{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,27]],"date-time":"2026-04-27T04:38:50Z","timestamp":1777264730390,"version":"3.51.4"},"reference-count":25,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2026,4,26]],"date-time":"2026-04-26T00:00:00Z","timestamp":1777161600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"},{"start":{"date-parts":[[2026,4,26]],"date-time":"2026-04-26T00:00:00Z","timestamp":1777161600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Clinical Case Reports"],"published-print":{"date-parts":[[2026,5]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>Hypertrophic olivary degeneration (HOD) is a rare form of transsynaptic degeneration resulting from disruption of the Guillain\u2013Mollaret triangle, typically presenting with palatal tremor, ataxia, and nystagmus. Mutations in the Ring Finger Protein 170 (RNF170) gene have been associated with autosomal dominant sensory ataxia. However, the coexistence of HOD and an RNF170 mutation has rarely been reported. A 60\u2010year\u2010old female presented with progressive gait instability, vertigo, oscillopsia, and a sensation of walking on cotton. Neurological examination revealed pendular nystagmus, slow rhythmic palatal myoclonus, broad\u2010based gait, positive Romberg sign, and clumsy tandem gait. Cognitive function was intact. Magnetic resonance imaging showed bilateral hypertrophy of the inferior olivary nuclei with T2 hyperintensity, consistent with HOD, along with mild right cerebellar atrophy. No brainstem infarction, hemorrhage, or demyelinating lesions were identified. Genetic testing revealed a heterozygous mutation in the RNF170 gene. There was no family history of ataxia, and screening for common spinocerebellar ataxia genes was negative. This case describes an unusual presentation of HOD without an identifiable secondary structural lesion, occurring in a patient with an RNF170 mutation. The findings suggest that RNF170\u2010related neurodegeneration may predispose to or mimic HOD. In patients with unexplained HOD and prominent ataxia, screening for RNF170 gene mutations should be considered.<\/jats:p>","DOI":"10.1002\/ccr3.72590","type":"journal-article","created":{"date-parts":[[2026,4,27]],"date-time":"2026-04-27T03:56:26Z","timestamp":1777262186000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Mutation in\n                    <scp>RNF170<\/scp>\n                    Causes Unsteady Gait with Hypertrophic Olivary Degeneration"],"prefix":"10.1002","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0009-0007-8255-8196","authenticated-orcid":false,"given":"Change","family":"Wang","sequence":"first","affiliation":[{"name":"Clinical College of Neurology, Neurosurgery and Neurorehabilitation, Tianjin Medical University  Tianjin China"},{"name":"Department of Neurology Tianjin Huanhu Hospital, Tianjin Key Laboratory of Cerebral Vascular and Neurodegenerative Diseases, Tianjin Neurosurgical Institute  Tianjin China"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2026,4,26]]},"reference":[{"key":"e_1_2_12_2_1","doi-asserted-by":"publisher","DOI":"10.1148\/radiology.192.2.8029428"},{"key":"e_1_2_12_3_1","doi-asserted-by":"publisher","DOI":"10.5334\/tohm.188"},{"key":"e_1_2_12_4_1","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awf126"},{"issue":"5","key":"e_1_2_12_5_1","doi-asserted-by":"crossref","first-page":"2736","DOI":"10.1152\/jn.1997.77.5.2736","article-title":"Low\u2010Amplitude Oscillations in the Inferior Olive: A Model Based on Electrical Coupling of Neurons With Heterogeneous Channel Densities","volume":"77","author":"Manor Y.","year":"1997","journal-title":"Journal of Neurophysiology"},{"key":"e_1_2_12_6_1","doi-asserted-by":"publisher","DOI":"10.1007\/s10048\u2010021\u201000662\u20105"},{"key":"e_1_2_12_7_1","doi-asserted-by":"publisher","DOI":"10.3389\/fneur.2017.00302"},{"issue":"1","key":"e_1_2_12_8_1","doi-asserted-by":"crossref","first-page":"137","DOI":"10.1017\/cjn.2018.375","article-title":"An Acute Clinical Presentation Associated With Hypertrophic Olivary Degeneration","volume":"46","author":"Howard C.","year":"2019","journal-title":"Canadian Journal of Neurological Sciences"},{"issue":"5","key":"e_1_2_12_9_1","doi-asserted-by":"crossref","first-page":"301","DOI":"10.1590\/0004-282x20200021","article-title":"Transaxonal Degenerations of Cerebellar Connections: The Value of Anatomical Knowledge","volume":"78","author":"Raeder M. 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