileptic medication in patients who had previously stopped treatment, control EEG showed a normal background rhythm with no epileptic abnormalities throughout the monitoring period. Conclusion This study shows that the progressive disappearance of EEG abnormalities is rather due to antiepileptic treatment than a gradual spontaneous tendency to decrease over time.
ISSN:0987-7053
1769-7131
DOI:10.1016/j.neucli.2016.03.003