Bilateral Cerebral Calcifications in Secondary Fahr's Syndrome.

Abstract

Fahr's syndrome is a rare, slowly progressive neurodegenerative disorder characterised by bilateral cerebral calcifications, mostly in the basal ganglia. These cerebral calcifications are composed of calcium and phosphate and are the result of disturbances in calcium-phosphate homeostasis. The clinical manifestations include neurological, neurocognitive and psychiatric symptoms. This article describes three rare cases of pronounced bilateral cerebral calcifications. All three patients were admitted to the hospital due to a first-time epileptic seizure. In all three cases, laboratory tests showed significant hypocalcaemia, and cerebral computed tomography showed pronounced bilateral cerebral calcifications in various brain areas. After calcium substitution and anticonvulsant treatment, the patients returned to their prehospital condition and were discharged home seizure free. The aim of this article is to highlight the clinical importance of long-term follow-up biochemical laboratory testing and neurocranial imaging in high-risk patients (e.g., after thyroidectomy) to prevent avoidable neurological and psychiatric complications through pharmaceutical and nutritional substitution therapy.