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Fahr's Sendromu: İki Olgunun Raporları

Year 2007, Volume: 12 Issue: 1, 70 - 72, 01.02.2007

Mehmet Şenoğlu Deniz Tuncel F.özlem Orhan Zafer Yuksel Mustafa Gokçe

Abstract

Fahr sendromu esas olarak ekstrapiramidal bulguların ve aynı zaman dametabolik, biyokimyasal, nöroradyolijik ve nöropsikiyatrik durumların birlikte prezente oldugu nadir bir klınik antitedir. Genellikle bilateralin trakraniyal kalsifikasyonlar insidental radyolijik bulgudur. Bu calışmada, biz iki fahr sendromlu olguyu literatur ışıgında tartışdık. Olgulardan birisi idiyopatik ve digeri hipoparatiroidizme sekonder oluşan olgu idi. Klinik olarak olgulardan biri nöbet ile ve digeri denge bozuklugu ile prezente oldu. Heriki hastanin kranial bilgisayarlı tomografisinde talamus, bazal ganglionlar ve serebellumda intrakranyaldiffuz bilateral kalsifikasyonlar mevcut idi. Fahr sendromu nadir karsılaşılan bir antite olmasına ragmen, anormal intrakraniyal kalsifikasyonlu vakaların ayırıcı tanısında diger familyal, konjenital ve metabolik hastaliklar ve sendromlar ile birlikte göz önünde bulundurulmalıdır. ©2007, Fırat Üniversitesi, Tıp Fakültesi

Keywords

Fahr’s sendromu hipoparatiroidizm epilepsi bilateral intrakranial kalsifikasyonlar

References

  • 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; 241:381-384.
  • Beall SS, Patten BM, Mallette L, Jankovic J. Abnormal systemic metabolism of iron, porphyrin, and calcium in Fahr's syndrome. Ann Neurol 1989; 26:569-575.
  • Brannan TS, Burger AA, Chaudhary MY. Bilateral basal ganglia calcifications visualised on CT scan. J Neurol Neurosurg Psychiatry 1980; 14: 403-406.
  • Fahr T: Idiopathische Verkalkungen der HirngefaBe. Zentralbiatt Allgemeine für Pathologie 1930; 50: 129-133.
  • Fenelon G, Gray F, Paillard F, Thibierge M, Mahieux F, Guillani A. A prospective study of patients with CT detected pallidal calcifications. J Neurol Neurosurg Psychiatry 1993; 56:622-625.
  • Harati Y, Jackson JA, Benjamin E. Adult onset idiopathic familial brain calcifications. Arch Intern Med 1984; 144:2425- 2427.
  • Harrington MG, Macpherson P, Mcintosh WB, Allam BF, Bone I. The significance of the incidental finding of basal ganglia calcification on computed tomography. J Neurol Neurosurg Psychiatry 1981; 44:1168-1170.
  • Hempel A, Henze M, Berghoff C, Garcia N, Ody R, Schroder J. PET findings and neuropsychological deficits in a case of Fahr's disease. Psychiatry Res 2001; 30 108: 133-40.
  • Taxer F, Haller R, Konig P. Clinical early symptoms and CT findings in Fahr syndrome Nervenarzt 1986; 57: 583-588
  • Kowdley KV, Coul BM, Orwall ES. Cognitive impairment and intracranial calcification in chronic hypoparathyroidism. Am J Med Sci 1999; 317:273-277.
  • Manyam BV, Bhatt MH, Moore WD, Devleschoward AB, Anderson DR, Calne DB. Bilateral striopallidodentate calcinosis: Cerebrospinal fluid, imaging, and electrophysiological studies. Ann Neurol 1992; 31:379-384.
  • Manyam BV, Walters AS, Keller LA, Ghobrial M. Parkinsonism associated with autosomal dominant bilateral striopallidodentate calcinosis. Pakinsonism and Related Disorders 2001; 7:289-295.
  • Manyam BV. Bilateral strio-pallido-dentate calcinosis. proposed classifications of genetic and secondary causes. Mov Disord 1990; 5:94-97
  • Shigeyuki O. Imaging of bilateral striopallidodentate calcinosis. Clinical Nuclear Medicine 2002; 27: 721-724.
  • Stellamor K, Stellamor W. Roentgen diagnosis of Fahr’s disease. Rontgenblatter 1983; 36: 194-196.
  • Trautner RJ, Cummings JL, Read SL, Benson DF. Idiopathic basal ganglia calcification and organic mood disorder. Am J Psychiatry 1988; 145:350-353.
  • Kabul Tarihi: 06.06.2006

Fahr's Syndrome: A Report of Two Cases

Year 2007, Volume: 12 Issue: 1, 70 - 72, 01.02.2007

Mehmet Şenoğlu Deniz Tuncel F.özlem Orhan Zafer Yuksel Mustafa Gokçe

Abstract

Fahr's syndrome is a rare clinical entity that presents mainly with extrapyramidal signs and accompanied with metabolic, biochemical, neuroradiological and neuro-psychiatric situations at the same time. Bilateral intracranial calcifications are usually encountered as an incidental radiological finding. In this study, we discuss two cases of Fahr's syndrome in the light of the literature; one of them idiopathic and the other one occurring secondary to hypoparathyroidism. Clinically, one of the cases presented with seizures and the other one with imbalance of the coordination system. Cranial computerized scans of both patients revealed intracranial diffuse bilateral calcifications in thalamus, basal ganglia and cerebellum. Fahr's syndrome, although encountered rarely, should also be taken into account in the differential diagnosis of cases with abnormal intracranial calcifications along with other familial, congenital and metabolic diseases and syndromes.©2007, Firat University, Medical Faculty

Keywords

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References

  • 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; 241:381-384.
  • Beall SS, Patten BM, Mallette L, Jankovic J. Abnormal systemic metabolism of iron, porphyrin, and calcium in Fahr's syndrome. Ann Neurol 1989; 26:569-575.
  • Brannan TS, Burger AA, Chaudhary MY. Bilateral basal ganglia calcifications visualised on CT scan. J Neurol Neurosurg Psychiatry 1980; 14: 403-406.
  • Fahr T: Idiopathische Verkalkungen der HirngefaBe. Zentralbiatt Allgemeine für Pathologie 1930; 50: 129-133.
  • Fenelon G, Gray F, Paillard F, Thibierge M, Mahieux F, Guillani A. A prospective study of patients with CT detected pallidal calcifications. J Neurol Neurosurg Psychiatry 1993; 56:622-625.
  • Harati Y, Jackson JA, Benjamin E. Adult onset idiopathic familial brain calcifications. Arch Intern Med 1984; 144:2425- 2427.
  • Harrington MG, Macpherson P, Mcintosh WB, Allam BF, Bone I. The significance of the incidental finding of basal ganglia calcification on computed tomography. J Neurol Neurosurg Psychiatry 1981; 44:1168-1170.
  • Hempel A, Henze M, Berghoff C, Garcia N, Ody R, Schroder J. PET findings and neuropsychological deficits in a case of Fahr's disease. Psychiatry Res 2001; 30 108: 133-40.
  • Taxer F, Haller R, Konig P. Clinical early symptoms and CT findings in Fahr syndrome Nervenarzt 1986; 57: 583-588
  • Kowdley KV, Coul BM, Orwall ES. Cognitive impairment and intracranial calcification in chronic hypoparathyroidism. Am J Med Sci 1999; 317:273-277.
  • Manyam BV, Bhatt MH, Moore WD, Devleschoward AB, Anderson DR, Calne DB. Bilateral striopallidodentate calcinosis: Cerebrospinal fluid, imaging, and electrophysiological studies. Ann Neurol 1992; 31:379-384.
  • Manyam BV, Walters AS, Keller LA, Ghobrial M. Parkinsonism associated with autosomal dominant bilateral striopallidodentate calcinosis. Pakinsonism and Related Disorders 2001; 7:289-295.
  • Manyam BV. Bilateral strio-pallido-dentate calcinosis. proposed classifications of genetic and secondary causes. Mov Disord 1990; 5:94-97
  • Shigeyuki O. Imaging of bilateral striopallidodentate calcinosis. Clinical Nuclear Medicine 2002; 27: 721-724.
  • Stellamor K, Stellamor W. Roentgen diagnosis of Fahr’s disease. Rontgenblatter 1983; 36: 194-196.
  • Trautner RJ, Cummings JL, Read SL, Benson DF. Idiopathic basal ganglia calcification and organic mood disorder. Am J Psychiatry 1988; 145:350-353.
  • Kabul Tarihi: 06.06.2006
There are 17 citations in total.

Details

Primary Language Turkish
Journal Section Articles
Authors

Mehmet Şenoğlu This is me

Deniz Tuncel This is me

F.özlem Orhan This is me

Zafer Yuksel This is me

Mustafa Gokçe This is me

Publication Date February 1, 2007
Published in Issue Year 2007 Volume: 12 Issue: 1

Cite

APA Şenoğlu, M., Tuncel, D., Orhan, F., Yuksel, Z., et al. (2007). Fahr's Sendromu: İki Olgunun Raporları. Fırat Tıp Dergisi, 12(1), 70-72.
AMA Şenoğlu M, Tuncel D, Orhan F, Yuksel Z, Gokçe M. Fahr's Sendromu: İki Olgunun Raporları. Fırat Tıp Dergisi. February 2007;12(1):70-72.
Chicago Şenoğlu, Mehmet, Deniz Tuncel, F.özlem Orhan, Zafer Yuksel, and Mustafa Gokçe. “Fahr'S Sendromu: İki Olgunun Raporları”. Fırat Tıp Dergisi 12, no. 1 (February 2007): 70-72.
EndNote Şenoğlu M, Tuncel D, Orhan F, Yuksel Z, Gokçe M (February 1, 2007) Fahr's Sendromu: İki Olgunun Raporları. Fırat Tıp Dergisi 12 1 70–72.
IEEE M. Şenoğlu, D. Tuncel, F. Orhan, Z. Yuksel, and M. Gokçe, “Fahr's Sendromu: İki Olgunun Raporları”, Fırat Tıp Dergisi, vol. 12, no. 1, pp. 70–72, 2007.
ISNAD Şenoğlu, Mehmet et al. “Fahr'S Sendromu: İki Olgunun Raporları”. Fırat Tıp Dergisi 12/1 (February 2007), 70-72.
JAMA Şenoğlu M, Tuncel D, Orhan F, Yuksel Z, Gokçe M. Fahr's Sendromu: İki Olgunun Raporları. Fırat Tıp Dergisi. 2007;12:70–72.
MLA Şenoğlu, Mehmet et al. “Fahr'S Sendromu: İki Olgunun Raporları”. Fırat Tıp Dergisi, vol. 12, no. 1, 2007, pp. 70-72.
Vancouver Şenoğlu M, Tuncel D, Orhan F, Yuksel Z, Gokçe M. Fahr's Sendromu: İki Olgunun Raporları. Fırat Tıp Dergisi. 2007;12(1):70-2.