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The causal factor is a pathologically expanded CAG repeat in the Huntingtin gene. Age at onset is inversely correlated with CAG repeat length. Juvenile\u2010onset patients have distinct symptoms and signs with more severe pathology of involved brain structures in comparison with disease onset in adulthood. The aim of this review is to compare clinical and pathological features in juvenile\u2010 and adult\u2010onset Huntington disease and to explore which processes potentially contribute to the observed differences. A specific focus is placed on molecular mechanisms of mutant huntingtin in early neurodevelopment and the interaction of a neurodegenerative disease and postnatal brain maturation. The importance of a better understanding of pathophysiological differences between juvenile\u2010 and adult\u2010onset Huntington disease lies in development and implementation of new therapeutic strategies. \u00a9 2021 The Authors.\n                    <jats:italic>Movement Disorders<\/jats:italic>\n                    published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society\n                  <\/jats:p>","DOI":"10.1002\/mds.28823","type":"journal-article","created":{"date-parts":[[2021,10,15]],"date-time":"2021-10-15T21:03:31Z","timestamp":1634331811000},"page":"16-24","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":88,"title":["Juvenile\u2010Onset Huntington Disease Pathophysiology and Neurodevelopment: A Review"],"prefix":"10.1002","volume":"37","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8469-043X","authenticated-orcid":false,"given":"Hannah S.","family":"Bakels","sequence":"first","affiliation":[{"name":"Department of Neurology Leiden University Medical Center  Leiden the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Raymund A.C.","family":"Roos","sequence":"additional","affiliation":[{"name":"Department of Neurology Leiden University Medical Center  Leiden the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Willeke M.C.","family":"van Roon\u2010Mom","sequence":"additional","affiliation":[{"name":"Department of Human Genetics Leiden University Medical Center  Leiden the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Susanne T.","family":"de Bot","sequence":"additional","affiliation":[{"name":"Department of Neurology Leiden University Medical Center  Leiden the Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2021,10,12]]},"reference":[{"key":"e_1_2_11_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/0092-8674(93)90585-E"},{"key":"e_1_2_11_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.mad.2005.09.012"},{"key":"e_1_2_11_4_1","doi-asserted-by":"publisher","DOI":"10.1034\/j.1399-0004.2003.00108.x"},{"key":"e_1_2_11_5_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1445-5994.1998.tb02075.x"},{"key":"e_1_2_11_6_1","doi-asserted-by":"publisher","DOI":"10.1186\/1750-1172-5-40"},{"key":"e_1_2_11_7_1","doi-asserted-by":"publisher","DOI":"10.1371\/4f8606b742ef3"},{"key":"e_1_2_11_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/mds.27640"},{"key":"e_1_2_11_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(18)30294-1"},{"key":"e_1_2_11_10_1","doi-asserted-by":"publisher","DOI":"10.1093\/med\/9780199236121.001.0001"},{"key":"e_1_2_11_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(84)92510-8"},{"key":"e_1_2_11_12_1","doi-asserted-by":"publisher","DOI":"10.2310\/7010.2006.00055"},{"key":"e_1_2_11_13_1","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.64.6.813"},{"key":"e_1_2_11_14_1","doi-asserted-by":"publisher","DOI":"10.3390\/brainsci10060340"},{"key":"e_1_2_11_15_1","volume-title":"Juvenile Huntington Disease and Other Trinucleotide Repeat Disorders. 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