Abstract
Idiopathic basal ganglia calcification (IBGC), commonly referred to as Fahr’s disease, is a rare neurological disorder characterized by the abnormal, symmetrical, and bilateral calcification of the basal ganglia and other brain regions. Patients typically present in their forties and fifties with various neurologic and/or psychiatric symptoms, including movement disorders, Parkinsonism, psychosis, and depression.The pathophysiology of this disease is not completely understood; however, several gene mutations have been identified in the pathogenesis of Fahr’s disease. These mutations display an autosomal dominant inheritance pattern. Furthermore, the regional phenotypic expression of calcifications differs greatly from patient to patient, as do their clinical presentations. Here, we describe a patient who presented with psychiatric manifestations and imaging consistent with Fahr’s disease.
Highlights
Fahr’s disease, known as idiopathic basal ganglia calcification (IBGC), or bilateral striopallidodentate calcinosis, is a condition characterized by calcium deposition in the brain, most often the basal ganglia
At the age of 47, we would expect him to present with movement disorders, as they are found in the majority of patients, with Parkinsonism being the most common [1,2]
In the presence of a positive family history, the diagnosis can be made in the absence of bilateral calcification or progressive neurologic dysfunction and neuropsychiatric manifestations
Summary
Fahr’s disease, known as idiopathic basal ganglia calcification (IBGC), or bilateral striopallidodentate calcinosis, is a condition characterized by calcium deposition in the brain, most often the basal ganglia. When the patient was told that he would be discharged from the emergency department, he began complaining of chest tightness, shortness of breath, and nausea, all of which he denied on a review of symptoms during the initial evaluation He stated that the chest discomfort had been present all day, was non-radiating, and rated at a 4/10 in severity. The CT scan revealed bilateral symmetric calcifications of the basal ganglia, putamen, caudate, thalami, dentate nuclei of the cerebellum, and cerebral white matter, consistent with Fahr's disease (Figure 1). Laboratory values, such as parathyroid hormone (PTH), thyroid stimulating hormone (TSH), T3, and T4, were all within the normal reference limits. Sagittal CT image showing calcifications throughout the basal ganglia and cerebellum
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