İDİOPATİK HİPOPARATİROİDİZME BAĞLI 3 FAHR HASTALIĞI VAKASI Three Cases of Fahr Disease due to Idiopathic Hypoparathyroidism

Yıl 2016, Cilt: 6 Sayı: 2, 62 - 65, 19.12.2016

Elif Turan Sait Gönen Gülsüm Gönülalan Mustafa Kulaksızoglu Ahmet Kaya

Öz

Bazal ganglionlarda bilateral kalsifikasyona bağlı nörolojik belirtiler Fahr hastalığı olarak bilinmektedir.

Fahr hastalığının etiyolojisinde; idiopatik, konjenital, kalsiyum bozuklukları; ağırlıklı olarak

hipoparatiroidiler, infeksiyonlar yer almaktadır. Bizim yazımızda hipoparatiroidiye bağlı bazal ganglionlarda

bilateral kalsifikasyon ve ekstrapiramidal semptomları olan üç vakadan bahsedilecek.

ABSTRACT

Bilateral calcifications in the basal ganglia associated with neurological manifestations have been

diagnosed as Fahr disease. The most frequent etiology of this syndrome is, together with idiopathic

causes and congenital, infections, calcium disorders, mainly primary hypoparathyroidism. We

present the cases of three patients with bilateral calcifications in the basal ganglia who presented

hypoparathyroidism and extra-pyramidal syndrome.

Anahtar Kelimeler

Hipoparatiroidi

Kaynakça

• 1. Marx SJ. Hyperparathyroid and hypoparathyroid disorders. N Engl J Med. 2000; 343(25): 1863-1875
• 2. Kobari M, Nogawa S. Familial idiopathic brain calcifications with autosomal dominant
• inheritance. Neurology. 1997; 48(3):645-649.
• 3. Jan De Beur, S. M., E. A. Streeten, and M. A. Levine. “Hypoparathyroidism and other causes of hypocalcemia.“Principles and Practice of Endocrinology and Metabolism.” Lippincot Williams and Wilkins; Philadephia (2001): 586-602.
• 4. Manyam BV. Bilateral strio-pallido-dentate calcinosis: a proposed classification of genetic and secondary causes. Mov Disord. 1990;5(Suppl 1):94S.
• 5. Cummings JL, Gosenfeld LF, Houlihan JP, McCaffrey Tet. Neuropsychiatric disturbances associated with idiopathic calcification of the basal ganglia. Biol Psychiatry. 1983; 18(5):591-601.
• 6. Manyam BV. What is and what is not Fahr’s disease’. Parkinsonism Relat Disord. 2005;11(2):73-80.
• 7. Menkes JH, Sarnat HB. Child Neurology. 6th ed, Philadelphia, Lippincott Williams&Wilkins 2000; 183-4
• 8. Manyam BV, Walters AS, Narla KR. Bilateral striopallidodentate calcinosis: clinical characteristics of patients seen in a registry. Mov Disord. 2001;16(2):258-64.
• 9. Koller WC, Cochran JW, Klawans HL. Calcification of the basal ganglia: computerized tomography and clinical correlation. Neurology.1979;29(3):328-33.