{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,9]],"date-time":"2026-10-09T17:20:45Z","timestamp":1791566445587,"version":"4.3.4"},"reference-count":35,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2025,5,8]],"date-time":"2025-05-08T00:00:00Z","timestamp":1746662400000},"content-version":"vor","delay-in-days":7,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Euro J of Neurology"],"published-print":{"date-parts":[[2025,5]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Cerebellar ataxia is associated with greater disability in glutamic acid decarboxylase\u201065 (GAD65) antibody\u2010associated neurological disorders and can occur in isolation or as part of stiff person syndrome (SPS) spectrum disorders (SPSD). We sought to phenotypically characterize this subpopulation with cerebellar dysfunction.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Observational study of GAD65 antibody\u2010seropositive individuals with cerebellar involvement seen at Johns Hopkins (1997\u20132024). Subjects were divided into two groups based on cerebellar dysfunction in the presence (SPSD; SPS\u2010plus and progressive encephalomyelitis with rigidity and myoclonus [PERM]) or absence (pure cerebellar ataxia [pCA]) of classic SPS features. Clinical and paraclinical findings were analyzed descriptively.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Seventy\u2010two patients were selected among 356 (62 SPSD, 10 pCA). Mean age for patients with pCA was 58\u2009\u00b1\u200916\u2009years versus 46\u2009\u00b1\u200915\u2009years for SPSD (<jats:italic>p<\/jats:italic>\u2009=\u20090.012). Males comprised 50% of the pCA group versus 19% SPSD (<jats:italic>p<\/jats:italic>\u2009=\u20090.049). High GAD65 antibody serum titers occurred in 76% without group differences, while cerebrospinal fluid antibody positivity occurred in 35\/37 (95%) of SPSD versus 5\/8 (62%) pCA (<jats:italic>p<\/jats:italic>\u2009=\u20090.033). Although the modified Rankin scale was similar (median 3, interquartile range 2\u20134) in both groups, the brief ataxia rating scale indicated a higher burden of cerebellar abnormalities in pCA versus SPSD, and there was a trend toward greater cerebellar atrophy by MRI in pCA (<jats:italic>p<\/jats:italic>\u2009=\u20090.44). Rituximab and benzodiazepine use was more frequent in SPSD versus pCA.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>GAD65 antibody\u2010associated ataxia is disabling irrespective of accompanying SPS features. Patients with pCA were older, more commonly male, and may have more frequent cerebellar atrophy than those with SPSD. Prospective validation of cerebellar outcomes and neuroimaging findings is needed.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ene.70166","type":"journal-article","created":{"date-parts":[[2025,5,8]],"date-time":"2025-05-08T07:29:25Z","timestamp":1746689365000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":4,"title":["Clinical Features and Outcomes of Glutamic Acid Decarboxylase\u201065 Antibody\u2010Associated Pure Cerebellar Ataxia and Stiff Person Syndrome Spectrum Disorders: A Single\u2010Center Cohort Study"],"prefix":"10.1111","volume":"32","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4964-3341","authenticated-orcid":false,"given":"Jonathan D.","family":"Krett","sequence":"first","affiliation":[{"name":"Department of Neurology Johns Hopkins University School of Medicine  Baltimore Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yujie","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Neurology Johns Hopkins University School of Medicine  Baltimore Maryland USA"},{"name":"Department of Neurology University of Washington Medical Center \u2013 Northwest  Seattle Washington USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ashley","family":"Miles","sequence":"additional","affiliation":[{"name":"Department of Neurology Johns Hopkins University School of Medicine  Baltimore Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Herbert R.","family":"Chen","sequence":"additional","affiliation":[{"name":"Department of Neurology Johns Hopkins University School of Medicine  Baltimore Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hanyeh","family":"Afshar","sequence":"additional","affiliation":[{"name":"Department of Neurology Johns Hopkins University School of Medicine  Baltimore Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Aisha","family":"Elfasi","sequence":"additional","affiliation":[{"name":"Department of Neurology Johns Hopkins University School of Medicine  Baltimore Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Doris D.","family":"Lin","sequence":"additional","affiliation":[{"name":"Department of Radiology and Radiological Science Johns Hopkins University School of Medicine  Baltimore Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Scott D.","family":"Newsome","sequence":"additional","affiliation":[{"name":"Department of Neurology Johns Hopkins University School of Medicine  Baltimore Maryland USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2025,5,8]]},"reference":[{"issue":"15","key":"e_1_2_8_2_1","first-page":"421","article-title":"Progressive Fluctuating Muscular Rigidity and Spasm (\u201cStiff\u2010Man\u201d Syndrome); Report of a Case and Some Observations in 13 Other Cases","volume":"38","author":"Moersch F. 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