{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,10]],"date-time":"2026-04-10T02:12:04Z","timestamp":1775787124517,"version":"3.50.1"},"reference-count":125,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2024,9,19]],"date-time":"2024-09-19T00:00:00Z","timestamp":1726704000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Cell. Neurosci."],"abstract":"<jats:p>Glucose is the brain\u2019s main fuel source, used in both energy and molecular production. Impaired glucose metabolism is associated with adult and pediatric neurodegenerative diseases such as Alzheimer\u2019s disease (AD), Parkinson\u2019s disease (PD), GLUT1 deficiency syndrome, and progressive myoclonus epilepsies (PMEs). PMEs, a group of neurological disorders typical of childhood and adolescence, account for 1% of all epileptic diseases in this population worldwide. Diffuse glucose hypometabolism is observed in the brains of patients affected by PMEs such as Lafora disease (LD), dentatorubral-pallidoluysian (DRPLA) atrophy, Unverricht\u2013Lundborg disease (ULD), and myoclonus epilepsy with ragged red fibers (MERRFs). PMEs also include neuronal ceroid lipofuscinoses (NCLs), a subgroup in which lysosomal and autophagy dysfunction leads to progressive loss of vision, brain atrophy, and cognitive decline. We examine the role of impaired glucose metabolism in neurodegenerative diseases, particularly in the NCLs. Our literature review, which includes findings from case reports and animal studies, reveals that glucose hypometabolism is still poorly characterized both <jats:italic>in vitro<\/jats:italic> and <jats:italic>in vivo<\/jats:italic> in the different NCLs. Better identification of the glucose metabolism pathway impaired in the NCLs may open new avenues for evaluating the therapeutic potential of anti-diabetic agents in this population and thus raise the prospect of a therapeutic approach able to delay or even halt disease progression.<\/jats:p>","DOI":"10.3389\/fncel.2024.1445003","type":"journal-article","created":{"date-parts":[[2024,9,19]],"date-time":"2024-09-19T10:41:50Z","timestamp":1726742510000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":3,"title":["Glucose metabolism impairment as a hallmark of progressive myoclonus epilepsies: a focus on neuronal ceroid lipofuscinoses"],"prefix":"10.3389","volume":"18","author":[{"given":"Lorenzo","family":"Santucci","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sara","family":"Bernardi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rachele","family":"Vivarelli","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Filippo Maria","family":"Santorelli","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maria","family":"Marchese","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2024,9,19]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","first-page":"1939","DOI":"10.1002\/glia.22575","article-title":"Glial influence on the blood brain barrier","volume":"61","author":"Alvarez","year":"2013","journal-title":"Glia"},{"key":"ref2","doi-asserted-by":"crossref","first-page":"693","DOI":"10.1002\/(SICI)1097-4547(19990901)57:5<693::AID-JNR11>3.0.CO;2-X","article-title":"Insulin-sensitive GLUT4 glucose transporters are Colocalized with GLUT3-expressing cells and demonstrate a chemically distinct neuron-specific localization in rat brain","volume":"57","author":"Apelt","year":"1999","journal-title":"J. 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