Konvülsiyon ile Başvuran Fahr Sendromu: Olgu Sunumu

Fahr sendromu; kalsiyum ve fosfor metabolizma bozukluğu sonucu gelişen, çift taraflı simetrik intrakranyal kalsifikasyon ile seyreden nörodejeneratif bozukluklarla karakterize nadir görülen bir hastalıktır. Etyolojisi kesin olarak bilinmemektedir. Klinik olarak genellikle yürümede dengesizlik, konuşma bozukluğu, istemsiz hareketler, ya da kas krampları ile başlayıp tabloya psikoz, kişilik değişiklikleri gibi nöropsikiyatrik semptomlar da eklenebilir.Yazımızda, nöroloji tarafından epilepsi tanısıyla takip edilirken, acil servise tonik klonik nöbetle getirilen, idyopatik hipoparatiroidizme sekonder gelişen hipokalsemi ve BT’de bazal ganglionlarda bilateral yaygın kalsifikasyon saptanan Fahr sendromu olgusu sunulmuştur.

Fahr Syndrome Admıitted with Convulsion: A Case Report

Fahr's syndrome is caused by calcium and phosphorus metabolism disorders, is a rare disease, characterized by bilateral symmetrical intracranial calcification and neurodegenerative disorders. The etiology is uncertain. Clinically, it is usually start with unsteadiness, slurred speech, involuntary movements and muscle cramps. Psychosis, neuropsychiatric symptoms such as personality changes can be added. In this article, we described a patient brought to emergency room with tonic-clonic seizures. He had diagnosed of epilepsy by neurology clinic before applying us. We diagnosed Fahr syndrome due to idiopathic hypoparathyroidism with diffuse bilateral basal ganglia calcification on computed tomography.

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  • 1. Hoque MA, Siddiqui MR, Arafat Y, et al. Fahr's disease: a very rare cause of epilepsy. Mymensingh Med J 2010; 19: 127-29.
  • 2. Manyam BV. What is and what is not ‘Fahr’s disease’. Parkinsonism Relat Disord 2005; 11: 73-80.
  • 3. Andrea H Nemeth. GeneReviews™ [Internet]. Pagon RA, Bird TD, Dolan CR, et al, editors. Seattle (WA): University of Washington, Seattle; 1993.
  • 4. Anderson JR. Intracerebral calcifications in a case of SLE with nuerologic manifestations. Neuroradiology 1980; 19: 213-14.
  • 5. Illum F. Calcification of basal ganglia following carbon monoxide poisoning. Neurodiology 1980; 19: 213-14.
  • 6. Harati Y, Jackson JA, Benjamin E. Adult onset idiopathic familial brain calcifications. Arch Intern Med 1984; 144: 2425- 27.
  • 7. Modrego PJ, Mojonero J. Fahr’s syndrome presenting with pure and progresive presenil dementia. Neurol Sci 2005; 26: 367-69.
  • 8. Shibayama H, Kobayashi H, Nakagawa M, et al. Non Alzheimer, non-Pick dementia with Fahr’s syndrome. Clin Neuropathol 1992; 11: 237-50.
  • 9. Stelmasiak Z, Tarach JS. Idiopatic hypoparathyroidism with intracranial calcification and dominant skin manifestions. Med Sci Monit 2000; 6: 145-50.
Fırat Tıp Dergisi-Cover
  • ISSN: 1300-9818
  • Başlangıç: 2015
  • Yayıncı: Fırat Üniversitesi Tıp Fakültesi