Fahr syndrome is a rare neurological disorder characterized by calcium deposits in the basal ganglia and cerebral cortex, causing symptoms such as dementia, motor function erosion, and involuntary movements. It is considered an inherited, genetically dominant condition with no known cure. Treatment focuses on symptom management.
Basal ganglia calcification, better known as Fahr syndrome, is a disorder characterized by abnormal amounts of calcium deposited primarily in the basal ganglia and cerebral cortex. It is considered an inherited, genetically dominant condition. Fahr syndrome is also known as non-arteriosclerotic cerebral calcification, idiopathic basal ganglia calcification, striopalidodentate calcinosis, cerebrovascular ferrocalcinosis, or Fahr disease.
This neurological disorder was first described in 1930, when a medical researcher named Karl Theodor Fahr discovered calcium deposits in places where they shouldn’t be, including the basal ganglia and cerebral cortex, which are connected together at the base of the forebrain. These structures are collectively responsible for functions such as eye movement, awareness, memory and motor skills.
The most prominent symptoms of this condition are dementia and erosion of motor function. Other symptoms include dysarthria or slurred speech; spasticity or stiffness in the limbs; and athetosis, or involuntary, jerky movements of the arms, fingers, neck, and legs. Signs of Parkinson’s disease, such as shuffling, lack of facial expression and dystonia, or abnormal muscle contractions, are common. Basal ganglia calcification is also known to cause microcephaly, or narrowing of the skull, and the eye disorder glaucoma.
The cause of this disease is unknown, so it is sometimes referred to as an idiopathic disease. It is also extremely rare. Some medical professionals, however, theorize that the disorder is inherited in cases where both the mother and father have the so-called Fahr gene. Children therefore have a 25% risk of inheriting it as an autosomal recessive trait. This leads some people to classify the disease as a neurogenic disorder, rather than simply being neurological.
IBGC1 is the official name of the Fahr gene; “IBGC” stands for idiopathic basal ganglia calcification. The disease affects men and women equally and can also appear at any stage of life, although it appears to occur somewhat more frequently in people between the ages of 30 and 60.
Since there is no cure for the disease, treatment usually consists of symptom management. Failure to meaningfully address the disease medically only accelerates its progress, which ultimately results in disability and death. Even with management of Fahr’s disease symptoms, however, the prognosis for patients is very poor.
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