Key result
Patients with ring chromosome 20 syndrome exhibited atypical absence status triggered by emotional behavior, with ictal EEG characterized by bifrontal slow or sharp waves.
Case Report (n=3)
Highlights the characteristic features of non-convulsive status in ring chromosome 20 syndrome using video-EEG.
May aid recognition of emotion-triggered absence status in ring chromosome 20 syndrome; leaves open validation in larger cohorts.
We report the clinical history and the video-EEG recordings of three young adults with r(20), who exhibited repeated episodes of non-convulsive status epilepticus, often triggered by emotional events. This recognizable clinical entity associates a slight mental deficiency, behavioural disorder and epilepsy, often resistant to treatment and with a particular clinical presentation. We demonstrate with video EEG recording, atypical absence status triggered by emotional behaviour with ictal EEG characterized by bifrontal slow waves, often rhythmic, or sharp waves. Our report highlights the characteristic features of non-convulsive status in this electro-clinical syndrome.
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Petit et al. (1999) conducted a case report in Ring chromosome 20 syndrome (n=3). Ring chromosome 20 syndrome was evaluated on Clinical and EEG characteristics of non-convulsive status epilepticus. Patients with ring chromosome 20 syndrome exhibited atypical absence status triggered by emotional behavior, with ictal EEG characterized by bifrontal slow or sharp waves.
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