Idiopathic basal ganglia calcification

Introduction
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By Shaheda N Azher MD

Idiopathic basal ganglia calcification (IBGC) is also known as or subsumes Calcification of the basal ganglia, Fahr syndrome, Familial basal ganglionic calcification, Familial idiopathic cerebral calcifications, Idiopathic nonarteriosclerotic cerebral calcification, Striopallidodentate calcification, and Symmetrical calcification of the basal ganglia. -ed.

Idiopathic basal ganglia calcification, or bilateral striopallidodentate calcinosis, commonly referred to as “Fahr disease,” has been reported in asymptomatic individuals and in people with a variety of neurologic conditions. Parkinsonism or other movement disorders appear to be the most common clinical manifestation, followed by cognitive impairment and ataxia. CT scan is considered more sensitive than MRI for finding deposits in idiopathic basal ganglia calcification. In this clinical article, Dr. Shaheda Azher, Neurologist at Mid Hudson Medical Group in Poughkeepsie, New York, and Dr. Joseph Jankovic, Professor and Director of the Parkinson’s Disease Center and Movement Disorders Clinic at Baylor College of Medicine in Houston, Texas, discuss the etiology, pathogenesis, genetics, classification, and clinical manifestations of idiopathic basal ganglia calcification and secondary bilateral calcification.

In This Article

Introduction
Historical note and nomenclature
Clinical manifestations
Clinical vignette
Etiology
Pathogenesis and pathophysiology
Epidemiology
Differential diagnosis
Diagnostic workup
Prognosis and complications
Management
References cited
Contributors