Non-convulsive status in the ring chromosome 20 syndrome: a video illustration of 3 cases.
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.
Petit
J
J
Centre médical de la Teppe, Hermitage, France. jepetit@caramail.com
Roubertie
A
A
Inoue
Y
Y
Genton
P
P
D000328:Adult; D001931:Brain Mapping; D002890:Chromosomes, Human, Pair 20; D004200:Diseases in Twins; D004569:Electroencephalography; D004644:Emotions / Q000502:physiology; D005260:Female; D005625:Frontal Lobe / Q000503:physiopathology; D006801:Humans; D007621:Karyotyping; D012303:Ring Chromosomes; D013226:Status Epilepticus / Q000175:diagnosis / Q000235:genetics* / Q000503:physiopathology; D013577:Syndrome; D014429:Twins, Dizygotic; D014741:Video Recording