Fahr's Syndrome: Patologic Intracerebral Calcification

Authors

  • Dewa Gede Putra Angga Pradnyana General Practitioner, Palangka Raya General Hospital
  • Sarita Sharchis Neurologist, RSUD dr. Doris Sylvanus, Palangka Raya, Kalimantan Tengah

DOI:

https://doi.org/10.55175/cdk.v51i9.1167

Keywords:

Basal ganglia, intracerebral calcification, Fahr's syndrome

Abstract

Fahr’s syndrome is a collection of symptoms accompanied by symmetrical and bilateral abnormal calcification in the basal ganglia, thalamus, dentate nucleus, cerebral cortex, cerebellum, and hippocampus. Fahr syndrome can occur in young to middle-aged adults. Some of the most important etiologies are endocrine disorders, infections, and autoimmune diseases. The clinical symptoms are very diverse. Diagnostic criteria are established. One of the routine imaging examinations is a head CT scan. There is no specific therapy for Fahr syndrome, the treatment focuses on the symptoms and the underlying disease.

Downloads

Download data is not yet available.

References

Jaworski K, Styczynka M, Mandecka M, Walecki J, Kosior DA. Fahr syndrome – An important piece of a puzzle in the differential diagnosis of many diseases. Polish J Radiol. 2017;82:490-3. DOI: 10.12659/PJR.902024.

Donzuso G, Mostile G, Nicoletti A, Zappia M. Basal ganglia calcifications (Fahr’s syndrome): Related conditions and clinical features. Fondazione Societa Italiana di Neurologia 2019;40:2251-63. DOI: 10.1007/s10072-019-03998-x.

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. DOI: 10.1186/1750-1172-8-156.

Powers J, Joy K, Ruscio A, Lagast H. Prevalence and incidence of hypoparathyroidism in the United States using a large claims database. J Bone Mineral Res. 2013;28(12):2570-6. DOI: 10.1002/jbmr.2004.

Mendes EM, Meireles-Brandao L, Meira C, Morais N, Ribeiro C, Guerra D. Primary hypoparathyroidism presenting as basal ganglia calcification secondary to extreme hypocalcemia. Clinics and Practice 2018;8(1):1007. DOI: 10.4081/cp.2018.1007.

Tsai DR, Huang SH, Lin SH. Bilateral striopallidodentate calcinosis secondary to postsurgical hypoparathyroidism. BMJ Case Rep. 2013;13:2013. DOI: 10.1136/bcr-2013-200591.

Ramos EM, Oliveira J, Sobrido MJ, Coppola G. Primary familial brain calcification. GeneReviewsTM [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2023 [cited 2023 Dec 8]. Available from: http://www.ncbi.nlm.nih.gov/books/NBK1421/.

Bentancur JF, Gomez-Puerta JA, Llano JF, Lopez-Ochoa GA, Ramirez B, Martinez OD. Bilateral striopallidal calcinosis secondary to systemic lupus erithematosus. Radiology Case Rep. 2022;17(6):2257-61. DOI: 10.1016/j.radcr.2022.03.114.

Pistacchi M, Gioulis M, Sanson F, Marsala SZ. Fahr’s syndrome and clinical correlation: A case series and literature review. Folia Neuropathologica 2016;54(3):282-94. DOI: 10.5114/fn.2016.62538.

Ho M, Sharma R, El-Feky M. Fahr syndrome. Reference article, Radiopaedia.org [Internet]. 2023 [cited Dec 8]. Available from: https://radiopaedia.org/articles/7289.

Perugula ML, Lippmann S. Differential diagnosis – Fahr’s disease or fahr’s syndrome? Innovation in Clin Neurosci. 2016;13(7-8):45-6.

Agarwal R, Lahiri D, Biswas A, Mukhopadhyay J, Maity P, Roy MK. A rare cause of seizures, parkinsonian, and cerebellar signs: Brain calcinosis secondary to thyroidectomy. North Am J Med Sci. 2014;6(10):540-2. DOI: 10.4103/1947-2714.143287.

Subbiah S, Natarajan V, Bhagadurshah RR. Fahr’s disease and hypoparathyroidism – A missing link. Neurol India. 2022;70:1154-6. DOI: 10.4103/0028-3886.349669.

Published

10-09-2024

How to Cite

Angga Pradnyana, D. G. P., & Sharchis, S. (2024). Fahr’s Syndrome: Patologic Intracerebral Calcification. Cermin Dunia Kedokteran, 51(9), 512–515. https://doi.org/10.55175/cdk.v51i9.1167