Abstract
Fahr sendromu; bazal ganglionlar, serebellar dentat nukleus ve sentrum semiovalede kalsifikasyon
görülmesi ile karakterize bir durumdur. Klinik bulgular sıklıkla parkinsonizm, distoni, tremor, kore,
ataksiye ek olarak demans ve duygu durum bozukluklarıdır. Biz de kasılma şikayeti ile başvuran Fahr
sendromu saptadığımız bir olguyu klinik, laboratuar ve görüntüleme bulguları ile sunmayı amaçladık.
Kasılma şikayeti ile başvuran 74 yaşında erkek hastanın muayenesi normal olarak değerlendirildi.
Bilgisayarlı beyin tomografi (BBT) incelemesinde her iki serebellar hemisfer ve bazal ganglionlar hizasında
yaygın simetrik kalsifikasyon tespit edildi. Bazal ganglionlar, serebellum ve derin kortikal yapılarda
kraniyal BT ile saptanan kalsifikasyonların herhangi bir nedene bağlanamadığı durumlarda Fahr sendromu
ayırıcı tanıda düşünülmelidir.
References
- 1) Rastogi R, Norman J, Ladenson BPW. Calcifications of the Basal Ganglia in Chronic Hypoparathyroidizm. The Journal of Clinical Endocrinology and Metabolism 2003; 88(4): 1476-7.
- 2) Özer T, Savranlar A, Bayraktaroğlu T, Asil K. İntracranial calcifications due to hypoparathyroidism after thyroidectomy. Nörol Bil Dergisi. 2004; 21(4): 4-7.
- 3) Lang C, Huk W, Pichl J. Comparison of extensive brain calcification in postoperative hypoparathyoidism on CT and NMR scan. Neuroradiology 1989; 31(1): 29- 32.
- 4) Beall SS, Patten BB, Mallette L, Jankovic J. Abnormal systemic metobolism of iron, porphirin and calcium in Fahr's seyndrome. Annals of neurology 1989; 26 (4): 569- 75.
- 5) Manyam BV. What is and what is not 'Fahr's disease'. Parkinsonism and Related Disorders 2005; 11(2): 73–80.
- 6) Arranz PM, Ergueta MP, Gonzalez SE, Maranon CA. Fahr's disease and Hypocalcemic Syndromes: Presentation of a Clinical Case. An Med Interna 1992; 9(10): 495-7.
- 7) Scotti G, Scialfa G. MR imaging in Fahr disease. J Comput Asist Tomogr 1985; 9(4): 790-2.
- 8) Babbitt DP, Tang T, Dobbs J, Berk R. Idiopathic familial cerebrovascular ferrocalcinosis (Fahr's disease) and review of differential diagnosis of intracranial calcification in children. Am J Roentgenol Radium Ther Nucl Med 1969; 105(2): 352-8.
- 9) Lam JSP, Fong SYY, Yiu GC, Wing YK. Fahr's disease: a differential diagnosis of frontal lobe syndrome. Hong Kong Med J 2007; 13(1): 75-7.
- 10) Konig P. Psychopathological alterations in cases of symmetrical basal ganglia sclerosis. Biol Psychiatry 1989; 25 (4): 459-68.
Abstract
Fahr 's syndrome is a condition which characterized by the calcification in basal ganglia, cerebellar dentate
nucleus and centrum semiovale. Clinical findings are often parkinsonism , dystonia, tremor, chorea ,ataxia
in addition to mood disorders and dementia. We aimed to report a case of Fahr's syndrome which is
presenting with complaint of contraction clinical, laboratory and imaging findings. Admitted with complaint
of contraction 74 -year-old male patient's physical examination was normal. Computed tomography
imaging was detected bilaterally symmetrical calcification in both cerebellar hemispheres and basal
ganglia. Fahr's syndrome should be considered in the differential diagnosis of unable to connect to any
reason detected calcifications in the cranial CTin basal ganglia, cerebellum and deep cortical structures.
References
- 1) Rastogi R, Norman J, Ladenson BPW. Calcifications of the Basal Ganglia in Chronic Hypoparathyroidizm. The Journal of Clinical Endocrinology and Metabolism 2003; 88(4): 1476-7.
- 2) Özer T, Savranlar A, Bayraktaroğlu T, Asil K. İntracranial calcifications due to hypoparathyroidism after thyroidectomy. Nörol Bil Dergisi. 2004; 21(4): 4-7.
- 3) Lang C, Huk W, Pichl J. Comparison of extensive brain calcification in postoperative hypoparathyoidism on CT and NMR scan. Neuroradiology 1989; 31(1): 29- 32.
- 4) Beall SS, Patten BB, Mallette L, Jankovic J. Abnormal systemic metobolism of iron, porphirin and calcium in Fahr's seyndrome. Annals of neurology 1989; 26 (4): 569- 75.
- 5) Manyam BV. What is and what is not 'Fahr's disease'. Parkinsonism and Related Disorders 2005; 11(2): 73–80.
- 6) Arranz PM, Ergueta MP, Gonzalez SE, Maranon CA. Fahr's disease and Hypocalcemic Syndromes: Presentation of a Clinical Case. An Med Interna 1992; 9(10): 495-7.
- 7) Scotti G, Scialfa G. MR imaging in Fahr disease. J Comput Asist Tomogr 1985; 9(4): 790-2.
- 8) Babbitt DP, Tang T, Dobbs J, Berk R. Idiopathic familial cerebrovascular ferrocalcinosis (Fahr's disease) and review of differential diagnosis of intracranial calcification in children. Am J Roentgenol Radium Ther Nucl Med 1969; 105(2): 352-8.
- 9) Lam JSP, Fong SYY, Yiu GC, Wing YK. Fahr's disease: a differential diagnosis of frontal lobe syndrome. Hong Kong Med J 2007; 13(1): 75-7.
- 10) Konig P. Psychopathological alterations in cases of symmetrical basal ganglia sclerosis. Biol Psychiatry 1989; 25 (4): 459-68.
Details
| Primary Language | Turkish |
|---|---|
| Journal Section | Case Report |
| Authors | |
| Publication Date | August 29, 2016 |
| Submission Date | February 26, 2016 |
| Acceptance Date | April 2, 2016 |
| Published in Issue | Year 2016 Volume: 13 Issue: 2 |
Harran Üniversitesi Tıp Fakültesi Dergisi / Journal of Harran University Medical Faculty