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All of these seven patients exhibited status epilepticus and required transient respiratory care. Isolated diffuse cerebellar cortical atrophy was observed in two adult patients with mild motor regression. Five patients without apparent deterioration displayed almost normal brain findings.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>The areas of atrophy were consistent with the areas of increased expression of the Na<jats:sup>+<\/jats:sup>\/K<jats:sup>+<\/jats:sup>\u2010<jats:styled-content style=\"fixed-case\">ATP<\/jats:styled-content>ase \u03b13 subunit encoded by <jats:italic><jats:styled-content style=\"fixed-case\">ATP<\/jats:styled-content>1A3<\/jats:italic>. Some of paroxysmal and nonparoxysmal neurological symptoms are considered as related to the areas of brain atrophy.<\/jats:p><\/jats:sec>","DOI":"10.1002\/mdc3.12451","type":"journal-article","created":{"date-parts":[[2016,9,22]],"date-time":"2016-09-22T15:07:24Z","timestamp":1474556844000},"page":"406-411","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":24,"title":["Progressive Brain Atrophy in Alternating Hemiplegia of Childhood"],"prefix":"10.1002","volume":"4","author":[{"given":"Masayuki","family":"Sasaki","sequence":"first","affiliation":[{"name":"Department of Child Neurology National Center of Neurology and Psychiatry Kodaira Tokyo Japan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Atsushi","family":"Ishii","sequence":"additional","affiliation":[{"name":"Department of Pediatrics and Central Research Institute for the Molecular Pathomechanisms of Epilepsy Fukuoka University School of Medicine Fukuoka 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