{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,12]],"date-time":"2026-06-12T18:07:58Z","timestamp":1781287678532,"version":"3.54.1"},"reference-count":22,"publisher":"SAGE Publications","issue":"6","license":[{"start":{"date-parts":[[2012,12,1]],"date-time":"2012-12-01T00:00:00Z","timestamp":1354320000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Neuroradiol J"],"published-print":{"date-parts":[[2012,12]]},"abstract":"<jats:p>Studies on magnetic resonance imaging (MRI) observations in neuronal ceroid lipofuscinosis (NCL) are few and far between. We evaluated the MRI characteristics of patients with NCL and its subtypes. Forty-three out of 68 patients with ultrastructurally confirmed NCL (M:F=46:22; age at evaluation: 6.3\u00b15.2 years) underwent brain MRI evaluation (1998-2010). The demography, phenotype and subtypes of NCL (n=43) [infantile (I-NCL): 5; late infantile (LI-NCL): 26; Juvenile (J-NCL): 11; adult (A-NCL): 1] were recorded. MRI (brain) was carried out using standard sequences in all. Brain atrophy and signal alterations were assessed visually. Brain MRI was abnormal in all 43 patients - diffuse cerebral atrophy (100%), cerebellar atrophy (40%), cerebral leucoencephalopathy (65%) and thalamic T2W-hypointensity (33%). Diffuse cerebral atrophy was noted in: I-NCL (100%), LI-NCL (62%), J-NCL (36%) and A-NCL (100%) [p=0.05]. Cerebellar atrophy was present in: LI-NCL (42%) and J-NCL (55%). Leucoencephalopathy was observed in: I- NCL (100%), LI-NCL (65%), J-NCL (45%) and A-NCL (100%)). Thalamic T2W-hypointensity was most frequent in I-NCL (80%) followed by J-NCL (36%) and LI-NCL (23%). This is the largest study of its kind. The presence of cerebellar\/cerebral atrophy, leucoencephalopathy and thalamic T2W-hypointensity in an appropriate clinical setting might provide clues to the diagnosis of NCL: leucoencephalopathy and thalamic hypointensity (T2W) were noted more frequently in I-NCL, periventricular and parieto-occipital hyperintensities in LI-NCL, and cerebellar atrophy in J-NCL.<\/jats:p>","DOI":"10.1177\/197140091202500616","type":"journal-article","created":{"date-parts":[[2015,4,22]],"date-time":"2015-04-22T17:40:15Z","timestamp":1429724415000},"page":"755-761","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":19,"title":["Magnetic Resonance Imaging in Neuronal Ceroid Lipofuscinosis and its Subtypes"],"prefix":"10.1177","volume":"25","author":[{"given":"R.H.","family":"Jadav","sequence":"first","affiliation":[{"name":"Departments of Neurology, National Institute of Mental Health and Neurosciences; Bangalore, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S.","family":"Sinha","sequence":"additional","affiliation":[{"name":"Departments of Neurology, National Institute of Mental Health and Neurosciences; Bangalore, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"T.C.","family":"Yasha","sequence":"additional","affiliation":[{"name":"Neuropathology, National Institute of Mental Health and Neurosciences; Bangalore, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H.","family":"Aravinda","sequence":"additional","affiliation":[{"name":"NIIR, National Institute of Mental Health and Neurosciences; Bangalore, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S.","family":"Rao","sequence":"additional","affiliation":[{"name":"Biostatistics, National Institute of Mental Health and Neurosciences; Bangalore, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P.S.","family":"Bindu","sequence":"additional","affiliation":[{"name":"Departments of Neurology, National Institute of Mental Health and Neurosciences; Bangalore, India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P.","family":"Satishchandra","sequence":"additional","affiliation":[{"name":"Departments of Neurology, National Institute of Mental Health and Neurosciences; 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