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Like many other chromosome syndromes, it is characterized by multiple congenital anomalies and developmental delays. Typical of the condition are retinal anomalies and drug\u2010resistant epilepsy. These latter manifestations are not found in individuals who are carriers of comparable 14q deletions without formation of a ring (linear deletions). To find an explanation for this apparent discrepancy and gain insight into the mechanisms leading to seizures, we reviewed and compared literature cases of both ring and linear deletion syndrome with respect to both their clinical manifestations and the role and function of potentially epileptogenic genes. Knowledge of the epilepsy\u2010related genes in chromosome 14 is an important premise for the search of new and effective drugs to combat seizures. Current clinical and molecular evidence is not sufficient to explain the known discrepancies between ring and linear deletions.<\/jats:p>","DOI":"10.1111\/epi.16754","type":"journal-article","created":{"date-parts":[[2020,11,18]],"date-time":"2020-11-18T08:32:52Z","timestamp":1605688372000},"page":"25-40","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":16,"title":["Chromosome 14 deletions, rings, and epilepsy genes: A riddle wrapped in a mystery inside an enigma"],"prefix":"10.1111","volume":"62","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8413-621X","authenticated-orcid":false,"given":"Alessandro","family":"Vaisfeld","sequence":"first","affiliation":[{"name":"Institute of Genomic Medicine Catholic University School of Medicine Rome Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7095-9968","authenticated-orcid":false,"given":"Serena","family":"Spartano","sequence":"additional","affiliation":[{"name":"Institute of Genomic Medicine Catholic University School of Medicine Rome Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Giuseppe","family":"Gobbi","sequence":"additional","affiliation":[{"name":"Residential Center for Rehabilitation Luce Sul Mare Rimini Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0573-6175","authenticated-orcid":false,"given":"Annamaria","family":"Vezzani","sequence":"additional","affiliation":[{"name":"Department of Neuroscience IRCCS\u2010Istituto di Ricerche Farmacologiche Mario Negri Milano Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7642-0130","authenticated-orcid":false,"given":"Giovanni","family":"Neri","sequence":"additional","affiliation":[{"name":"Institute of Genomic Medicine Catholic University School of Medicine Rome Italy"},{"name":"J.C. 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