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Current trials using thyroid hormone derivatives to overcome the transporter defect have failed to achieve patient\u2010oriented therapeutic goals.<\/jats:p><\/jats:sec><jats:sec><jats:title>Objectives<\/jats:title><jats:p>Our aim was to define the type of movement disorder in AHDS in an observational cohort study and to investigate the causative role of the dopaminergic system.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We present longitudinal clinical data from the DEEPTYPE registry of 11 patients with video documentation, standardized phenotyping, cerebrospinal fluid (CSF) analysis, neuroimaging data, and the treatment response to levodopa\/carbidopa supplementation.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Children presented with signs of childhood parkinsonism, including hypokinesia, hypomimia, inability to sit or stand, rigidity, dystonia, and autonomic dysfunction. CSF homovanillic acid concentrations were decreased (n\u2009=\u200912), suggesting an isolated dopamine pathway impairment. Seven out of 8 patients responded favorably to <jats:sc>l<\/jats:sc>\u2010dopa\/carbidopa supplementation and we did not observe any adverse drug reactions.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>AHDS is associated with childhood parkinsonism, which is linked with biochemical abnormalities of dopamine metabolism. It can be treated with <jats:sc>l<\/jats:sc>\u2010dopa\/carbidopa supplementation. However, further research is needed to elucidate the exact effect of MCT8 deficiency on dopamine metabolism. \u00a9 2025 The Author(s). <jats:italic>Movement Disorders<\/jats:italic> published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.<\/jats:p><\/jats:sec>","DOI":"10.1002\/mds.30152","type":"journal-article","created":{"date-parts":[[2025,3,15]],"date-time":"2025-03-15T06:14:17Z","timestamp":1742019257000},"page":"938-949","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Patients with Allan\u2010Herndon\u2010Dudley Syndrome (<scp>MCT8<\/scp> Deficiency) Display Symptoms of Parkinsonism in Childhood and Respond to Levodopa\/Carbidopa Treatment"],"prefix":"10.1002","volume":"40","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9453-8335","authenticated-orcid":false,"given":"Nina\u2010Maria","family":"Wilpert","sequence":"first","affiliation":[{"name":"Department of 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