Idiopathic basal ganglia calcification

Historical note and nomenclature
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By Shaheda N Azher MD

Bilateral, symmetric calcification involving the striatum, pallidum, with or without deposits in dentate nucleus, thalamus, and white matter has been reported from asymptomatic individuals in a variety of neurologic conditions (Lowenthal and Bruyn 1968; Oliveira 2004; Manyam 2005; Schmidt 2005). Idiopathic basal ganglia calcification, or bilateral striopallidodentate calcinosis, commonly referred to as Fahr disease, has several names in the literature. Secondary bilateral calcification also has been reported in a variety of genetic, developmental, metabolic, infectious, and other conditions (Manyam 2005). Delecour first described vascular calcifications of the basal ganglia in 1850 in a 56-year-old man who had stiffness and weakness of lower extremities with tremor. In 1855, Bamberger described the histopathologic entity of calcifications in a woman who had mental retardation and seizures (Bamberger 1855; Manyam 2005). Later, in 1930, Fahr described an 81-year-old man with a long history of dementia, “immobility without paralysis,” with pathologic findings of “rough granular cortex and calcifications in centrum semiovale and striatum” (Fahr 1930). Attaching Fahr’s name to the syndrome of idiopathic basal ganglia calcification is not justified as he was not the first to describe the disorder nor did he contributed significantly to the understanding of the disorder. Fritzsche gave the first roentgenographic description of the condition in 1935. As these calcifications tend to extend beyond the basal ganglia into the dentate nuclei and to a lesser degree other subcortical structures, a descriptive term, bilateral striopallidodentate calcinosis, has been suggested as more appropriate, but because the hallmark is involvement of the basal ganglia, the term “idiopathic basal ganglia calcification” is well established in the literature, and idiopathic basal ganglia calcification is used in the OMIM registry, we will use the term in this review.

A detailed historical description of idiopathic basal ganglia calcification is provided by Lowenthal and Bruyn (Lowenthal and Bruyn 1968).