You are here

Home » Content

NADİR BİR HİPOKALSEMİ NEDENİ: FAHR HASTALIĞI

RARE CAUSE OF HYPOCALCEMİA: FAHR'S DISEASE

Journal Name:

  • İstanbul Tıp Fakültesi Dergisi

Publication Year:

  • 2010

Key Words:

Keywords (Original Language):

Author Name
Abstract (2. Language): 
Fahr’s disease is a rare neurological disorder characterized by bilateral basal ganglia calcinosis. Calcifications cause obliteration in micro vessels, neuronal degeneration and gliosis accumulating bilaterally at the basal ganglia, thalamus, centrum semiovale and the dentate nucleus. The most used diagnostic method is cranial tomography to determine the calcifications. Fahr’s disease presents sporadically, inheritedly or secondarily (tumor, inflammation, hypoxia, endocrine, toxic, metabolic, degenerative, malabsorbtive). We want to emphasize the consequence of this syndrome for the patients presenting with hypoparathyroidism.
Bookmark/Search this post with
Abstract (Original Language): 
Fahr hastalığı, bilateral bazal ganglionlarda kalsinozis tablosudur. Kalsiyum; kapillerler, arterioller, küçük venler ve perivasküler alanlarda birikir ve kraniyal mikrodamarlarda obliterasyon, damar çevresinde nöronal dejenerasyon ve gliozise sebep olur. Kalsifikasyonlar çoğunlukla simetrik olarak dentat çekirdek, bazal ganglion, talamus ve centrum semiovalede görülür. Tanıda, kalsiyum birikimlerini göstermede en sık kullanılan inceleme yöntemi kranial BT’dir. Etiyolojik olarak Fahr hastalığı, ailevi, sporadik veya sekonder (enflamatuar, tümoral, hipoksik, vasküler, endokrin, toksik, metabolik, dejeneratif, malabsorptif) olabilir. Biz bu nadir görülen sendromu hipoparatiroidi kliniği ile baĢvuran hastalarda akılda tutulması amacıyla tartıĢmaya sunuyoruz
FULL TEXT (PDF): 
PDF icon arastirmax-nadir-bir-hipokalsemi-nedeni-fahr-hastaligi.pdf
  • 3
82-84
  • Turkish

REFERENCES

References: 

1. Adachi M, Wellmann KF, Volk BW. Histochemical studies on the pathogenesis of idiopathic non-arteriosclerotic cerebral calcification. J Neuropathol Exp Neurol 27:483-99,1969.
2. Beall SS, Patten BM, Mallette L, Jankovic J. Abnormal systemic metabolism of iron, porphyrin and calcium in Fahr’s syndrome. Ann Neurol 26:569-75,1989.
3. Billard C, Dulac O, Bouloche J, Echenne B, Lebon P, Motte J, Robain O, Santini JJ. Encephalopathy with calcifications of the basal ganglia in children. A reappraisal of Fahr’s syndrome with recpect to 14 new cases. Neuropediatrics 20:12-9,1989.
4. El Maghraoui A, Birouk N, Zaim A, Slassi I, Yahyaoui M, Chkili T. Fahr syndrome and dysparathyroidism. 3 cases. Presse Med 24: 1301-1304, 1995.
5. Fahr T. Von. Idiopathische verkalkung der hirngefasse. Zentrabl. Allg. Pathol 50:129-133, 1930.
6. Karimi M, Habibzadeh F, De Sanctis V. Hypoparathyroidism with extensive intracerebral calcification in patients with betathalassemia major. J Pediatr Endocrinol Metab; 16: 883-886, 2003.
7. Margolin D, Hammerstad J, Orwoll E, McClung M, Calhoun D. Intracranial calcification in hyperparathyroidism associated with gait apraxia and parkinsonism. Neurology 30:1005-1007, 1980.
8. MorganteL, Vita G, Meduri M et al. Fahr’s syndrome: local inflammatory factors in the pathogenesis of calcification. J Neurol 233:19-22,1986.
9. Paprocka J, Jamroz E, Wackermann-Ramos A, Sokol M, Marszal E. [Neurological picture and 1H MRS in 4 children with hypoparathyroidism] Przegl Lek.; 62: 680-684, 2005.
10. Smiths M, Gabreels F, Froeling P. Progressive idiopathic hypoparathyroidism and nervous system dysfunction: report on three cases and review of literature. JNeurol 228:113-122, 1982.
11. Villarreal-Núñez F, Adames-Quintero AE.Late onset epilepsy as the first symptom of pseudohypoparathyroidism. Rev Neurol. 2005;41:155-158, 2005.
12. Windeck R, Menken U. Basal ganglia calcification in pseudohypoparathyroidism typeII. Clin Endocrinol 15:57-63, 1981.

Thank you for copying data from http://www.arastirmax.com