Fahr’s Syndrome
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Abstract
Fahr’s syndrome is a rare neurodegenerative disorder. Bilateral basal ganglia and dentate nuclei of the cerebellum are involved in this disorder and deposition of calcium is the hallmark of this syndrome. It has been recognized as a sporadic or inherited disease with variable presentations. In this article, we report a 40-year-old man with incidentally discovered brain calcifcation as the sole manifesation of Fahr’s syndrome. A 40-year-old male without any comorbidities was presented with brain calcifcation that was found incidentally. Brain imaging revealed symmetric calcifcations in bilateral basal ganglia, internal capsules, and cerebral white matter. This pattern of calcifcation is highly suspicious of Fahr’s syndrome. Other pathologic processes that could lead to intracranial calcifcation were excluded. We present a young patient with sporadic and asymptomatic Fahr’s syndrome after ruling out abnormalities of known calcium metabolism and developmental defects.
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[2] Saleem S, Aslam HM, Anwar M, Anwar S, Saleem M, Saleem A, et al. Fahr’s syndrome: Literature review of current evidence. Orphanet Journal of Rare Diseases. 2013; 8(1):156. [DOI:10.1186/1750-1172-8-156] [PMID] [PMCID]
3] Mufaddel AA, Al Hassani GA. Familial idiopathic basal ganglia calcification (Fahr’s disease). Neurosciences. 2014; 19(3):171-7. [PMID] [PMCID]
[4] Faye AD, Gawande S, Tadke R, Kirpekar VC, Bhave SH. A case of psychosis due to Fahr’s syndrome and response to behavioral disturbances with risperidone and oxcarbazepine. Indian Journal of Psychiatry. 2014; 56(2):188-90. [DOI:10.4103/0019-5545.130506] [PMID] [PMCID]
[5] Geschwind DH, Loginov M, Stern JM. Identification of a locus on chromosome 14q for idiopathic basal ganglia calcification (Fahr disease). The American Journal of Human Genetics. 1999; 65(3):764-72. [DOI:10.1086/302558] [PMID] [PMCID]
[6] Manyam BV. What is and what is not ‘Fahr’s disease’. Parkinsonism & Related Disorders. 2005; 11(2):73-80. [DOI:10.1016/j. parkreldis.2004.12.001] [PMID]
[7] Mrunal Milind D, Chatterjee A. Fahr syndrome: A rare case report. Clinical Advances in Periodontics. 2015; 5(4):223-8. [DOI:10.1902/cap.2014.130093]
[8] Manyam BV, Walters AS, Narla KR. Bilateral striopallidodentate calcinosis: Clinical characteristics of patients seen in a registry. Movement Disorders. 2001; 16(2):258-64. [DOI:10.1002/mds.1049] [PMID]
[9] Nicolas G, Guillin O, Borden A, Bioux S, Lefaucheur R, Hannequin D. Psychosis revealing familial idiopathic basal ganglia calcification. General Hospital Psychiatry. 2013; 35(5):575. e3-e5. [DOI:10.1016/j.genhosppsych.2012.09.008]
[10] Doğan O, Meydan G, Semiz M, Yıldırım O, Yontar G. Fahr hastalığı: Olgu sunumu. Noropsikiyatri Arsivi. 2011; 1(48):82- 4. [DOI:10.4274/npa.y5580]
[11] Viteva E, Djurkova A. Fahr’s disease with epilepsy, deafness, schizophreniform psychosis and autoimmune polymyositis: A case report. Rare Diseases and Orphan Drugs. 2015; 2(2):34-7.
[12] Shirahama M, Akiyoshi J, Ishitobi Y, Tanaka Y, Tsuru J, Matsushita H, et al. A young woman with visual hallucinations, delusions of persecution and a history of performing arson with possible three‐generation Fahr disease. Acta Psychiatrica Scandinavica. 2010; 121(1):75-7. [DOI:10.1111/j.1600- 0447.2009.01423.x] [PMID]
[13] Shouyama M, Kitabata Y, Kaku T, Shinosaki K. Evaluation of regional cerebral blood flow in Fahr disease with schizophrenia-like psychosis: A case report. American Journal of Neuroradiology. 2005; 26(10):2527-9. [PMID]
[14] Cartier L, Passig C, Gormaz A, Lopez J. Neuropsychological and neurophysiological features of Fahr’s disease. Revista médica de Chile. 2002; 130(12):1383-90. [PMID]
[15] Modrego P, Mojonero J, Serrano M, Fayed N. Fahr’s syndrome presenting with pure and progressive presenile dementia. Neurological Sciences. 2005; 26(5):367-9. [DOI:10.1007/s10072-005-0493-7] [PMID]
[16] Hoque M, Siddiqui M, Arafat Y, Khan S, Rahman K, Mondol B, et al. Fahr’s disease: A very rare cause of epilepsy. Mymensingh Medical Journal. 2010; 19(1):127-9. [PMID]
[17] Loeb JA. Functional improvement in a patient with cerebral calcinosis using a bisphosphonate. Movement Disorders. 1998; 13(2):345-9. [DOI:10.1002/mds.870130225] [PMID]
[18] Loeb JA, Sohrab SA, Huq M, Fuerst DR. Brain calcifications induce neurological dysfunction that can be reversed by a bone drug. Journal of the Neurological Sciences. 2006; 243(1):77-81. [DOI:10.1016/j.jns.2005.11.033] [PMID]
[2] Saleem S, Aslam HM, Anwar M, Anwar S, Saleem M, Saleem A, et al. Fahr’s syndrome: Literature review of current evidence. Orphanet Journal of Rare Diseases. 2013; 8(1):156. [DOI:10.1186/1750-1172-8-156] [PMID] [PMCID]
3] Mufaddel AA, Al Hassani GA. Familial idiopathic basal ganglia calcification (Fahr’s disease). Neurosciences. 2014; 19(3):171-7. [PMID] [PMCID]
[4] Faye AD, Gawande S, Tadke R, Kirpekar VC, Bhave SH. A case of psychosis due to Fahr’s syndrome and response to behavioral disturbances with risperidone and oxcarbazepine. Indian Journal of Psychiatry. 2014; 56(2):188-90. [DOI:10.4103/0019-5545.130506] [PMID] [PMCID]
[5] Geschwind DH, Loginov M, Stern JM. Identification of a locus on chromosome 14q for idiopathic basal ganglia calcification (Fahr disease). The American Journal of Human Genetics. 1999; 65(3):764-72. [DOI:10.1086/302558] [PMID] [PMCID]
[6] Manyam BV. What is and what is not ‘Fahr’s disease’. Parkinsonism & Related Disorders. 2005; 11(2):73-80. [DOI:10.1016/j. parkreldis.2004.12.001] [PMID]
[7] Mrunal Milind D, Chatterjee A. Fahr syndrome: A rare case report. Clinical Advances in Periodontics. 2015; 5(4):223-8. [DOI:10.1902/cap.2014.130093]
[8] Manyam BV, Walters AS, Narla KR. Bilateral striopallidodentate calcinosis: Clinical characteristics of patients seen in a registry. Movement Disorders. 2001; 16(2):258-64. [DOI:10.1002/mds.1049] [PMID]
[9] Nicolas G, Guillin O, Borden A, Bioux S, Lefaucheur R, Hannequin D. Psychosis revealing familial idiopathic basal ganglia calcification. General Hospital Psychiatry. 2013; 35(5):575. e3-e5. [DOI:10.1016/j.genhosppsych.2012.09.008]
[10] Doğan O, Meydan G, Semiz M, Yıldırım O, Yontar G. Fahr hastalığı: Olgu sunumu. Noropsikiyatri Arsivi. 2011; 1(48):82- 4. [DOI:10.4274/npa.y5580]
[11] Viteva E, Djurkova A. Fahr’s disease with epilepsy, deafness, schizophreniform psychosis and autoimmune polymyositis: A case report. Rare Diseases and Orphan Drugs. 2015; 2(2):34-7.
[12] Shirahama M, Akiyoshi J, Ishitobi Y, Tanaka Y, Tsuru J, Matsushita H, et al. A young woman with visual hallucinations, delusions of persecution and a history of performing arson with possible three‐generation Fahr disease. Acta Psychiatrica Scandinavica. 2010; 121(1):75-7. [DOI:10.1111/j.1600- 0447.2009.01423.x] [PMID]
[13] Shouyama M, Kitabata Y, Kaku T, Shinosaki K. Evaluation of regional cerebral blood flow in Fahr disease with schizophrenia-like psychosis: A case report. American Journal of Neuroradiology. 2005; 26(10):2527-9. [PMID]
[14] Cartier L, Passig C, Gormaz A, Lopez J. Neuropsychological and neurophysiological features of Fahr’s disease. Revista médica de Chile. 2002; 130(12):1383-90. [PMID]
[15] Modrego P, Mojonero J, Serrano M, Fayed N. Fahr’s syndrome presenting with pure and progressive presenile dementia. Neurological Sciences. 2005; 26(5):367-9. [DOI:10.1007/s10072-005-0493-7] [PMID]
[16] Hoque M, Siddiqui M, Arafat Y, Khan S, Rahman K, Mondol B, et al. Fahr’s disease: A very rare cause of epilepsy. Mymensingh Medical Journal. 2010; 19(1):127-9. [PMID]
[17] Loeb JA. Functional improvement in a patient with cerebral calcinosis using a bisphosphonate. Movement Disorders. 1998; 13(2):345-9. [DOI:10.1002/mds.870130225] [PMID]
[18] Loeb JA, Sohrab SA, Huq M, Fuerst DR. Brain calcifications induce neurological dysfunction that can be reversed by a bone drug. Journal of the Neurological Sciences. 2006; 243(1):77-81. [DOI:10.1016/j.jns.2005.11.033] [PMID]
| Files | ||
| Issue | Vol 3 No 3 (2018): Summer | |
| Section | Case Report(s) | |
| Keywords | ||
| Fahr’s syndrome Basal ganglia calcification Brain Magnetic Resonance Imaging (MRI) | ||
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How to Cite
1.
Saadat P, Hejazian T, Mohseni Ahangar R. Fahr’s Syndrome. CRCP. 2019;3(3):73-77.
