{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,27]],"date-time":"2026-08-27T14:22:39Z","timestamp":1787840559616,"version":"build-2784847793"},"reference-count":16,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"2","license":[{"start":{"date-parts":[[2025,5,1]],"date-time":"2025-05-01T00:00:00Z","timestamp":1746057600000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-sa\/4.0"}],"content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":["Ann Mov Disord"],"published-print":{"date-parts":[[2025,5]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Being a rare disorder, Creutzfeldt\u2013Jakob disease (CJD) reports are limited to a few Indian case series. To date, only three kindreds of Gerstmann\u2013Straussler\u2013Scheinker (GSS) syndrome have been reported from India. We report the clinical, electroencephalographic, and radiological profile of 7 CJD patients observed over a period of 5 years, from January 2018 to February 2023, at a tertiary care centre in Northern India. Of these, 6 (85.7%) were diagnosed with sporadic CJD (sCJD), and one case was genetically diagnosed with GSS syndrome. In the six sCJD patients, the median age of onset and disease duration were 59 (44\u201366) years and 3 (1\u201311) months, respectively. Three sCJD patients were males, and three were female. Abnormal gait (100%), myoclonus (100%), impaired cognition (100%), and parkinsonism (100%) were the most common neurological features. Brain magnetic resonance imaging (MRI) revealed diffusion restriction with low Apparent Diffusion Coefficient involving the bilateral striatum in all sCJD patients, with the characteristic electroencephalogram (EEG) finding of generalized periodic sharp wave complexes at 1\u20132 Hz seen in 4 (66.7%) patients. The GSS patient was a 32-year-old female with predominantly ataxia of 10 years duration. Her brain MRI revealed cerebral and cerebellar atrophy, and the EEG showed focal and generalized epileptiform discharges. While sCJD is the most prevalent prion disorder, GSS should be considered in hereditary ataxia patients negative for common genetic conditions.<\/jats:p>","DOI":"10.4103\/aomd.aomd_118_24","type":"journal-article","created":{"date-parts":[[2025,8,12]],"date-time":"2025-08-12T09:00:27Z","timestamp":1754989227000},"page":"131-135","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":1,"title":["Case Series of Prion Disease Patients from Northern India: Insights from Clinical, EEG, and Imaging Data"],"prefix":"10.4103","volume":"8","author":[{"given":"Niraj","family":"Kumar","sequence":"first","affiliation":[{"name":"Department of Neurology, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India"},{"name":"Department of Neurology, All India Institute of Medical Sciences, Hyderabad, Telangana, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Govind","family":"Madhaw","sequence":"additional","affiliation":[{"name":"Department of Neurology, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India"},{"name":"Department of Neurology, Centre of Neurosciences, Ranchi, Jharkhand, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mritunjai","family":"Kumar","sequence":"additional","affiliation":[{"name":"Department of Neurology, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ashutosh","family":"Tiwari","sequence":"additional","affiliation":[{"name":"Department of Neurology, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India"},{"name":"Department of Neurology, All India Institute of Medical Sciences, Gorakhpur, Uttar Pradesh, India"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","published-online":{"date-parts":[[2025,8,11]]},"reference":[{"key":"CIT0001-20250812","doi-asserted-by":"crossref","first-page":"14","DOI":"10.1038\/s41572-024-00497-y","article-title":"Creutzfeldt\u2013Jakob disease and other prion diseases","volume":"10","author":"Zerr","year":"2024","journal-title":"Nat Rev Dis Primer"},{"key":"CIT0002-20250812","doi-asserted-by":"crossref","first-page":"93","DOI":"10.1159\/000110515","article-title":"Creutzfeldt-Jakob disease in western India","volume":"2","author":"Singhal","year":"1983","journal-title":"Neuroepidemiology"},{"key":"CIT0003-20250812","first-page":"338","article-title":"Creutzfeldt-Jakob disease: Report of 10 cases from North India","volume":"49","author":"Mehndiratta","year":"2001","journal-title":"Neurology India"},{"key":"CIT0004-20250812","doi-asserted-by":"crossref","first-page":"39","DOI":"10.4103\/0976-3147.143189","article-title":"A study of 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magnetic resonance imaging features in sporadic Creutzfeldt\u2013Jakob disease: A case series of four patients","volume":"6","author":"Verma","year":"2020","journal-title":"Indian J Case Rep"},{"key":"CIT0008-20250812","doi-asserted-by":"crossref","first-page":"14","DOI":"10.5334\/tohm.753","article-title":"Spectrum and pattern of movement disorders in patients with sporadic Creutzfeldt-Jakob disease","volume":"13","author":"Gurram","year":"2023","journal-title":"Tremor Hyperkinetic Mov"},{"key":"CIT0009-20250812","doi-asserted-by":"crossref","first-page":"438","DOI":"10.4103\/0253-7176.191376","article-title":"Creutzfeldt-Jakob disease phenotype and course: Our experience from a tertiary center","volume":"38","author":"Chandra","year":"2016","journal-title":"Indian J Psychol Med"},{"key":"CIT0010-20250812","doi-asserted-by":"crossref","first-page":"1431","DOI":"10.4103\/0028-3886.304068","article-title":"Familial prion disease: First Indian kindred with Gerstmann\u2013Str\u00e4ussler\u2013Scheinker syndrome","volume":"68","author":"Bhatia","year":"2020","journal-title":"Neurology India"},{"key":"CIT0011-20250812","doi-asserted-by":"crossref","first-page":"107218","DOI":"10.1016\/j.parkreldis.2024.107218","article-title":"Gerstmann-Straussler-Scheinker disease: Lessons from two new Indian kindreds","volume":"130","author":"Mustafa","year":"2025","journal-title":"Parkinsonism Relat Disord"},{"key":"CIT0012-20250812","doi-asserted-by":"crossref","first-page":"e2146319","DOI":"10.1001\/jamanetworkopen.2021.46319","article-title":"Validation of revised international Creutzfeldt-Jakob disease surveillance network diagnostic criteria for sporadic Creutzfeldt-Jakob disease","volume":"5","author":"Watson","year":"2022","journal-title":"JAMA Netw Open"},{"key":"CIT0013-20250812","doi-asserted-by":"crossref","first-page":"108492","DOI":"10.1016\/j.clineuro.2024.108492","article-title":"Phenomenological patterns and aetiological spectrum in patients visiting a tertiary care movement disorders service in India: An observational study","volume":"245","author":"Kumar","year":"2024","journal-title":"Clin Neurol Neurosurg"},{"key":"CIT0014-20250812","doi-asserted-by":"crossref","first-page":"790","DOI":"10.1016\/S0140-6736(05)67193-0","article-title":"Did BSE in the UK originate from the Indian subcontinent?","volume":"366","author":"Shankar","year":"2005","journal-title":"Lancet"},{"key":"CIT0015-20250812","doi-asserted-by":"crossref","first-page":"1893","DOI":"10.1002\/mds.29152","article-title":"Prevalence and treatments of movement disorders in prion diseases: A longitudinal cohort study","volume":"37","author":"Sequeira","year":"2022","journal-title":"Mov Disord Off J Mov Disord Soc"},{"key":"CIT0016-20250812","doi-asserted-by":"crossref","first-page":"760","DOI":"10.4103\/aian.AIAN_672_20","article-title":"EEG observations in probable sporadic CJD","volume":"23","author":"Mundlamurri","year":"2020","journal-title":"Ann 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