What is globus pallidus calcification?
Primary familial brain calcification (PFBC), also referred to as Idiopathic Basal Ganglia Calcification (IBGC) or “Fahr’s disease,” is a clinical condition characterized by symmetric and bilateral calcification of globus pallidus and also basal ganglions, cerebellar nuclei, and other deep cortical structures.
What happens if the globus pallidus is damaged?
If the globus pallidus is damaged, it can cause movement disorders, as its regulatory function will be impaired. There may be cases in which damage is deliberately induced, as in a procedure known as a pallidotomy, in which a lesion is created to reduce involuntary muscle tremors.
What causes Fahr disease?
Fahr’s syndrome has been known to be associated with the Kenny Caffey Syndrome Type 1. Being caused by a mutation in the TBCE gene, this syndrome is characterized by the symptoms of growth delay, cortical thickening of long bones, hypocalcaemia, hypothyroidism, and calcification of basal ganglia [41].
What is Fahr syndrome?
Fahr’s Syndrome is a rare, genetically dominant, inherited neurological disorder characterized by abnormal deposits of calcium in areas of the brain that control movement, including the basal ganglia and the cerebral cortex.
What does globus pallidus do?
The main function of the globus pallidus is to control conscious and proprioceptive movements. The GPe is the intrinsic nucleus, whereas the GPi is the output nucleus. The intrinsic nucleus acts as a relay for information. The output nucleus, primarily, sends information to the thalamus.
What is a calcified Tumour?
A calcified brain tumour is one in which calcium has built up. A range of different types of brain tumour can show different patterns and extent of calcification. Calcification happens when tumours are no longer able to regulate the movement of calcium in and out of their cells.
How is brain calcification treated?
Levodopa therapy was found to be effective in treating parkinsonian features in one individual who had PFBC and Parkinson disease. The anticonvulsant oxcarbazepine was effective in treating a Turkish patient with basal ganglia calcification and dyskinesia.
Which is a normal feature of the globus pallidus?
The globus pallidus is typically slightly hypointense relative to the putamen, a normal feature that is attributable to progressive iron deposition as one ages ( 1 ).
Is it normal to have basal ganglia calcification?
Basal ganglia calcification is common and is seen in approximately 1% of all CT scans of the brain, depending on the demographics of the scanned population. It is seen more frequently in older patients and is considered a normal incidental and idiopathic finding in an elderly patient but should be considered pathological in persons younger than
Is it normal to have bilateral dentate nucleus calcification?
Bilateral dentate nucleus calcification was seen in one patient. Skull radiograms were normal in all but one. Patients had diverse symptoms that were often explained by other findings, suggesting that calcifications may be coincidental and that basal ganglia calcification may not be a nosologic entity.
What is the diagnosis of Primary familial brain calcification?
Primary familial brain calcification (PFBC), also referred to as Idiopathic Basal Ganglia Calcification (IBGC) or “Fahr’s disease,” is a clinical condition characterized by symmetric and bilateral calcification of globus pallidus and also basal ganglions, cerebellar nuclei, and other deep cortical structures.