Basal ganglia calcification, better known as Fahr’s Syndrome, is a disorder that is characterized by abnormal amounts of calcium mainly deposited in the basal ganglia, as well as the cerebral cortex. It is considered an inherited, genetically dominant condition. Basal ganglia calcification is also known as non-arteriosclerotic cerebral calcification, idiopathic basal ganglia calcification, striopallidodentate calcinosis, cerebrovascular ferrocalcinosis or Fahr disease.

The neurological disorder was first described in 1930, when a medical researcher by the name of Fahr discovered deposits of calcium in places where they are not supposed to be. With basal ganglia calcification, the common sites are the basal ganglia and the cerebral cortex, which are connected with each other at the base of the forebrain. These structures are collectively responsible for functions such as eye movement, awareness, memory and motor skills.

Thus, the most important symptoms of basal ganglia calcification are dementia and the erosion of motor function. Other symptoms of basal ganglia calcification include dysarthria, or loss of articulation in speech; spasticity, or stiffness of the limbs; and athetosis, or involuntary, writhing movements of the arms, fingers, neck and legs. Signs of Parkinson's disease, such as shuffling movement, lack of facial expression, and dystonia, or abnormal muscle contractions, are common. Basal ganglia calcification is also known to cause microcephaly, or shrinking of the skull, and the eye disorder glaucoma.

The cause of basal ganglia calcification is unknown, thus it is sometimes referred to as an idiopathic disorder. It is also exceedingly rare. Some physicians, however, theorize that the disorder is inherited in cases when both the mother and father have what is referred to as a Fahr gene. The children then have a 25 percent risk of inheriting basal ganglia calcification as an autosomal recessive trait. This leads some people to classify the disease as a neurogenetic disorder, rather than being simply neurological.

IBGC1 is the official name of the Fahr gene; "IBGC" is an acronym for idiopathic basal ganglia calcification. The disease affects males and females alike. It can also appear at any stage of life, although it seems that it occurs in people between the ages of 30 and 60 a little more frequently.

Since there is no cure for the disease, treatment usually consists of managing the symptoms. Failure to significantly address the disease medically only hastens the progressive nature of the disease, which eventually results in disability and death. Even with the management of Fahr disease's symptoms, however, the prognosis for patients is very poor.