Fahr's syndrome in Southern Tunisia: A broad spectrum of clinical and etiological features

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Published Oct 24, 2018
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Vol. 96 No. 8 (2018): Issue Aug 2018

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Sahar El Aoud
Mouna Elleuch
Nadia Charfi
Faten Hadj kacem
Mouna Mnif
Nabila Rekike
Fatma Mnif
Mohamed Abid

Abstract

Aim: We describe the clinical and etiological profile of patients with Fahr's syndrome (FS).
Methods: Charts of sixteen patients diagnosed with FS between 1999 and 2014 were retrospectively assessed.
Results:  The mean age at diagnosis was 44.68 years (11-67 years). The most main presenting neurological features were seizures in 6 cases, headaches in 5 cases and parkinson's syndrome in 3 cases. Psychiatric disorders were observed in 2 patients including memory loss and iritability. Hypocalcemia clinical features were observed in 7 cases. The mean value of hypocalcemia was 1.69 mmol/l.
Etiologies included idiopathic hypoparathyroidism in 4 patients, pseudohypoparathyroidism in 5 cases, secondary hypoparathyroidism, isolated hypovitaminosis D and cerebral radiotherapy in one case for each and Fahr's disease in 4 patients.  Oral calcium and vitamin D substitution were started in patients with parathyroid disturbances with favorable outcome.
Conclusion : In this report, we propose to discuss the clinical manifestations of FS, its etiologies especially parathyroid disturbances and its therapeutic modalities.

Keywords:

Fahr's syndrome, hypocalcemia, hypoparathyroidism, pseudohypoprathyroidism.

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