{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,12,3]],"date-time":"2025-12-03T03:27:50Z","timestamp":1764732470464},"reference-count":15,"publisher":"Wiley","issue":"10","license":[{"start":{"date-parts":[[2012,3,14]],"date-time":"2012-03-14T00:00:00Z","timestamp":1331683200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Develop Med Child Neuro"],"published-print":{"date-parts":[[2012,10]]},"abstract":"<jats:p>At 7\u2003years of age, a female with mucopolysaccharidosis type II (MPS II) showed a sudden deterioration in neurological function, a sleep disorder, and progressive behavioural impairment. Electroencephalography was performed 1\u2003year and 8\u2003months after the onset of the neurological regression and revealed continuous ictal activity in the frontal regions. The female was diagnosed as having frontal non\u2010convulsive status epilepticus. After 5\u2003weeks of therapy with ethosuximide, the ictal electroencephalographic activity disappeared. At the same time, her sleep and cognitive and behavioural functions were observed to improve. This is the first case of MPS type II reported in an individual with non\u2010convulsive status epilepticus. Two main forms of MPS II can be recognized: attenuated and severe. Severe MPS II is characterized by neurodegeneration. No study has explored the relationship between epilepsy and neurological deterioration in MPS II. Our observation shows that epilepsy may be a treatable cause of neurological regression in individuals with MPS II.<\/jats:p>","DOI":"10.1111\/j.1469-8749.2012.04228.x","type":"journal-article","created":{"date-parts":[[2012,3,14]],"date-time":"2012-03-14T06:16:25Z","timestamp":1331705785000},"page":"961-964","update-policy":"http:\/\/dx.doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":13,"title":["Non\u2010convulsive status epilepticus of frontal origin in mucopolysaccharidosis type II successfully treated with ethosuximide"],"prefix":"10.1111","volume":"54","author":[{"given":"PAOLO","family":"BONANNI","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"MARCO","family":"GUBERNALE","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"FEDERICA","family":"MARTINEZ","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"GIOVANNA","family":"RANDAZZO","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"LUCA","family":"MILANTONI","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"ANDREA","family":"MARTINUZZI","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"CLEMENTINA","family":"BONIVER","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"MARILENA","family":"VECCHI","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"MAURIZIO","family":"SCARPA","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2012,3,14]]},"reference":[{"key":"e_1_2_6_2_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00431-007-0635-4"},{"key":"e_1_2_6_3_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.pediatrneurol.2004.10.009"},{"key":"e_1_2_6_4_2","first-page":"455","article-title":"The Hunter syndrome in females: is there an autosomal recessive form of iduronate sulfatase deficiency?","volume":"29","author":"Neufeld EF","year":"1977","journal-title":"Am J Hum Genet"},{"key":"e_1_2_6_5_2","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-009-9023-8"},{"key":"e_1_2_6_6_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1651-2227.2007.00212.x"},{"key":"e_1_2_6_7_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1469-8749.1983.tb13794.x"},{"key":"e_1_2_6_8_2","doi-asserted-by":"publisher","DOI":"10.1136\/jmg.19.6.408"},{"key":"e_1_2_6_9_2","first-page":"241","volume-title":"Aicardi\u2019s Epilepsy in Children","author":"Guerrini R","year":"2004"},{"key":"e_1_2_6_10_2","volume-title":"Stanford\u2013Binet Intelligence Scale","author":"Thorndike RL","year":"1986"},{"key":"e_1_2_6_11_2","volume-title":"Vineland Adaptive Behavior Scales. 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