{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,21]],"date-time":"2026-09-21T05:10:17Z","timestamp":1789967417947,"version":"4.0.1"},"reference-count":7,"publisher":"The Endocrine Society","issue":"6","license":[{"start":{"date-parts":[[2023,12,1]],"date-time":"2023-12-01T00:00:00Z","timestamp":1701388800000},"content-version":"vor","delay-in-days":30,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2023,11,3]]},"abstract":"<jats:title>Abstract<\/jats:title>\n               <jats:p>Fahr syndrome is a rare neurologic disorder, usually affecting young and middle-aged adults, that can present with symptoms ranging from extrapyramidal to neuropsychiatric abnormalities. Pseudohypoparathyroidism (PHP), characterized by parathyroid hormone (PTH)-resistance or PTH-unresponsiveness at target organs, is associated with Fahr syndrome and typically presents with hypocalcemia. The following case presents a 39-year-old-woman with PHP complicated by symptomatic hypocalcemia, hypokalemia, and movement disturbances, who had computed tomography imaging showing basal ganglia calcifications consistent with Fahr syndrome. She initially presented with headache and was hospitalized for hypertensive emergency and severe hypocalcemia. Examination, including the neurologic examination, was unrevealing aside from hypertension and central adiposity. Laboratory tests were consistent with PHP, showing hypocalcemia with elevated PTH, and negative for hyperaldosteronism. Management of hypocalcemia consisted of intravenous calcium infusion, oral calcium carbonate, oral vitamin D3, and oral calcitriol. Patients with severe hypocalcemia and elevated PTH who present with new neurological symptoms despite normal general neurologic examination may warrant consideration for brain imaging to evaluate for Fahr syndrome. Further investigations are necessary to determine the prevalence of Fahr syndrome and hypokalemia in patients with PHP, explore if these findings are significantly associated with PHP-1b subtype, and ultimately inform potential new screening pathways for these patients.<\/jats:p>","DOI":"10.1210\/jcemcr\/luad147","type":"journal-article","created":{"date-parts":[[2023,12,1]],"date-time":"2023-12-01T11:22:13Z","timestamp":1701429733000},"source":"Crossref","is-referenced-by-count":5,"title":["Fahr Syndrome Secondary to Pseudohypoparathyroidism"],"prefix":"10.1210","volume":"1","author":[{"ORCID":"https:\/\/orcid.org\/0009-0009-0886-6806","authenticated-orcid":false,"given":"Sharon W","family":"Shu","sequence":"first","affiliation":[{"name":"Department of Internal Medicine, Ochsner Medical Center , New Orleans, LA 70121 , USA"},{"name":"University of Queensland-Ochsner Clinical School , Brisbane, QLD 4072 , Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sakshi","family":"Sharma","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Ochsner Medical Center , New Orleans, LA 70121 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qasim Z","family":"Iqbal","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Ochsner Medical Center , New Orleans, LA 70121 , USA"},{"name":"Endocrinology Division, Indiana University School of Medicine , Indianapolis, IN 46202 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karina G","family":"Romo","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine, Ochsner Medical Center , New Orleans, LA 70121 , USA"},{"name":"Department of Endocrinology, Cedars-Sinai Medical Center , Los Angeles, CA 90048 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"80","published-online":{"date-parts":[[2023,12,1]]},"reference":[{"key":"2023121603033352500_luad147-B1","volume-title":"StatPearls","author":"Amisha","year":"2020"},{"issue":"11","key":"2023121603033352500_luad147-B2","doi-asserted-by":"crossref","first-page":"2251","DOI":"10.1007\/s10072-019-03998-x","article-title":"Basal ganglia calcifications (Fahr's Syndrome): related conditions and clinical features","volume":"40","author":"Donzuso","year":"2019","journal-title":"Neurol Sci"},{"key":"2023121603033352500_luad147-B3","doi-asserted-by":"crossref","first-page":"156","DOI":"10.1186\/1750-1172-8-156","article-title":"Fahr's syndrome: literature review of current evidence","volume":"8","author":"Saleem","year":"2013","journal-title":"Orphanet J Rare Dis"},{"key":"2023121603033352500_luad147-B4","volume-title":"StatPearls","author":"Ucciferro","year":"2023"},{"issue":"1","key":"2023121603033352500_luad147-B5","doi-asserted-by":"crossref","first-page":"98","DOI":"10.1186\/s12902-022-01011-9","article-title":"Clinical and genetic analysis of pseudohypoparathyroidism complicated by hypokalemia: a case report and review of the literature","volume":"22","author":"Huang","year":"2022","journal-title":"BMC Endocr Disord"},{"issue":"1","key":"2023121603033352500_luad147-B6","first-page":"50","article-title":"A case of sporadic pseudohypoparathyroidism type 1B presented with hypokalemia","volume":"54","author":"Yang","year":"2021","journal-title":"Horm Metab Res"},{"issue":"3","key":"2023121603033352500_luad147-B7","doi-asserted-by":"crossref","first-page":"luad072","DOI":"10.1210\/jcemcr\/luad072","article-title":"Fahr syndrome presenting with Status epilepticus after COVID-19 infection","volume":"1","author":"Bitew","year":"2023","journal-title":"JCEM Case Reprts"}],"container-title":["JCEM Case Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/academic.oup.com\/jcemcr\/article-pdf\/1\/6\/luad147\/54078573\/luad147.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/academic.oup.com\/jcemcr\/article-pdf\/1\/6\/luad147\/54078573\/luad147.pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,12,16]],"date-time":"2023-12-16T03:03:48Z","timestamp":1702695828000},"score":1,"resource":{"primary":{"URL":"https:\/\/academic.oup.com\/jcemcr\/article\/doi\/10.1210\/jcemcr\/luad147\/7456747"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2023,11]]},"references-count":7,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2023,11,3]]}},"URL":"https:\/\/doi.org\/10.1210\/jcemcr\/luad147","relation":{},"ISSN":["2755-1520"],"issn-type":[{"value":"2755-1520","type":"electronic"}],"subject":[],"published-other":{"date-parts":[[2023,11]]},"published":{"date-parts":[[2023,11]]},"article-number":"luad147"}}