What is Fahr's disease and is it dangerous?

Fahr's disease is a neurological disease characterized by bilateral calcification of the basal ganglia and brain parenchyma. People with fahr disease have disorders in coordination of movements, movements and other mental disorders depending on the area of ​​​​the brain that is damaged. Fahr disease is a familial disease, but its prevalence in the community is not high.

Fahr disease is a progressive neurological disease. Fahr disease is the result of primary calcification of different areas of the brain, most commonly in the basal ganglia, thalamus, and cortical regions. The calcification process occurs spontaneously with no known cause, leading to disturbances in motor and mental development, memory impairment and other mental disorders. In the final stage of fahr disease, patients often have to deal with muscle spasms and convulsions that lead to limited mobility and reduced quality of life.
The cause of fahr disease is currently not well understood. Gene mutations are thought to be the main factor responsible for the occurrence and development of the disease. Fahr disease is hereditary. Children born to parents with the disease have a higher risk of developing fahr disease than other children. However, the emergence of new mutations during fetal life is also considered as another cause of fahr disease.

Fahr disease was first reported in 1885 with a case of calcification in two basal ganglia. Fahr disease is named after the neurologist Theodor Fahr when he described a subsequent case of bilateral basal ganglia and other areas of the brain calcification. Fahr disease is recognized as a familial disease, which can be inherited dominantly or recessively depending on the case. The frequency of fahr disease in the community is rare, although not well documented.

Many neurologists believe that fahr disease may have been present for a long time, in adolescence, and progressed gradually. People with fahr disease often present with clinical symptoms only in the late stages of the disease.
Calcified lesions of brain parenchyma and cerebral ganglia appear at an early stage and may be incidentally detected in children over 10 years of age. Imaging facilities play an important role in the diagnosis and detection of fahr disease at an early stage of the disease.
When entering the late stage, patients often face other mental and motor disorders. Middle-aged patients may present clumsy in coordination of movements and daily movements, difficulty in speech. However, some cases of fahr disease do not show any signs until the end of life.

The types of movement disorders that the patient may experience are: Tremor when moving or changing position Stiff gait or difficulty walking Choreography Spontaneous epileptic seizures Face stiff and unexpressed emotions In addition, people with fahr disease also develop memory loss gradually. Impaired mobility, memory loss, and mental disorders are the heavy consequences that fahr disease leaves for patients.

Clinical symptoms of fahr disease are not specific, so the diagnosis of fahr disease is only made when the treating doctor thinks about it. Information about personal and family medical history, combined with the analysis of the patient's symptoms, plays a role in guiding the diagnosis.
Definitive diagnosis of fahr disease is established by means of diagnostic imaging. Bilateral symmetrical calcifications in multiple areas of the brain are specific evidence of fahr disease. Computed tomography of the brain and magnetic resonance imaging of the brain are the first-line diagnostic tools often prescribed by doctors. A number of other laboratory tests should also be performed for the differential diagnosis of diseases with similar manifestations. The appointment and selection of tests by doctors change flexibly based on each specific case.

Currently, there is no specific treatment for fahr disease. People with fahr disease are unlikely to be completely cured, but clinical symptoms can be controlled and relieved. It is also the main goal in the current treatment of fahr disease to improve the patient's quality of life.
Prominent motor coordination disorder and impairment in patients with fahr disease should be prioritized for treatment. Certain aids and devices, as well as physical therapy exercises, help restore part of the day-to-day activities.
Seizures or mental disorders will be treated by medical methods with the use of alone or in combination drugs of the class of antiepileptic drugs, anti-sedatives, antidepressants.
People with fahr disease need to inform other family members. Doing this can benefit generations after conducting genetic counseling during pregnancy because it is a disease that can be passed on if a parent has it.

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