Fahr’s Syndrome is rare and often presents as a movement disorder.


The hallmark of the syndrome is bilateral, symmetrical calcification of the basal ganglia seen on CNS imaging studies.


Other sites that may show calcifications include:

(1) thalamus

(2) hippocampus

(3) cerebral cortex

(4) cerebellar subcortical white matter



(1) familial autosomal dominant

(2) familial autosomal recessive

(3) sporadic


Genetic loci associated with the disorder:

(1) IBGC1 on chromosome 14q

(2) SLC20A2 on chromosome 8p21

(3) chromosome 2q37


Clinical findings may include:

(1) extrapyramidal movement disorder (chorea, tremors, clumsiness, Parkinson-like, dystonia, bradykinesia, other)

(2) cerebellar signs

(3) impaired memory and/or concentration and/or inability to make decisions

(4) dementia

(5) psychiatric disorder (depression, psychosis, change in behavior)

(6) impaired speech

(7) seizures


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