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Ninety\u2010seven (77.6%, 97\/125) patients had experienced seizures. The seizures onset age was 36.5\u2009\u00b1\u20099.0\u2009years, which was 6.9\u2009years later than cortical tremors. The seizures were largely rare (&lt;1\/year, 58.8%) and occasional (1\u20136\/year, 37.1%). Prolonged prodromes were reported in 57.7% (56\/97). Thirty\u2010one patients (24.8%, 31\/125) reported photosensitivity history, and 79.5% (31\/39) had a photoparoxysmal response. Interictal epileptiform discharges (IEDs) were recorded in 69.1% (56\/81) of patients. Thirty\u2010three patients showed generalized IEDs and 72.7% (24\/33) were occipitally dominant, while 23 patients presented with focal IEDs with 65.2% (15\/23) taking place over the occipital lobe. Overnight EEG of FCMTE patients displayed paradoxical sleep\u2013wake fluctuation, with a higher average IED index of 0.82\u2009\u00b1\u20090.88\/min during wakefulness and a lower IED index of 0.04\u2009\u00b1\u20090.06\/min during non\u2010rapid eye movement sleep stages I\u2013II.<\/jats:p><\/jats:sec><jats:sec><jats:title>Interpretation<\/jats:title><jats:p>FCMTE type 1 has a benign course of epilepsy and distinct clinical and electrophysiological features. In addition to a positive family history and cortical myoclonus tremor, the seizure prodromes, specific seizure triggers, photosensitivity, distribution of IEDs, and unique fluctuations during sleep\u2013wake cycle are cues for proper genetic testing and an early diagnosis of FCMTE.<\/jats:p><\/jats:sec>","DOI":"10.1002\/acn3.51961","type":"journal-article","created":{"date-parts":[[2023,12,8]],"date-time":"2023-12-08T03:29:09Z","timestamp":1702006149000},"page":"414-423","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Seizures and electrophysiological features in familial cortical myoclonic tremor with epilepsy 1"],"prefix":"10.1002","volume":"11","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0599-8872","authenticated-orcid":false,"given":"Yao","family":"Ding","sequence":"first","affiliation":[{"name":"Department of Neurology Second Affiliated Hospital, School of Medicine, Zhejiang University  Hangzhou 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