{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,13]],"date-time":"2025-11-13T07:59:20Z","timestamp":1763020760726,"version":"3.45.0"},"reference-count":17,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2025,9,2]],"date-time":"2025-09-02T00:00:00Z","timestamp":1756771200000},"content-version":"vor","delay-in-days":244,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Intl J of Devlp Neuroscience"],"published-print":{"date-parts":[[2025,1]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>Niemann\u2010Pick disease type C (NPC) is a lysosomal storage disorder characterized by progressive neurological deterioration. Although there is no curative treatment, early initiation of miglustat, prior to significant neurological decline, may slow disease progression.<\/jats:p>\n                  <jats:p>This case report describes a patient whose initial symptoms emerged around age 9 and who was diagnosed with NPC at age 14 following gradual neurological decline. Despite being treated with miglustat, the patient's neurological symptoms worsened significantly. A high\u2010ratio ketogenic diet (KD), based on fat to protein plus carbohydrate, was initiated as an adjunctive therapy. Following dietary modification, neurological findings stabilized. An initially abnormal electroencephalogram (EEG) normalized during follow\u2010up. Due to significant weight loss, financial constraints and adherence difficulties, the KD ratio was reduced to 1:1 by the sixth month. At the time of the last evaluation, the patient had been maintained on the low\u2010ratio KD for 4\u2009years with continued adherence. The ketogenic diet is known to offer therapeutic benefits in various neurological disorders. In NPC patients treated with miglustat\u2014particularly those experiencing seizures\u2014a ketogenic diet may support neurological stabilization. A lower ratio KD may enhance long\u2010term compliance.<\/jats:p>","DOI":"10.1002\/jdn.70052","type":"journal-article","created":{"date-parts":[[2025,9,3]],"date-time":"2025-09-03T04:42:02Z","timestamp":1756874522000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Ketogenic Diet in Niemann\u2010Pick Type C: Insights From a Case Report"],"prefix":"10.1002","volume":"85","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2147-9316","authenticated-orcid":false,"given":"Bahar","family":"Kulu","sequence":"first","affiliation":[{"name":"Department of Inherited Metabolic Disorders Dokuz Eylul University Faculty of Medicine  Izmir Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6481-3172","authenticated-orcid":false,"given":"Pelin\u00a0Teke","family":"K\u0131sa","sequence":"additional","affiliation":[{"name":"Department of Inherited Metabolic Disorders Dokuz Eylul University Faculty of Medicine  Izmir Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8867-0508","authenticated-orcid":false,"given":"Esra","family":"Er","sequence":"additional","affiliation":[{"name":"Department of Inherited Metabolic Disorders Dr. Beh\u00e7et Uz Children's Research and Training Hospital  \u0130zmir Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1686-7536","authenticated-orcid":false,"given":"Serdar","family":"Pekuz","sequence":"additional","affiliation":[{"name":"Pediatric Neurology Department, Health Sciences University Dr. Beh\u00e7et Uz Children's Education and Research Hospital  Izmir Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2865-4810","authenticated-orcid":false,"given":"Zeynep","family":"Akisin","sequence":"additional","affiliation":[{"name":"Department of Nutrition and Dietitian Dr. Beh\u00e7et Uz Children's Diseases Education and Research Hospital  \u0130zmir Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2786-1911","authenticated-orcid":false,"given":"Serdar","family":"Ceylaner","sequence":"additional","affiliation":[{"name":"Intergen Genetic Diagnosis and Research Center  Ankara Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3151-3741","authenticated-orcid":false,"given":"Nur","family":"Arslan","sequence":"additional","affiliation":[{"name":"Department of Inherited Metabolic Disorders Dokuz Eylul University Faculty of Medicine  Izmir Turkey"},{"name":"\u0130zmir Biomedicine and Genome Center  \u0130zmir Turkey"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2025,9,2]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10545-011-9368-7"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.pediatrneurol.2017.12.014"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.3390\/ijms22126600"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ymgme.2021.11.008"},{"issue":"4","key":"e_1_2_9_6_1","first-page":"255","article-title":"How Does the Ketogenic Diet Work? Four Potential Mechanisms","volume":"83","author":"Danial N. 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