{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T02:22:23Z","timestamp":1760149343330,"version":"build-2065373602"},"reference-count":41,"publisher":"MDPI AG","issue":"3","license":[{"start":{"date-parts":[[2023,7,26]],"date-time":"2023-07-26T00:00:00Z","timestamp":1690329600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Research Committee of Prion Disease and Slow Virus Infection","award":["21K07450","22HA1003","JP20K07875"],"award-info":[{"award-number":["21K07450","22HA1003","JP20K07875"]}]},{"name":"Research on Policy Planning and Evaluation for Rare and Intractable Diseases","award":["21K07450","22HA1003","JP20K07875"],"award-info":[{"award-number":["21K07450","22HA1003","JP20K07875"]}]},{"name":"Health and Labour Sciences Research Grants","award":["21K07450","22HA1003","JP20K07875"],"award-info":[{"award-number":["21K07450","22HA1003","JP20K07875"]}]},{"name":"the Ministry of Health, Labour and Welfare","award":["21K07450","22HA1003","JP20K07875"],"award-info":[{"award-number":["21K07450","22HA1003","JP20K07875"]}]},{"DOI":"10.13039\/501100001691","name":"JSPS KAKENHI","doi-asserted-by":"publisher","award":["21K07450","22HA1003","JP20K07875"],"award-info":[{"award-number":["21K07450","22HA1003","JP20K07875"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003478","name":"MHLW","doi-asserted-by":"publisher","award":["21K07450","22HA1003","JP20K07875"],"award-info":[{"award-number":["21K07450","22HA1003","JP20K07875"]}],"id":[{"id":"10.13039\/501100003478","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001691","name":"JSPS KAKENHI","doi-asserted-by":"publisher","award":["21K07450","22HA1003","JP20K07875"],"award-info":[{"award-number":["21K07450","22HA1003","JP20K07875"]}],"id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Neurology International"],"abstract":"<jats:p>A 58-year-old woman with a history of systemic lupus erythematosus (SLE) who was taking prednisolone and mycophenolate mofetil presented with gait disturbances that progressively worsened over a period of 3 months. Her blood test and cerebrospinal fluid (CSF) examination results did not indicate active SLE. Initial brain magnetic resonance imaging (MRI) revealed a small spotty lesion in the left cerebellar peduncle. The clinical course was consistent with rapidly progressive cerebellar syndrome (RPCS), which sometimes involves neuronal antibodies. The line blot assay detected anti-Yo antibodies, but no malignancy was found. Immunohistological techniques using rat brain sections yielded a negative result for anti-Yo antibodies. The second MRI revealed a focal lesion and surrounding spotty lesion in the left cerebellar peduncle, which was consistent with the punctate pattern observed in progressive multifocal leukoencephalopathy (PML). The CSF JCV-DNA test indicated the presence of cerebellar PML. Immunosuppressants were reduced, and mefloquine and mirtazapine were initiated. After approximately 2 years and 1 month, the CSF JCV-DNA results became negative. Cerebellar PML may exhibit a clinical course that is consistent with RPCS. The punctate pattern should be recognized as an early manifestation of PML. The CSF JCV-DNA copy number may serve as a useful indicator of PML stabilization.<\/jats:p>","DOI":"10.3390\/neurolint15030059","type":"journal-article","created":{"date-parts":[[2023,7,26]],"date-time":"2023-07-26T01:11:26Z","timestamp":1690333886000},"page":"917-925","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Cerebellar Progressive Multifocal Leukoencephalopathy Mimicking Anti-Yo-Antibody-Associated Rapidly Progressive Cerebellar Syndrome"],"prefix":"10.3390","volume":"15","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3390-5792","authenticated-orcid":false,"given":"Takayoshi","family":"Akimoto","sequence":"first","affiliation":[{"name":"Division of Neurology, Department of Medicine, Nihon University School of Medicine, Tokyo 173-8610, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Makoto","family":"Hara","sequence":"additional","affiliation":[{"name":"Division of Neurology, Department of Medicine, Nihon University School of Medicine, Tokyo 173-8610, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Satoshi","family":"Hirose","sequence":"additional","affiliation":[{"name":"Division of Neurology, Department of Medicine, Nihon University School of Medicine, Tokyo 173-8610, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7594-2607","authenticated-orcid":false,"given":"Kazuo","family":"Nakamichi","sequence":"additional","affiliation":[{"name":"Department of Virology 1, National Institute of Infectious Diseases, Tokyo 162-8640, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2154-9196","authenticated-orcid":false,"given":"Hideto","family":"Nakajima","sequence":"additional","affiliation":[{"name":"Division of Neurology, Department of Medicine, Nihon University School of Medicine, Tokyo 173-8610, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2023,7,26]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"178","DOI":"10.1080\/1040841X.2016.1189873","article-title":"45 years after the discovery of human polyomaviruses BK and JC: Time to speed up the understanding of associated diseases and treatment approaches","volume":"43","author":"Barth","year":"2017","journal-title":"Crit. Rev. Microbiol."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"578","DOI":"10.1093\/infdis\/jiq097","article-title":"Pathogenesis of progressive multifocal leukoencephalopathy\u2014Revisited","volume":"203","author":"White","year":"2011","journal-title":"J. Infect. Dis."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"1430","DOI":"10.1212\/WNL.0b013e31828c2fa1","article-title":"PML diagnostic criteria: Consensus statement from the AAN Neuroinfectious Disease Section","volume":"80","author":"Berger","year":"2013","journal-title":"Neurology"},{"key":"ref_4","first-page":"23","article-title":"Progressive multifocal leukoencephalopathy","volume":"137","author":"Alstadhaug","year":"2017","journal-title":"Tidsskr. Nor. Laegeforen."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"775","DOI":"10.1212\/01.WNL.0000081306.86961.33","article-title":"Productive infection of cerebellar granule cell neurons by JC virus in an HIV+ individual","volume":"61","author":"Corey","year":"2003","journal-title":"Neurology"},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"404","DOI":"10.1002\/ana.24574","article-title":"Mechanisms of immune escape in central nervous system infection with neurotropic JC virus variant","volume":"79","author":"Jelcic","year":"2016","journal-title":"Ann. Neurol."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"438","DOI":"10.1016\/S1474-4422(10)70028-4","article-title":"Natalizumab-associated progressive multifocal leukoencephalopathy in patients with multiple sclerosis: Lessons from 28 cases","volume":"9","author":"Clifford","year":"2010","journal-title":"Lancet Neurol."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"37","DOI":"10.1111\/cen3.12440","article-title":"Drug-induced progressive multifocal leukoencephalopathy in multiple sclerosis: A comprehensive review","volume":"9","author":"Yukitake","year":"2018","journal-title":"Clin. Exp. Neuroimmunol."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"59","DOI":"10.3109\/13550289809113482","article-title":"Progressive multifocal leukoencephalopathy in patients with HIV infection","volume":"4","author":"Berger","year":"1998","journal-title":"J. Neurovirol."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"299","DOI":"10.1016\/S0733-8619(18)31105-8","article-title":"Progressive multifocal leukoencephalopathy","volume":"2","author":"Brooks","year":"1984","journal-title":"Neurol. Clin."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"2944","DOI":"10.1007\/s00330-020-07362-y","article-title":"Progressive multifocal leukoencephalopathy: MRI findings in HIV-infected patients are closer to rituximab- than natalizumab-associated PML","volume":"31","author":"Alleg","year":"2021","journal-title":"Eur. Radiol."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"59","DOI":"10.1159\/000444874","article-title":"Progressive Multifocal Leukoencephalopathy and Systemic Lupus Erythematosus: Focus on Etiology","volume":"8","author":"Berntsson","year":"2016","journal-title":"Case Rep. Neurol."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"302","DOI":"10.1007\/s12016-012-8327-x","article-title":"Systemic lupus erythematosus, progressive multifocal leukoencephalopathy, and T-CD4+ lymphopenia","volume":"43","author":"Marinho","year":"2012","journal-title":"Clin. Rev. Allergy Immunol."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"1560","DOI":"10.1016\/j.jfma.2019.05.021","article-title":"Progressive multifocal leukoencephalopathy in a 27-year-old lady with systemic lupus erythematosus\u2014Rheumatoid arthritis overlap syndrome","volume":"118","author":"Cheng","year":"2019","journal-title":"J. Formos. Med. Assoc."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"85","DOI":"10.1097\/NRL.0b013e318247b868","article-title":"Progressive multifocal leukoencephalopathy in a patient treated with etanercept","volume":"18","author":"Robinson","year":"2012","journal-title":"Neurologist"},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"731","DOI":"10.1007\/s00415-019-09629-x","article-title":"Successful treatment of non-HIV progressive multifocal leukoencephalopathy: Case report and literature review","volume":"267","author":"Hamaguchi","year":"2020","journal-title":"J. Neurol."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"199","DOI":"10.1002\/ana.410110218","article-title":"Primary cerebellopontine progressive multifocal leukoencephalopathy diagnosed premortem by cerebellar biopsy","volume":"11","author":"Jones","year":"1982","journal-title":"Ann. Neurol."},{"key":"ref_18","first-page":"313","article-title":"Progressive multifocal leukoencephalopathy resembling central nervous system systemic lupus erythematosus","volume":"16","author":"Kinoshita","year":"1998","journal-title":"Clin. Exp. Rheumatol."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"272","DOI":"10.1080\/24725625.2021.1899763","article-title":"Infratentorial onset of progressive multifocal leukoencephalopathy in a patient with systematic lupus erythematosus complicated with lymphoma: A case report","volume":"5","author":"Sakuraba","year":"2021","journal-title":"Mod. Rheumatol. Case Rep."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1007\/s100720170031","article-title":"Reversal of CSF positivity for JC virus genome by cidofovir in a patient with systemic lupus erythematosus and progressive multifocal leukoencephalopathy","volume":"22","author":"Salmaggi","year":"2001","journal-title":"Neurol. Sci."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"196","DOI":"10.1136\/practneurol-2018-002096","article-title":"Diagnosis and management of progressive ataxia in adults","volume":"19","author":"Vallortigara","year":"2019","journal-title":"Pract. Neurol."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"e1014","DOI":"10.1212\/NXI.0000000000001014","article-title":"Updated Diagnostic Criteria for Paraneoplastic Neurologic Syndromes","volume":"8","author":"Graus","year":"2021","journal-title":"Neurol. R Neuroimmunol. Neuroinflammation"},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"1516","DOI":"10.1212\/WNL.0000000000002586","article-title":"Punctate pattern: A promising imaging marker for the diagnosis of natalizumab-associated PML","volume":"86","author":"Hodel","year":"2016","journal-title":"Neurology"},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"2727","DOI":"10.2169\/internalmedicine.0696-17","article-title":"A Punctate Magnetic Resonance Imaging Pattern in a Patient with Systemic Lupus Erythematosus Is an Early Sign of Progressive Multifocal Leukoencephalopathy: A Clinicopathological Study","volume":"57","author":"Ishii","year":"2018","journal-title":"Intern. Med."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"e559","DOI":"10.1212\/NXI.0000000000000559","article-title":"Three cases of non-carryover fingolimod-PML: Is the risk in Japan increased?","volume":"6","author":"Nakahara","year":"2019","journal-title":"Neurol. R Neuroimmunol. Neuroinflammation"},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"1176","DOI":"10.1136\/jnnp-2013-304986","article-title":"Punctate lesion pattern suggestive of perivascular inflammation in acute natalizumab-associated progressive multifocal leukoencephalopathy: Productive JC virus infection or preclinical PML-IRIS manifestation?","volume":"84","author":"Wattjes","year":"2013","journal-title":"J. Neurol. Neurosurg. Psychiatry"},{"key":"ref_27","doi-asserted-by":"crossref","unstructured":"Nakamichi, K., Kawamoto, M., Ishii, J., and Saijo, M. (2019). Improving detection of JC virus by ultrafiltration of cerebrospinal fluid before polymerase chain reaction for the diagnosis of progressive multifocal leukoencephalopathy. BMC Neurol., 19.","DOI":"10.1186\/s12883-019-1476-2"},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"243","DOI":"10.1016\/j.jcv.2013.03.009","article-title":"Multiplex qPCR assay for ultra sensitive detection of JCV DNA with simultaneous identification of genotypes that discriminates non-virulent from virulent variants","volume":"57","author":"Ryschkewitsch","year":"2013","journal-title":"J. Clin. Virol."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"1717","DOI":"10.1212\/01.wnl.0000219042.60538.92","article-title":"Scale for the assessment and rating of ataxia: Development of a new clinical scale","volume":"66","author":"Baliko","year":"2006","journal-title":"Neurology"},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"155","DOI":"10.1186\/1752-1947-6-155","article-title":"Anti-Yo and anti-glutamic acid decarboxylase antibodies presenting in carcinoma of the uterus with paraneoplastic cerebellar degeneration: A case report","volume":"6","author":"Panegyres","year":"2012","journal-title":"J. Med. Case Rep."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"19","DOI":"10.1159\/000494423","article-title":"Immune Mediated Cerebellar Ataxia: An Unknown Manifestation of Graft-versus-Host Disease","volume":"141","author":"Raanani","year":"2019","journal-title":"Acta Haematol."},{"key":"ref_32","first-page":"7401","article-title":"Subacute cerebellar ataxia as presenting symptom of systemic lupus erythematosus","volume":"22","author":"Casciato","year":"2018","journal-title":"Eur. Rev. Med. Pharmacol. Sci."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"e236825","DOI":"10.1136\/bcr-2020-236825","article-title":"Cerebellar ataxia as a primary manifestation of neuropsychiatric systemic lupus erythematosus","volume":"14","author":"Sy","year":"2021","journal-title":"BMJ Case Rep."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"1482","DOI":"10.3389\/fimmu.2020.01482","article-title":"The Diagnostic Value of Onconeural Antibodies Depends on How They Are Tested","volume":"11","author":"Saiz","year":"2020","journal-title":"Front. Immunol."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"e200076","DOI":"10.1212\/NXI.0000000000200076","article-title":"N2 Year in Review","volume":"10","author":"Dalmau","year":"2023","journal-title":"Neurol. R Neuroimmunol. Neuroinflammation"},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"617","DOI":"10.1177\/0961203315622819","article-title":"Progressive multifocal leukoencephalopathy in patients with systemic lupus erythematosus: A systematic literature review","volume":"25","author":"Henegar","year":"2016","journal-title":"Lupus"},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"1526","DOI":"10.1007\/s00330-007-0788-6","article-title":"Infratentorial progressive multifocal leucoencephalopathy (PML) in a patient with SLE (2008: 4b)","volume":"18","author":"Svensson","year":"2008","journal-title":"Eur. Radiol."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"804","DOI":"10.1111\/imj.14318","article-title":"John Cunningham virus granule cell neuronopathy in a mildly immunosuppressed patient with systemic lupus erythematosus","volume":"49","author":"Zhong","year":"2019","journal-title":"Intern. Med. J."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"738","DOI":"10.1086\/427698","article-title":"Prognostic significance of JC virus DNA levels in cerebrospinal fluid of patients with HIV-associated progressive multifocal leukoencephalopathy","volume":"40","author":"Bossolasco","year":"2005","journal-title":"Clin. Infect. Dis."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"755","DOI":"10.1002\/acn3.114","article-title":"Outcome and survival of asymptomatic PML in natalizumab-treated MS patients","volume":"1","author":"Richman","year":"2014","journal-title":"Ann. Clin. Transl. Neurol."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"351","DOI":"10.1007\/s13365-013-0173-y","article-title":"A study of mefloquine treatment for progressive multifocal leukoencephalopathy: Results and exploration of predictors of PML outcomes","volume":"19","author":"Clifford","year":"2013","journal-title":"J. Neurovirol."}],"container-title":["Neurology International"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2035-8377\/15\/3\/59\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,10]],"date-time":"2025-10-10T20:19:02Z","timestamp":1760127542000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2035-8377\/15\/3\/59"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,7,26]]},"references-count":41,"journal-issue":{"issue":"3","published-online":{"date-parts":[[2023,9]]}},"alternative-id":["neurolint15030059"],"URL":"https:\/\/doi.org\/10.3390\/neurolint15030059","relation":{},"ISSN":["2035-8377"],"issn-type":[{"type":"electronic","value":"2035-8377"}],"subject":[],"published":{"date-parts":[[2023,7,26]]}}}