A Case of Seizure Revealing Fahr’s Syndrome with Primary Hypoparathyroidism

Abstract

Patient: Male, 52Final Diagnosis: Fahr’s syndromeSymptoms: SeizureMedication: —Clinical Procedure: —Specialty: Endocrinology and MetabolicObjective:Rare diseaseBackground:Idiopathic basal ganglia calcification, also known as Fahr’s disease or Fahr’s syndrome, is a rare neurological disorder characterized by abnormal calcified deposits in the basal ganglia. Here, we report a case of Fahr’s syndrome with calcification of the basal ganglia due to hypoparathyroidism in a patient with seizures.Case Report:A 52-year-old male patient visited our clinic with seizures. Brain computed tomography (CT) showed bilateral symmetrical calcifications in cerebellar white matter, the corpus striatum, the posterior thalami, and the centrum semiovale of both cerebral hemispheres. He had symptoms of hypocalcemia and low parathyroid hormone levels. The patient was diagnosed with Fahr’s syndrome due to primary hypoparathyroidism. He underwent calcium supplementation and calcifediol treatment. His symptoms improved, and he was discharged from the hospital.Conclusions:In patients with hypocalcemia accompanied by parathyroid dysfunction, neurological examination and CT should be performed to confirm abnormal intracranial calcification.

Bilateral lesions of the basal ganglia and thalami (central grey matter)—pictorial review

“…44 ), followed by the cerebral cortex, hippocampus, subcortical and cerebellar white matter. The extent of calcification is variable, depending on the stage of the disease and duration of metabolic abnormalities [ 85 , 86 ]. Associated findings in primary hypoparathyroidism are diffuse patchy osteolytic lesions in the skull, whereas plaque-like dural calcifications and vascular calcifications, most notably of carotid arteries, are encountered in secondary hyperparathyroidism.…”

From cranial nerve palsy to seizures—All the signs that lead to secondary Fahr’s syndrome

“…Despite of a significant number of patients remaining asymptomatic (approximately 20%) until accidental diagnosis through neuroimaging, the clinical manifestation may be a varied spectrum of neuropsychiatric symptoms including movement disorders, seizures, speech disorders, spasticity, psychosis, cognitive impairment, and syncope 4,5 …”

“…Multiple clinical conditions are associated with intracranial calcifications, including infectious diseases (acquired immune deficiency syndrome, toxoplasmosis, cytomegalovirus, herpes simplex, brucellosis), genetic conditions (Fahr's disease, pseudohypothyroidism), vascular, toxic exposure (lead poisoning, hypervitaminosis D), autoimmune disease (systemic lupus), and endocrine disorders (calcium/phosphorus abnormalities) 9,10 . Hypoparathyroidism is a common cause of basal ganglia calcification, and its etiology can be idiopathic (uncommon condition characterized by the absence or atrophy of the parathyroid glands) or secondary (usually a complication of thyroid surgery) 5,11,12 …”

Primary Hypoparathyroidism Mimicking Ankylosing Spondylitis in a Young Man with Fahr's Syndrome: A Case Report

“…Patients with Fahr's syndrome may have neurological features such as seizures, myoclonus, dystonia, and Parkinsonism. Fifty-seven percent present with movement disorders, of which Parkinsonism is the most prevalent [7]. Studies by Hempel et al [8] and Lopez-Villegas et al [9] had shown that clinical presentation in Fahr's syndrome depends upon the area of brain involvement.…”