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While both disorders may share overlapping clinical features, their pathophysiology remain distinct and incompletely understood. Advanced resting-state electroencephalogram (rsEEG) analysis is an established methodology to assess cortical dynamics and network dysfunction in brain disorders.\u00a0We applied advanced rsEEG analysis to identify disease-specific electrophysiological patterns in\n                    <jats:italic>CACNA1A-<\/jats:italic>\n                    and GAA-\n                    <jats:italic>FGF14<\/jats:italic>\n                    -related diseases. Routine scalp EEG examinations from genetically confirmed patients were retrospectively collected at the Department of Neurology of the Medical University Innsbruck. EEGs from matched healthy controls were retrieved from a publicly available database. Using a Bayesian hierarchical modeling framework, we analyzed spectral bandpower and functional connectivity metrics.\u00a0Compared to healthy controls,\n                    <jats:italic>CACNA1A<\/jats:italic>\n                    patients (\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u200929) exhibited significantly increased theta-band power and reduced alpha peak frequency across all brain regions. Additionally, they showed enhanced functional connectivity in both the delta\/theta and gamma frequency bands. In contrast, findings in the GAA-\n                    <jats:italic>FGF14<\/jats:italic>\n                    \u2013related group (\n                    <jats:italic>n<\/jats:italic>\n                    \u2009=\u200915) largely overlapped with those of healthy controls, with only mild alterations characterized by increased beta power in posterior regions and a hyperconnectivity pattern in the alpha band.\u00a0\n                    <jats:italic>CACNA1A<\/jats:italic>\n                    -related disease is associated with widespread cortical network dysfunction, aligning with the clinical observation of frequent cognitive and neuropsychiatric symptoms\u2014unlike the pure motor presentation seen in GAA-\n                    <jats:italic>FGF14<\/jats:italic>\n                    \u2013related disease. Advanced rsEEG analysis allows for the non-invasive and repeatable detection and quantification of these alterations, holding promise for the development of surrogate markers for rare channelopathies.\n                  <\/jats:p>","DOI":"10.1007\/s12311-025-01924-w","type":"journal-article","created":{"date-parts":[[2025,11,15]],"date-time":"2025-11-15T04:23:44Z","timestamp":1763180624000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Resting-State EEG Analysis Characterizes the Signature of CACNA1A-and GAA-FGF14-Related Channelopathies"],"prefix":"10.1007","volume":"24","author":[{"given":"Raphael","family":"Angerbauer","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Iris","family":"Unterberger","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Wolfgang","family":"Nachbauer","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Matthias","family":"Amprosi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sylvia","family":"Boesch","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6554-1033","authenticated-orcid":false,"given":"Matteo","family":"Cesari","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0217-8630","authenticated-orcid":false,"given":"Elisabetta","family":"Indelicato","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2025,11,15]]},"reference":[{"key":"1924_CR1","doi-asserted-by":"crossref","unstructured":"Coutelier M, Coarelli G, Monin ML, Konop J, Davoine CS, Tesson C et al. 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