Publication:

Fahr’s syndrome secondary to idiopathic hypoparathyroidism: a report of two adolescent cases

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.