Epilepsy Revealing Fahr's Disease Associated with Hyperparathyroidism

Abstract

Introduction: Fahr's disease (FD) is a rare anatomo-clinical en-tity, characterized by the presence of intracerebral calcifications of the basal ganglia, most often associated with phosphocalcic disorders, mainly hypoparathyroidism. Seizures are rare in this disease. We report the case of FD revealed by recurrent convul-sive seizures taken medication for epilepsy for several years. Case report: 26-year-old man followed since the age of 16 for recurrent generalized tonic-clonic seizures. Initial investigations were without abnormalities leading to the diagnosis of epilepsy. The patient was treated with sodium valproate with partial sta-bilization. Since a year, the patient reported an increase in the frequency of epileptic seizures despite the strengthening of antiepileptic treatment. Cerebral CT scan showed the presence of multiple intracerebral calcifications with bilateral and symmetrical distri-bution, particularly in the central gray nuclei consistent with the diagnosis of FD. Biological analysis showed hypercalcemia at 2.70 mmol/l, hypophosphatemia at 0.4 mmol/l, and the parathyroid hormone was increased to 172 ng/l confirming the diagnosis of associated primary hyperparathyroidism. Cervical ultrasound revealed an upper left parathyroid nodule. After surgery of his adenoma, the subsequent evolution was marked by a net regression of the frequencies and the intensity of the seizures. Doses of valproic acid were significantly reduced. Conclusion: Epilepsy remains a rare and unusual manifestation of FD. It can exceptionally be the inaugural symptom of the disease. FD deserves to be known and screened for when the cause of seizure or epilepsy cannot be proven, especially since it is often associated with parathyroid disorders whose timely treatment can avoid secondary sometimes serious complications.