Fahr’s syndrome secondary to idiopathic hypoparathyroidism: a report of two adolescent cases
DOI:
https://doi.org/10.18203/2349-3291.ijcp20261144Keywords:
Fahr’s, Hypo-parathyroidism, Basal ganglia, Calcification, SeizuresAbstract
Fahr’s syndrome is a rare neurological disease characterized by calcification of basal ganglia and/or other areas of brain controlling motor activity. It occurs secondary to an identifiable condition, most commonly hypoparathyroidism. Hypoparathyroidism is an uncommon endocrinopathy with a wide range of presentations. We describe two adolescent cases presenting with seizures, neuropsychiatric manifestations and extra-pyramidal symptoms with bilateral basal ganglia calcification on neuroimaging. Both had hypo-calcemia, hyper-phosphatemia and low parathyroid levels. Vitamin D and calcium supplementation with or without phosphate binders is the mainstay of treatment with limited role of antiepileptics. Despite being rare, clinicians need to consider Fahr’s syndrome as a differential in such cases. Good response is seen after treatment of underlying hypo-parathyroidism.
References
Al-Azem H, Khan AA. Hypo-parathyroidism. Best Pract Res Clin Endocrinol Metabolism. 2012;26(4):517-22.
Zhou YY, Yang Y, Qiu HM. Hypo-parathyroidism with Fahr’s syndrome: A case report and review of the literature. World J Clin Cases. 2019;7(21):3662-70.
Saleem S, Aslam HM, Anwar M, Anwar S, Saleem M, Saleem A, et al. Fahr's syndrome: literature review of current evidence. Orphanet J Rare Dis. 2013;8:156.
Brandi ML, Bilezikian JP, Shoback D, Bouillon R, Clarke BL, Thakker RV, et al. Management of Hypoparathyroidism: Summary Statement and Guidelines. J Clin Endocrinol Metab. 2016;101:2273-83.
El Aoud S, Elleuch M, Charfi N, Hadj Kacem F, Mnif M, Rekike N, et al. Fahr's syndrome in Southern Tunisia: A broad spectrum of clinical and etiological features. Tunis Med. 2018;96:490-4.
López-Villegas D, Kulisevsky J, Deus J, Junqué C, Pujol J, Guardia E, et al. Neuropsychological alterations in patients with computed tomography-detected basal ganglia calcification. Arch Neurol. 1996;53(3):251-6.
Goswami R, Sharma R, Sreenivas V, Gupta N, Ganapathy A, Das S. Prevalence and progression of basal ganglia calcification and its pathogenic mechanism in patients with idiopathic hypo-parathyroidism. Clin Endocrinol (Oxf). 2012;77:200-6.
Maio SD, Soliman AT, Sanctis VD, Kattamis C. Current treatment of hypoparathyroidism: theory versus reality waiting guidelines for children and adolescents. Acta Biomed. 2018;89(1):122-31.
Liu MJ, Li JW, Shi XY, Hu LY, Zou LP. Epileptic seizure, as the first symptom of hypo-parathyroidism in children, does not require antiepileptic drugs. Childs Nerv Syst. 2017;33:297-305.
Modi S, Tripathi M, Saha S, Goswami R. Seizures in patients with idiopathic hypoparathyroidism: effect of antiepileptic drug withdrawal on recurrence of seizures and serum calcium control. Eur J Endocrinol. 2014;170:777-83.
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