{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,24]],"date-time":"2026-06-24T11:50:39Z","timestamp":1782301839483,"version":"3.54.5"},"reference-count":16,"publisher":"Gobierno de Navarra","issue":"1","license":[{"start":{"date-parts":[[2026,2,18]],"date-time":"2026-02-18T00:00:00Z","timestamp":1771372800000},"content-version":"unspecified","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-sa\/4.0\/deed.es"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["An Sist Sanit Navar"],"abstract":"<jats:p>El s\u00edndrome de Fahr es una enfermedad rara caracterizada por calcificaciones cerebrales bilaterales, generalmente asociadas a alteraciones del metabolismo fosfoc\u00e1lcico, siendo el hipoparatiroidismo idiop\u00e1tico la causa m\u00e1s frecuente.\nSe presenta el caso de un var\u00f3n de 56 a\u00f1os con convulsiones, alteraci\u00f3n del comportamiento con hipocalcemia severa, hiperfosfatemia y niveles bajos de PTH. Las pruebas de imagen evidenciaron las calcificaciones t\u00edpicas de este s\u00edndrome, siendo la tomograf\u00eda computarizada la prueba de imagen de referencia. El diagn\u00f3stico se apoya en la combinaci\u00f3n de hallazgos cl\u00ednicos, anal\u00edticos y radiol\u00f3gicos, y es crucial descartar otras causas de calcificaciones cerebrales como enfermedades autoinmunes, infecciosas o gen\u00e9ticas (enfermedad de Fahr). El tratamiento consiste en corregir la hipocalcemia y mantener una monitorizaci\u00f3n estricta del calcio.\nUn diagn\u00f3stico precoz del hipoparatiroidismo puede prevenir las complicaciones neurol\u00f3gicas del s\u00edndrome de Fahr, siendo esencial el papel del laboratorio en la identificaci\u00f3n de esta patolog\u00eda y su adecuado manejo.<\/jats:p>","DOI":"10.23938\/assn.1138","type":"journal-article","created":{"date-parts":[[2026,2,18]],"date-time":"2026-02-18T08:16:53Z","timestamp":1771402613000},"page":"e1138","source":"Crossref","is-referenced-by-count":0,"title":["Caso de hipocalcemia grave secundaria a s\u00edndrome de Fahr","A case report of severe hypocalcemia secondary to Fahr\u2019s syndrome"],"prefix":"10.23938","volume":"49","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3696-8254","authenticated-orcid":false,"given":"Miren","family":"Vallejo Ruiz","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0005-3027-199X","authenticated-orcid":false,"given":"In\u00e9s","family":"Moral Presa","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"10024","published-online":{"date-parts":[[2026,2,18]]},"reference":[{"key":"ref0","doi-asserted-by":"publisher","unstructured":"1. SALEEM S, ASLAM HM, ANWAR M, ANWAR S, SALEEM M, SALEEM A et al. Fahr's syndrome: Literature review of current evidence. Orphanet J Rare Dis 2013;8:156. https:\/\/doi.org\/10.1186\/1750-1172-8-156","DOI":"10.1186\/1750-1172-8-156"},{"key":"ref1","doi-asserted-by":"publisher","unstructured":"2. MOSKOWITZ MA, WINICKOFF RN, HEINZ ER. Familial calcification of the basal ganglions: A metabolic and genetic study. N Engl J Med 1971;285(2):72-77. https:\/\/doi.org\/10.1056\/NEJM197107082850202","DOI":"10.1056\/NEJM197107082850202"},{"key":"ref2","unstructured":"3. AMISHA F, MUNAKOMI S. Fahr Syndrome [Updated 2023 Aug 13]. Treasure Island (FL): StatPearls Publishing; 2025. https:\/\/www.ncbi.nlm.nih.gov\/books\/NBK560857\/"},{"key":"ref3","doi-asserted-by":"publisher","unstructured":"4. PISTACCHI M, GIOULIS M, SANSON F, MARSALA SM. Fahr's syndrome and clinical correlation: a case series and literature review. Folia Neuropathol 2016;54(3):282-294. https:\/\/doi.org\/10.5114\/fn.2016.62538","DOI":"10.5114\/fn.2016.62538"},{"key":"ref4","doi-asserted-by":"publisher","unstructured":"5. COOPER MS, GITTOES NJ. Diagnosis and management of hypocalcemia. BMJ. 2008;336(7656):1298-1302. https:\/\/doi.org\/10.1136\/bmj.39582.589433.BE","DOI":"10.1136\/bmj.39582.589433.BE"},{"key":"ref5","doi-asserted-by":"publisher","unstructured":"6. CLARKE BL. Hypoparathyroidism: update of guidelines from the 2022 International Task Force. Arch Endocrinol Metab 2022;66(5):604-610. https:\/\/doi.org\/10.20945\/2359-3997000000549","DOI":"10.20945\/2359-3997000000549"},{"key":"ref6","doi-asserted-by":"publisher","unstructured":"7. KALAMPOKINI S, GEORGOULI D, DADOULI K, NTELLAS P, RALLI S, VALOTASSIOU V, et al. Fahr's syndrome due to hypoparathyroidism revisited: A case of parkinsonism and a review of all published cases. Clin Neurol Neurosurg 2021;202:106514. https:\/\/doi.org\/10.1016\/j.clineuro.2021.106514","DOI":"10.1016\/j.clineuro.2021.106514"},{"key":"ref7","unstructured":"8. TRAOR\u00c9 M, DAO A, KON\u00c9 A, CISS\u00c9 BS, TRAOR\u00c9 N, DIARRA O et al. [Fahr's Syndrome with Hyperparathyroidism: A Case Report]. Health Res Afr 2025;3(11):7117. https:\/\/www.hsd-fmsb.org\/index.php\/hra\/article\/view\/7117\/5444"},{"key":"ref8","doi-asserted-by":"publisher","unstructured":"9. ALAM ST, ASWANI Y, ANANDPARA KJ, HIRA P. CT findings in Fahr's disease. BMJ Case Rep 2015;2015:bcr2014208812. https:\/\/doi.org\/10.1136\/bcr-2014-208812","DOI":"10.1136\/bcr-2014-208812"},{"key":"ref9","doi-asserted-by":"publisher","unstructured":"10. DONZUSO G, MOSTILE G, NICOLETTI A, ZAPPIA M. Basal ganglia calcifications (Fahr's syndrome): Related conditions and clinical features. Neurol Sci 2019;40(11):2251- 2263. https:\/\/doi.org\/10.1007\/s10072-019-03998-x","DOI":"10.1007\/s10072-019-03998-x"},{"key":"ref10","doi-asserted-by":"publisher","unstructured":"11. MANYAM BV. What is and what is not 'Fahr's disease'. Parkinsonism Relat Disord 2005;11(2):73-80. https:\/\/doi.org\/10.1016\/j.parkreldis.2004.12.001","DOI":"10.1016\/j.parkreldis.2004.12.001"},{"key":"ref11","doi-asserted-by":"publisher","unstructured":"12. GESCHWIND DH, LOGINOV M, STERN JM. Identification of a locus on chromosome 14q for idiopathic basal ganglia calcification (Fahr disease). Am J Hum Genet 1999;65(3):764-772. https:\/\/doi.org\/10.1086\/302558","DOI":"10.1086\/302558"},{"key":"ref12","doi-asserted-by":"publisher","unstructured":"13. PETERS MEM, DE BROUWER EJM, BARTSTRA JW, MALI WPTM, KOEK HL, ROZEMULLER AJM et al. Mechanisms of calcification in Fahr disease and exposure of potential therapeutic targets. Neurol Clin Pract 2020;10(5):449-457. https:\/\/doi.org\/10.1212\/CPJ.0000000000000782","DOI":"10.1212\/CPJ.0000000000000782"},{"key":"ref13","doi-asserted-by":"publisher","unstructured":"14. ELLIE E, JULIEN J, FERRER X. Familial idiopathic striopallidodentate calcifications. Neurology 1989;39(3):381-385. https:\/\/doi.org\/10.1212\/wnl.39.3.381","DOI":"10.1212\/WNL.39.3.381"},{"key":"ref14","doi-asserted-by":"publisher","unstructured":"15. ALMUTAIRI MM, AHMED ZJ, AHMED MY, ALSHALAN SM, AHMED AE. Progressive motor and cognitive dysfunction in Fahr's disease: a clinical case report. Cureus 2025;17(1):e77969. https:\/\/doi.org\/10.7759\/cureus.77969","DOI":"10.7759\/cureus.77969"},{"key":"ref15","doi-asserted-by":"publisher","unstructured":"16. BILEZIKIAN JP, BRANDI ML, CUSANO NE, MANNSTADT M, REJNMARK L, RIZZOLI R et al. Management of hypoparathyroidism: present and future. 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