Introduction: Unverricht-Lundborg disease (ULD) is the most prevalent form of progressive myoclonic epilepsy. The aim of the study was to describe the EEG characteristics of patients with ULD and their changes during the long-term follow-up. Methods: We retrospectively evaluated the EEG features of 7 patients with genetically confirmed ULD. Results: We included 2 females and 5 males with the mean age 36.2±6.5 years, the mean age at onset 8.9 years and the lenght of follow-up 1-18 years. Thirty-one EEGs were performed in 7 patients. 27 (87%) EEGs were performed during treatment with borad spectrum antiepileptic drugs. The average background rhythm was 8.1 Hz and was normal in one patients and mildly slowed in 6. The frequency of background activity remained stable over time. Spike/polyspike and wave discharges (SWD) were found in all patients in at least one recording and in 22 EEGs (70.1%). Photosensitivity was found in all patients and in 15 recordings (48.3%). Myoclonic jerks were recorded in all subjects, most of the jerks were not time-locked to epileptiform discharges. In all patients jerks were provoked by photostimulation. In 2 patients SWD and myoclonic jerks were aggravated by contraindicated drugs (phenytoin and carbamazepine) and their frequency diminished after treatment change. Despite the increased frequency and severity of myoclonic jerks the frequency of SWD tended to diminish with time. Conclusion: The most common EEG abnormalities in patients with ULD include background slowing and spike and wave discharges. The average background rhythm remains stable over time; epileptiform discharges tend to diminish with time.
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