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

OBJECTIVE

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.

CONCLUSIONS

In this report, we propose to discuss the clinical manifestations of FS, its etiologies especially parathyroid disturbances and its therapeutic modalities.