{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,18]],"date-time":"2026-05-18T07:07:52Z","timestamp":1779088072424,"version":"3.51.4"},"reference-count":15,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2025,3,13]],"date-time":"2025-03-13T00:00:00Z","timestamp":1741824000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"},{"start":{"date-parts":[[2025,3,13]],"date-time":"2025-03-13T00:00:00Z","timestamp":1741824000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/501100018949","name":"Oulun Yliopistollinen Sairaala","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100018949","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["movementdisorders.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Movement Disord Clin Pract"],"published-print":{"date-parts":[[2025,6]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Neurodegeneration with Brain Iron Accumulation (NBIA) rarely manifests after the age of 50\u2009years. The phenotype in these cases is most often parkinsonism.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>To present the case with the oldest age of NBIA onset reported so far.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Clinico\u2010pathological case.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>A female patient presented at 84\u2009years of age with wobbling of the head that had started approximately 2\u2009years ago. Choreiform movements of the head and upper body were observed and these abated when she focused on doing something else or lay down but started again when she was talking or moving. There were no cerebellar signs, abnormal reflexes or sensory disturbance. Cognitive screening tests were abnormal but significant cognitive symptoms absent. Magnetic Resonance Imaging (MRI) showed extensive iron accumulation in the basal ganglia and upper pons. Extensive analyses yielded no genetic diagnosis. She died suddenly 19\u2009months after her first appointment. In neuropathological analysis the basal ganglia, especially the lenticular nuclei, were macroscopically darker than normal with notable iron accumulation in the arterial walls in these areas. Prominent axonal ballooning was observed especially in the internal globus pallidus. Globus pallidus displayed iron accumulation, observed to a slightly lesser extent also in the substantia nigra pars reticulata. The neuropathological phenotype resembled classical pantothenate kinase\u2010associated neurodegeneration (PKAN). Concomitant beta\u2010amyloid, hyperphosphorylated tau protein (consistent with primary age\u2010related tauopathy, or PART) and TDP\u201043 (consistent with LATE\u2010NC) pathologies were also evident.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>NBIA may manifest at a very advanced age with a mild phenotype, likely influenced by coexisting neuropathology.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/mdc3.70032","type":"journal-article","created":{"date-parts":[[2025,3,13]],"date-time":"2025-03-13T10:14:47Z","timestamp":1741860887000},"page":"835-841","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Very Late\u2010Onset Neurodegeneration with Brain Iron Accumulation Associated with Mild Chorea: A Clinicopathological Case"],"prefix":"10.1002","volume":"12","author":[{"given":"Jussi O.T.","family":"Sipil\u00e4","sequence":"first","affiliation":[{"name":"Department of Neurology North Karelia Central Hospital  Joensuu Finland"},{"name":"Neurology, Institute of Clinical Medicine, School of Medicine, Faculty of Health Sciences University of Eastern Finland  Kuopio Finland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Aki","family":"Hietaharju","sequence":"additional","affiliation":[{"name":"Department of Neurology Tampere University Hospital  Tampere Finland"},{"name":"Faculty of Medicine and Health Technology Tampere University  Tampere Finland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anna Maija","family":"Saukkonen","sequence":"additional","affiliation":[{"name":"Department of Neurology North Karelia Central Hospital  Joensuu Finland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2790-5646","authenticated-orcid":false,"given":"Laura","family":"Kyt\u00f6vuori","sequence":"additional","affiliation":[{"name":"Research Unit of Clinical Medicine, Medical Research Center Oulu Oulu University Hospital and University of Oulu  Oulu Finland"},{"name":"Neurocenter, Neurology Oulu University Hospital  Oulu Finland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Liisu","family":"Balk","sequence":"additional","affiliation":[{"name":"Department of Neurology North Karelia Central Hospital  Joensuu Finland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3446-7093","authenticated-orcid":false,"given":"Valtteri","family":"Kaasinen","sequence":"additional","affiliation":[{"name":"Neurocenter Turku University Hospital  Turku Finland"},{"name":"Clinical Neurosciences University of Turku  Turku Finland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tuomas","family":"Rauramaa","sequence":"additional","affiliation":[{"name":"Unit of Pathology, Institute of Clinical Medicine University of Eastern Finland  Kuopio Finland"},{"name":"Department of Pathology Kuopio University Hospital  Kuopio Finland"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2025,3,13]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-014-1349-0"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-022-02524-2"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/B978-0-444-63233-3.00019-1"},{"key":"e_1_2_7_5_1","volume-title":"Neurodegeneration with Brain Iron Accumulation Disorders Overview","author":"Gregory 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