Introduction: Fahrs syndrome (SF) is a rare anatomo-clinical entity, defined by non-arteriosclerotic striato-pallido-serrated calcifications localized in basal ganglia. It may be idiopathic or secondary to many etiologies dominated by phosphocalcic metabolic abnormalities, the main cause being primary or postoperative hypoparathyroidism.
Materials: This is a retrospective, descriptive study involving 11 patients with FAHR syndrome hospitalized at the Department of Endocrinology, Diabetology and Metabolic Diseases CHU IBN ROCHD, Casablanca from January 2010 to December 2018.
Results: The average age of our patients was 37 years (2150), with sex ratio H/F: 4/7.The mean duration of evolution was 9 years, mean serum calcium at the time of diagnosis was 53.38 mg/l. The circumstances of discovery were epileptic seizures in ten cases (90.9%), extrapyramidal syndrome in one patient. Four patients had psychiatric disorders (36.3%) nine patients had bilateral cataract (81.8%). The brain scan showed bilateral and symmetrical basal ganglia calcifications in all patients. Hypoparathyroidism was secondary to thyroidectomy in two cases (18.1%) and idiopathic in nine cases (81.8%). The evolution was favorable with vitamin-calcium supplementation in all patients.
Conclusion: The SF, a rare entity whose clinical manifestations are not very specific, the association of hypoparthyroidism with epilepsy remains rare but deserves to be sought for the simplicity of the determination of the calcemia in order to detect a syndrome of Fahr and thus adopt the most appropriate therapeutic measures.
18 - 21 May 2019
European Society of Endocrinology