Hypocalcemia due to hypoparathyroidism leads to a large spectrum of clinical manifestations but a rare and unusual presentation is onset or aggravation of epilepsy.
We report a 77-year-old man who was found to have profound hypocalcaemia and idiopatic hypoparathyroidism when investigated for epileptic seizures. He had affective disturbances and only mild neurocognitive disorders. Cataract was present. The neurological examination showed an extrapyramidal syndrome with postural tremor and cerebellar ataxia. The deep tendon reflexes were normoactive in all four limbs. Chvosteks sign was present but Trousseau sign was not observed. Laboratory analysis showed: low concentration of serum ionized calcium at 2.9 mg/dl (normal: 4.25.4 mg/dl), total calcium at 5.9 mg/dl (normal: 8.8 to 10.0 mg/dl), hyperphosphoremia at 6.4 mg/dl (normal: 2.3 to 4.7 mg/dl) and 2.5 pg/ml intact-parathyroid hormone (normal: 11.0 to 67.0 pg/ml). Alkaline phosphatase, magnesium, calcitonin, serum thyroxin and thyroid-stimulating hormone levels were normal. Brain computed tomography demonstrated a symmetric, extensive, bilateral calcification of the basal ganglia, centrum semiovale, and bilateral dentate nuclei of the cerebellum, typical for Fahrs syndrome. The red nucleus and substantia nigra appeared normal. The diagnosis of Fahrs syndrome, secondary to hypoparathyroidism was posed. A prepontine meningioma was also found. The electrocardiogram showed normal QTc interval and the interictal electroencephalography and electromyography were normal. Bone densitometry was normal. After the patient was treated with oral calcium and active vitamin D (1-alphahydroxy vitamin D3), serum calcium levels returned to normal and seizure attacks ceased progressively resulting in stopping antiepileptic drugs. These cases illustrate the importance of search for disrupted phosphocalcic metabolism but also emphasizes the importance of the role of neuro-imaging in patients with new-onset epileptic seizures in order to detect hypocalcemia secondary to hypoparathyroidism.
28 - 31 May 2016
European Society of Endocrinology