Fahr Sendromu ve Strok: Olgu Sunumu

Fahr sendromu, bazal ganglionlarda, periventriküler beyaz cevherde, beyin sapında ve serebellumda bilateral simetrik kalsifikasyonlar ile seyreden ve nadir görülen nörodejeneratif bir hastalıktır. Sporadik, ailesel ve otozomal dominant geçişle ortaya çıkabilmektedir. Genellikle 3. ve 5. on yıllar arasında gözlenmektedir. Patogenezinde, perivasküler alanda yer alan kan-beyin bariyerinin bölgesel değişikliklerinden kaynaklanan kalsiyum birikimi ve buna bağlı nöronal metabolizmanın bozulması olduğu düşünülmektedir. Fahr hastalığının kliniğinde nöropsikiyatrik bulgular ve hareket bozuklukları ön plandadır. Fahr sendromlu hastalar nadiren serebrovasküler hastalık ve strok yakınması ile başvurmaktadırlar. Fahr sendromunun serebrovasküler hastalıklardaki rolü tam olarak bilinmemektedir. Biz burada, Fahr sendromu tanısı konulan ve strok hikayesi olan 59 yaşında erkek hastayı sunacağız ve nadir görülen bu hastalığı serebrovasküler hastalık geliştirmedeki rolünü literatür eşliğinde tartışacağız.

Fahr Syndrome and Stroke: Case Report

Fahr’s syndrome is a rare neurodegenerative disease characterized by bilateral symmetrical calcifications in the basal ganglia, periventriculer white matter, brain stem and cerebellum. It can occur with sporadic, familial or autosomal dominant transition. It is usually observed between 3. and 5. decades. Pathogenesis of Fahr’s syndrome may be based in the regional changes of the blood-brain barrier located in the perivascular area that cause calcium accumulation which leads to the deterioration of the neural metabolism. Neuropsychiatric findings and movement disorders are prominent in the clinic of Fahr's disease. Patients with Fahr’s syndrome rarely apply with cerebrovascular disease and stroke complaints. The role of Fahr's syndrome in cerebrovascular diseases is not known completely. Here, we present a 59-year-old male patient with a history of stroke diagnosed with Fahr’s syndrome, and discuss the role of this rare disease in developing cerebrovascular diseases accompanied with the literature.

Kaynakça

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