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Molecular genetics of this disease haven\u2019t been studied extensively; hence evidence at the molecular and genetic level is limited. Fahr\u2019s disease commonly affects young to middle aged adults. Etiology of this syndrome does not identify a specific agent but associations with a number of conditions have been noted; most common of which are endocrine disorders, mitochondrial myopathies, dermatological abnormalities and infectious diseases. Clinical manifestations of this disease incorporate a wide variety of symptoms, ranging from neurological symptoms of extrapyramidal system to neuropsychiatric abnormalities of memory and concentration to movement disorders including Parkinsonism, chorea and tremors amongst others. Diagnostic criteria for this disease has been formulated after modifications from previous evidence and can be stated briefly, it consist of bilateral calcification of basal ganglia, progressive neurologic dysfunction, absence of biochemical abnormalities, absence of an infectious, traumatic or toxic cause and a significant family history. Imaging modalities for the diagnosis include CT, MRI, and plain radiography of skull. Other investigations include blood and urine testing for hematologic and biochemical indices. Disease is as yet incurable but management and treatment strategies mainly focus on symptomatic relief and eradication of causative factors; however certain evidence is present to suggest that early diagnosis and treatment can reverse the calcification process leading to complete recovery of mental functions. Families with a known history of Fahr\u2019s disease should be counseled prior to conception so that the birth of affected babies can be prevented. This review was written with the aim to remark on the current substantial evidence surrounding this disease.<\/jats:p>","DOI":"10.1186\/1750-1172-8-156","type":"journal-article","created":{"date-parts":[[2013,10,8]],"date-time":"2013-10-08T05:01:06Z","timestamp":1381208466000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":228,"title":["Fahr\u2019s syndrome: literature review of current evidence"],"prefix":"10.1186","volume":"8","author":[{"given":"Shafaq","family":"Saleem","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hafiz Muhammad","family":"Aslam","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maheen","family":"Anwar","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shahzad","family":"Anwar","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria","family":"Saleem","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anum","family":"Saleem","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Muhammad Asim Khan","family":"Rehmani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2013,10,8]]},"reference":[{"key":"657_CR1","unstructured":"Bilateral striopallidodentate calcinosis.http:\/\/www.orpha.net\/consor\/cgi-bin\/OC_Exp.php?Lng=GB&Expert=1980."},{"issue":"2","key":"657_CR2","doi-asserted-by":"publisher","first-page":"258","DOI":"10.1002\/mds.1049","volume":"16","author":"BV Manyam","year":"2001","unstructured":"Manyam BV, Walters AS, Narla KR: Bilateral striopallidodentate calcinosis: clinical characteristics of patients seen in a registry. 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