Fahr's Disease

Yıl 2013, Cilt: 3 Sayı: 2, 133 - 135, 01.06.2013

Tezcan Çalışkan Sabri Grübüz

Öz

Fahr's disease refers to sporadic or familial idiopathic basal ganglia, cerebral and cerebellar calcification. Patients may remain symptom-free but approximately two-thirds of the patients are symptomatic. Typical presentation starts in the 4th to 5th decades of life. Patients present with pyramidal, extrapyramidal, cerebellar, psychiatric and cognitive manifestations. Various diagnostic studies can be used to detect Fahr's disease and associated abnormalities. There is no specific treatment other than symptomatic support. In this review, clinical features and different types of presentations of Fahr's disease are discussed under the light of current literature.

Anahtar Kelimeler

Fahr's disease, Clinical features, Presentation

Fahr Hastalığı

Öz

Fahr hastalığı, ailesel veya sporadik olabilen, idiopatik bazal ganglia, serebral ve serebellar kalsifikasyonla seyreden bir hastalıktır. Hastalar hiçbir belirti vermeyebilir, ancak hastaların yaklaşık olarak üçte ikisi semptomatiktir. Tipik prezentasyon dördüncü ve beşinci dekatlarda başlar. Hastalar piramidal, ekstrapiramidal, serebellar, psikiyatrik ve kognitif bulgularla başvurur. Bir çok tanısal çalışma hem Fahr hastalığının hem de ilişkili hastalıkların tanısını koymada kullanılabilir. Semptomatik tedavi dışında spesifik bir tedavisi yoktur. Bu derlemede Fahr hastalığının klinik özellikleri farklı prezentasyon şekilleri mevcut literatür eşliğinde incelenmiştir

Anahtar Kelimeler

Fahr hastalığı, Klinik özellikler, Prezentasyon

Kaynakça

• Ashtari F, Fatehi F. Fahr's disease: variable presentations in a family. Neurol Sci 31:665-667, 2010 (Epub)
• Manyam BV. What is and what is not 'Fahr's disease'. Parkinsonism Relat Disord 11:73-80, 2005
• Al-Jehani Hosam , Ajlan Abdulrazag , Sinclair David. Fahr's disease Presenting with Aneurysmal Subarachnoid Hemorrhage. J Clin Imaging Sci 2:2730, 2012
• Faria AV, Pereira IC, Nanni L. Computerized tomography findings in Fahr's syndrome. Arq Neuropsiquiatr 2004 62:789-792, 2004 (Epub)
• Hoque MA, Siddiqui MR, Arafat Y, Khan SU, Rahman KM, Mondol BA, Mohammad QD. Fahr's disease: a very rare cause of epilepsy. Mymensingh Med J 19:127-129, 2010
• Benke T, Karner E, Seppi K, Delazer M, Marksteiner J, Donnemiller E. Subacute dementia and imaging correlates in a case of Fahr's disease. J Neurol Neurosurg Psychiatry 75:1163-1165, 2004
• Lam JS, Fong SY, Yiu GC, Wing YK. Fahr's disease: a differential diagnosis of frontal lobe syndrome. Hong Kong Med J Feb;13:75-77, 2007
• Lazăr M, Ion DA, Streinu-Cercel A, Bădărău AI. Fahr's syndrome: diagnosis issues in patients with unknown family history of disease. Rom J Morphol Embryol 50:425-428, 2009
• Uygur GA, Liu Y, Hellman RS, Tikofsky RS, Collier BD. Evaluation of regional cerebral blood flow in massive intracerebral calcifications. J Nucl Med 36:610-612, 1995
• Simone O, Tortorella C, Antonaci G, Antonaci S. An unusual case of transient loss of consciousness: The Fahr's syndrome. Recenti Prog Med 99:93-96, 2008
• Victor M RA. Calcification of vessels in basal ganglia and cerebellum (hypoparathyroidism and Fahr syndrome), in Principles of neurology. RA Victor M, editor. New York: McGraw-Hill; 2001. p. 1032-1033. Cummings JL, Gosenfeld LF, Houlihan JP, McCaffrey T. Neuropsychiatric disturbances associated with idiopathic calcification of the basal ganglia. Biol Psychiatry 1983; 18:591-601.
• Koller WC, Cochran JW, Klawans HL. Calcification of the basal ganglia: computerized tomography and clinical correlation. Neurology 1979;29:328-33.
• Avrahami E, Cohn DF, Feibel M, Tadmor R. MRI demonstration and CT correlation of the brain in patients with idiopathic intracerebral calcification. J Neurol 1994;241:381-4.
• Hozumi I, Kohmura A, Kimura A, Hasegawa T, Honda A, Hayashi Y, Hashimoto K, Yamada M, Sakurai T, Tanaka Y, Satoh M, Inuzuka T. High Levels of Copper, Zinc, Iron and Magnesium, but not Calcium, in the Cerebrospinal Fluid of Patients withFahr's Disease. Case Rep Neurol. 2010 May 7;2(2):46-51.
• Berendes K, Dorstelmann D. Unsuccessful treatment with levodopa of a parkinsonian patient with calcification of the basal ganglia. J Neurol 1978;218:51-4.
• Klawans HL, Lupton M, Simon L. Calcification of the basal ganglia as a cause of levodopa-resistant parkinsonism. Neurology 1976;26:221-5.