Epileptic Seizures as te First Manifestation of Fahr’s Syndrome

Abstract

Introduction.Hypoparathyroidism is a rare endocrine disorder, leading to complications affecting the kidneys, the eyes, the cerebrum (Fahr's syndrome, epilepsy, parkinsonism, cognitive impairment), and the heart.Case report. We present the case of a 24-year old male that presented to our clinic for generalized tonicclonic seizures increasing in frequency for the last year or so. Furthermore, he was diagnosed with subcapsular cataract of both eyes and had intraocular lens implant surgery 12 years ago. CT scan performed at admission showed calcium deposits on the dentate nuclei, basal ganglia, thalamus, cerebellum and bilateral subcortical fronto-parietal white matter compatible with Fahr's disease. The results of the laboratory tests were normal except for hypocalcemia, hyperphosphoremia and low intact parathormone (iPTH). The patient was diagnosed with primary hypoparathyroidism which led to Fahr's syndrome. The patient was prescribed oral medication including calcium carbonate, calcidiol, magnesium orotate and levetiracetam. The follow-up after three months revealed a seizure free status and normalization of magnesium, calcium, and phosphorus levels.Conclusion. Fahr's syndrome can be a cause of epileptic seizures and should be considered in the clinical approach to an epileptic patient, especially in children, teenagers, and young adults.

Fahr syndrome and neurological manifestations in hypoparathyroidism patients

“…Eventhough maintaning calcium in normal level significantly correlate with seizure suppresion, Fahr's syndrome can be a cause of epileptic seizures and antiepileptic drug (AED) therapy should be considered. Presently there are still no specific guidelines regarding the efficiency of the antiepileptic treatment for patients with cerebral calcifications and hypocalcemia, as we seen in fahr syndrome caused by hypoparathyroidism [16] . However, there are some AED therapy, particularly long-term treatment, that known to be associated with vitamin D deficiency (eg, phenobarbital, phenytoin, and carbamazepine), leading to worsen hypocalcemia.…”

A Case of Fahr’s Disease with Epilepsy as the First Symptom in Infancy

“…Movement disorders, especially parkinsonism, ataxia, and cognitive impairment are the most common presentations. Seizures are rare and a few patients may not show any neurological, cognitive, or psychiatric symptoms [7].…”

Travesty of Undetected Hypoparathyroidism: A Series of Missed Opportunities

“…Other secondary aetiologies include vitamin D disorders, mitochondrial myopathy, neurodegenerative conditions, dermatological conditions (lipoid proteinosis), and infections, namely intrauterine or perinatal (toxoplasmosis, cytomegalovirus, herpes, rubella) [ 8 ]. The low levels of calcium and high levels of phosphorous seen in hypoparathyroidism eventually lead to calcification in different organs like the kidneys (which eventually causes nephrocalcinosis and renal insufficiency), the heart (leading to cardiac arrhythmias and congestive heart failure), the eyes (which lead to posterior subcapsular cataracts), and the cerebrum [ 9 ].…”