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Primary familial brain calcification [1] (PFBC), also known as familial idiopathic basal ganglia calcification (FIBGC) and Fahr's disease, [1] is a rare, [2] genetically dominant or recessive, inherited neurological disorder characterized by abnormal deposits of calcium in areas of the brain that control movement.
The basal ganglia (BG) or basal nuclei are a group of subcortical nuclei found in the brains of vertebrates. In humans and other primates , differences exist, primarily in the division of the globus pallidus into external and internal regions, and in the division of the striatum .
The basal ganglia is a collective group of structures in the brain. These include the striatum, (composed of the putamen and caudate nucleus), globus pallidus, substantia nigra, and the subthalamic nucleus. Along with other structures, the basal ganglia are part of a neural circuit that is integral to voluntary motor function. [1]
The most consistent finding are widespread calcifications, which involve the white matter of the cerebrum mostly adjacent to the junction with the grey matter, the thalami, the basal ganglia and the brainstem. [1] [2] The white matter of the cerebellum and the dentate nuclei are less often involved. However, the brain may appear normal in the ...
MRI: Multifocal infarct-like cortical areas in different stages of ischemic evolution, areas that do not conform to any known vascular territory. Initial lesions often occur in the occipital or parietal lobes with eventual involvement of the cerebellum, cerebral cortex, basal ganglia, and thalamus. [citation needed]
The caudate nucleus is one of the structures that make up the corpus striatum, which is part of the basal ganglia in the human brain. [1] Although the caudate nucleus has long been associated with motor processes because of its role in Parkinson's disease, [2] [clarification needed] [3] it also plays important roles in nonmotor functions, such as procedural learning, [4] associative learning ...
The basal ganglia are located bilaterally, and have rostral and caudal divisions. The putamen is located in the rostral division as part of the striatum. The basal ganglia receive input from the cerebral cortex, via the striatum. This is a transverse section of the striatum from a structural MR image.
Calcification [55–95%] of the cerebral cortex (especially depths of sulci, basal ganglia, cerebellum, thalamus; also of the arteries, arterioles, and capillaries). Vascular changes - String vessels, especially in areas of Metachromatic leukodystrophy, calcification in leptomeningeal vessels, accelerated atherosclerosis and arteriolosclerosis.