Rıza Taner BARAN, Hüseyin DEMİRBİLEK, Serpil BARAN, Mesut PARLAK, Eda PARLAK

Çocukluk çağında nadir görülen idiyopatik hipoparatiroidizm ve bazal gangliyon kalsifikasyonu birlikteliği: Fahr hastalığı

ahr hastalığı; bazal ganglionlar, serebellum, talamus ve beyin parankiminde çift taraflı simetrik kalsifikasyon ile seyreden, nadir, progresif nörodejeneratif bir hastalıktır. Hastalık genellikle erişkin yaş grubunda, 3-4. dekadlarda görülmekle beraber, çok nadir olarak çocukluk yaş grubunda da bildirilmiştir. Bu olgu sunumunda acils ervise idiyopatik hipoparatiroidiye bağlı hipokalsemik konvülzyonla başvuran ve sonar Fahr hastalığı tanısı alan 9.5 yaşında kız hasta sunulmuştur. İdiyopatik hipoparatiroidi saptanan çocuklarda, çocukluk yaş grubunda çok nadir görülen Fahr hastalığı akılda tutulmalı ve bu olgulara gerekirse kraniyal görüntüleme yapılmalıdır.

A rare clinical condition in childhood comorbidity of idiopathic hypoparathyroidism and basal ganglia calcification: Fahr disease

Fahr disease, a rare progressive neurodegenerative disease, is characterized by symmetrical intracranial calcifications of basal ganglia, cerebellum, thalamus and intraparanchymal regions. Although the typical presentation is at the adulthood, particularly 3rd to 4th decades, extremely limited number of children with Fahr diesease have been reported. In this case report a 9,5 year-old female had been admitted to emergency service with hypoclacemia due to idiopathic hypoparathyroidism then diagnosed as Fahr disease is presented. In pediatric patients presented with idiopathic hypoparathyroidism, Fahr disease should be kept in mind in the differential diagnosis and cranial imaging might be required.

Keywords:

Fahr disease, hypoparathyroidism, intracranial calcification, childhood,

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