What is basal ganglia calcification?

Basal ganglia calcification, better known as Fahr's syndrome, is a disorder that is characterized by an abnormal amount of calcium mainly stored in the basal ganglia and also in the cortex. It is considered to be a inherited genetically dominant condition. Fahr's syndrome is also known as nearterioskerotic brain calcification, calcification of idiopathic basal ganglia, Striopallidodate calcinosis, cerebrovascular ferrokalcinosis 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 should not be, including basal ganglia and cortex, which are connected to the front of the brain. These structures are together for functions such as eye movement, consciousness, memory and motor skills.

The most important symptoms of this condition are dementia and erosion of motor function. Other symptoms include dysarthria or loss of articulation in speech; spasticity or stiffness of the limbin; and athetosis or involuntary written movements of arms, fingers, neck and legs. Symptoms of Parkinson's disease, such as moving, lack of face expression and dystonia or abnormal muscle contractions, are common. It is also known that basal ganglia calcification causes microcephaly or reduction of the skull and glaucoma disorder.

The cause of this disease is unknown, so it is sometimes referred to as an idiopathic disorder. It is also extremely rare. However, some doctors theorized that the disorder is inherited in cases where the mother and father have what is referred to as the Fahr gene. Children then have a 25% risk of inheriting them as an autosomal recessive property. This leads to some people to classify the disease as a neurogenetic dissection rather than being simply neurological.

IBGC1 is the official name of the Fahr gene; "IBGC" is an abbreviation for calcification of the idiopathic basal ganglia. The disease affects the man iWomen A may also appear in any life phase, although it seems to occur a little more often in people aged 30 to 60 years.

Because there is no treatment for this disease, treatment usually consists in mastering symptoms. The inability to solve the disease significantly accelerates only its progress, which eventually leads to disability and death. However, even in the treatment of symptoms of Fahr disease, prognosis in patients is very poor.