Issue: Vojnosanit Pregl 2017; Vol. 74 (No. 2)

Fahr’s syndrome and idiopathic hypoparathyroidism – A case report

Authors:
Dejan M. Marinković, Tamara Dragović, Saša Kiković, Snežana Kuzmić Janković, Zorana Djuran, Zoran Hajduković

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Introduction. Fahr´s syndrome is a rare, slowly progressive,
neurodegenerative disorder, characterised by extensive, bilateral,
and symmetrical basal ganglia calcification. It is associated
with neuropsychiatric manifestations and gradually progressive
cognitive impairment. Fahr's syndrome is the secondary
form of brain calcification that is caused by various
metabolic, infectious, or degenerative diseases. Case report.
We presented a middle-aged male with Fahr's syndrome due
to primary idiopathic hypoparathyroidism. Clinical diagnosis
was based on signs and symptoms of hypocalcemia, progressive
neuropsychiatric illnesses, laboratory evidence of hypoparathyroidism,
and radiological signs of calcifications in
the basal ganglia. The patient improved after only a few days
of intravenous rehydration and calcium substitution, followed
by oral supplemental calcitriol. Conclusion. Timely recognition
of idiopathic and iatrogenic hypoparathyroidism allows
appropriate treatment that can prevent the development and
clinical manifestations of Fahr´s syndrome and potentially
slow its progression