Idiopathic basal ganglia calcification

Shaheda N Azher MD (Dr. Azher of Mid Hudson Medical Group has received honorariums from USWorldMed for consultantcy and advisory committee membership.)
Joseph Jankovic MD (Dr. Jankovic, Director of the Parkinson's Disease Center and Movement Disorders Clinic at Baylor College of Medicine, received research funding from Allergan, Allon, Ceregene, Chelsea, EMD Serono, Impax, Ipsen, Lundbeck, Medtronic, Merz, and Teva, and compensation for his services as a consultant or an advisory committee member by Allergan, Auspex, EMD Serono, Lundbeck, Merz, Neurocrine Biosciences, and Teva.)
Originally released June 25, 2007; last updated December 26, 2014; expires December 26, 2017

This article includes discussion of idiopathic basal ganglia calcification, Fahr syndrome, symmetrical calcification of the basal ganglia, calcification of the striopallidodentate system, striopallidodentate calcification, pallido dentate calcification, calcification of the carpus striatum and dentate nucleus, idiopathic calcification of cerebral capillaries, familial idiopathic cerebral calcifications, familial calcific dentate-striatal degeneration, familial idiopathic striopallidodentate calcification, familial basal ganglionic calcification, familial idiopathic brain calcification, progressive idiopathic striopallidodentate calcinosis, idiopathic familial brain calcifications, and idiopathic nonarteriosclerotic cerebral calcification. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

Idiopathic basal ganglia calcification (IBGC), 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 article, the authors discuss the etiology, pathogenesis, genetics, classification, and clinical manifestations of idiopathic basal ganglia calcification and secondary bilateral calcification.

Historical note and terminology

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).

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