Fahr Hastalığı İdiyopatik Bazal Gangliyon Kalsifikasyonu

Öz

Fahr hastalığı bilateral striyopallidodentat kalsinozis tab- losudur. Sık rastlanan klinik bulgular nöropsikiyatrik semptomlar, nöbetler, demans, konuşma bozuklukları, par- kinson, distoni, kore ve ataksiyi içermektedir. Kalsiyum metabolizması bozuklukları, sistemik hastalıklar, radyas- yon anoksi, toksinler, ensefalitler gibi bir takım bozukluk- ların bu hastalığa neden olabileceği ileri sürülmüştür. Bu makalede on yaşında Fahr sendromlu bir hasta klinik, endokrinolojik ve radyolojik yönleriyle sunulmaktadır

Anahtar Kelimeler

• Fahr hastalığı,bazal gangliyon kalsifikasyonu,bilgisayarlı tomografi

Fahr Disease Idiopathic Basal Ganglia Calcification

Öz

Fahr disease is a disorder of bilateral striopallidodentate calcinosis. Common clinical findings include neuropsychi- atric symptomes, seizures, dementia, speech difficulties, parkinsonism, distonia, chorea, and ataxia. It’s suggested that calcium metabolism disorders, systemic diseases, radiation, anoxia, toxins, and encephalits etc.may cause this disease. In this paper a 10-year old patient with Fahr syndrome is presented with relevant clinical, endocrinolo- gical and radiological aspects

Anahtar Kelimeler

• Fahr disease,basal ganglia calcification,computed tomography

Kaynakça

1. 1. Hanağası H, Hanağası F, Bilgiç B. Bilateral striopallidodentat kalsinozisin klinik spektrumu. Türk Nöroloji Dergisi 2004;5:430-7.
2. 2. Yoshikawa H, Abe T. Transient parkinsonism in bilateral striopallidodentate calcinosis. Pediatr Neurol 2003;29:75-7. http://dx.doi.org/10.1016/S0887-8994(03)00049-3
3. 3. Lauterbach EC. Fahr’s Syndrome. In: Psychiatric management in neurological disease. 1st ed. Washington: American Psychiatric Press; 2000:137-8.
4. 4. Cummings JL, Gosenfeld LF, Haulihan JP, McCaffrey T. Neuropsychiatric disturbances associated with idiopathic calcification of the basal ganglia. Biol Psychiatry 1983;18(5):591- 601. PMid:6860732
5. 5. Lester J, Zuniga C, Diaz S, Rugilo C, Micheli F. Diffuse intracranial calsinosis: Fahr disease. Arch Neurol 2006;63: 1806-7. http://dx.doi.org/10.1001/archneur.63.12.1806 PMid:17172625
6. 6. Lauterbach EC. Fahr’s Syndrome. In: Psychiatric management in neurological disease. 1st ed. Washington: American Psychiatric Press; 2000:140-1.
7. 7. Kobayashi S, Yamadori I, Miki H, Ohmori M. Idiopathic nonarteriosclerotic cerebral calcification (Fahr’s disease): an electron microscopic study. Acta Neuropathol (Berl) 1987;73(1):62-6. http://dx.doi.org/10.1007/BF00695503 PMid:3604574
8. 8. Bouras C, Giannakopoulos P, Good PF, Hsu A, Hof PR, Perl DP. A Laser microprobe mass analysis of the trace elements in brain mineralizations and capillaries in Fahr’s disea se. Acta Neurol 1989;26:569-75.

9. 9. Kazis AD. Contribution of CT scan to the diagnosis of Fahr’s syndrome. Acta Neurol Scand 1985;71:206-11. http://dx.doi.org/10.1111/j.1600-0404.1985.tb03190.x
10. 10. Ökten Aİ, Ergün R. Fahr’s Hastalığı (İerleyici İdiyopatik Strio-Pallido-Dentat Kalsinozis): Vaka Sunumu. Türk Serebrovasküler Hastalıklar Dergisi 2003;9(2):77-9.
11. 11. Baptista MV, Vale J, Leitao O. Strio-pallido-dentat calcifications. Presse Med 1995;24(28):1301-4. PMid:7501622
12. 12. Rossi M, Morena M, Zanardi M. Calcification of the basal ganglia and Fahr disease. Report of two clinical cases and review of the literature. Recenti Prog Med 1993;84(3):192-8. PMid:8465101