Neuropsychiatric disorders and the basal ganglia

Group of neurodegenerative diseases

This group of diseases is characterized by damage to the central nervous system. A certain number of neurons die, and this leads to neurological pathologies. Neurodegenerative diseases can have different causes and different clinical manifestations. It is characteristic that all diseases in this category are incurable. All therapy used is symptomatic. That is, it alleviates the manifestation of the disease, but does not in any way contribute to the regeneration of damaged areas. The most effective would be a transplantation of nerve tissue, but the method has both technical and moral obstacles. Similar diseases include Alzheimer's, Parkinson's, and Farah's diseases. As a rule, diagnosing such diseases in the early stages is difficult. Most often, when making a diagnosis, specialists rely on patient complaints and the results of observations of their behavior. But the main tool is tomography (computer, magnetic resonance).

Treatment of Farah's disease

There is no specific therapy for Farah's disease. Doctors prescribe drugs that improve blood circulation and help fight hyperkinesis, i.e. Parkinson's syndrome.

Treatment for this disease includes:

1. Antiparkinsonian drugs to restore normal dopamine metabolism - Levodopa. Medicines in this group: Madopar, Sinemet, Nakom.
2. Vasodilators: Stugeron, Cavinton.
3. Antihypoxants: Cytoflavin (Nicotinamide, Succinate, Riboflavin, Inosine), Mexidol, Mildronate, Citrulline.
4. Physiotherapy, kinesiology.
5. Nootropic drugs: Piracetam, Neopept, Glutamic acid.
6. Anticholinergics for the treatment of hyperkinesis (Atropine).
7. Benzodiazepines for relief of epileptiform seizures (Carbamazepine).

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Symptoms of the disease

Since this disease is a rather rare phenomenon, it is difficult for specialists to provide the correct clinical picture of the syndrome. Symptoms may appear if the patient is over 40 years of age. In younger people, Farah's disease symptoms may be vague and vague. Patients have impaired coordination of movement and parkinsonism. Also, the development of the disease may be indicated by such signs as tremor, dystonia, rapid and erratic movements of the limbs (chorea), involuntary contractions of the hands and feet. Since the disease affects areas of the brain, performance and mental capabilities decrease. A person’s memory also suffers. Speech is often impaired. Other neurological signs include pain and mental disorders. There is a juvenile form of the disease, which manifests itself in children and adolescents. The main symptoms are: dystonia, chorea, epileptic seizures. The senile form is typical for middle-aged and older people. In this case, parkinsonism, speech disorders, and other problems in the functioning of the nervous system are observed. Urinary incontinence may also occur.

Symptoms of Farah's disease

Calcification and accumulation of Fe ions in the arteries leads to a decrease in their elasticity, fragility and impaired capacity. As a result, people's memory deteriorates due to the death of neurons from chronic oxygen deficiency. Dementia (dementia) is observed.

Iron and calcium salts also accumulate in the basal ganglia - clots of gray matter that carry out motor functions. These nuclei, located at the base of the brain, make up the extrapyramidal system.

The extrapyramidal system consists of interconnected formations. They are responsible for the coordination of motor acts, the correct activation and consistent work of the flexors-extensors and other antagonist muscles.

When Farah disease affects the extrapyramidal system, the following symptoms occur:

1. Chorea (erratic movements), athetosis (tonic muscle contraction), tremors of the limbs and head at rest. Manifestations of parkinsonism and increased tendon reflexes are observed.
2. Nystagmus (quivering of the eyeballs).
3. Epileptiform seizures, local convulsions.
4. Muscle rigidity, i.e. their stiffness, tension.

The cerebral cortex, which is responsible for movement and sensitivity of the body, also suffers. Patients' cognitive functions (memory, attention, intelligence) decrease. Complications in children: oligophrenia, alexia, apraxia, agraphia. The work of the dentate nucleus of the cerebellum, responsible for the work of the lower and upper extremities, is disrupted. Weakness occurs.

Possible causes of Fahr syndrome

The reasons why the disease may develop have not been precisely established. However, it is known that disorders of the thyroid and parathyroid glands have a huge impact on its occurrence. In this case, disruptions occur in the metabolic processes of calcium and phosphorus. Similar neurodegenerative diseases can also occur when the acid-base balance in the body is disturbed. Alkalosis leads to significant loss of acids. But alkaline compounds are present in excess. There is also an opinion that Fahr syndrome occurs due to genetic disorders. That is, the gene that is responsible for the exchange of an element such as calcium mutates. However, this version has many contradictions. There are isolated cases where symptoms of the disease appeared after irradiation of the head area, poisoning with poisons, lead, in children with Down syndrome. Calcinosis has also been diagnosed in people who have had rubella (rarely). Another possible cause of basal ganglia calcification is birth trauma.

Fahr's disease - what is it? Causes, symptoms, treatment » iHealth health magazine

This group of diseases is characterized by damage to the central nervous system. A certain number of neurons die, and this leads to neurological pathologies. Neurodegenerative diseases can have different causes and different clinical manifestations. It is characteristic that all diseases in this category are incurable.

All therapy used is symptomatic. That is, it alleviates the manifestation of the disease, but does not in any way contribute to the regeneration of damaged areas. The most effective would be a transplantation of nerve tissue, but the method has both technical and moral obstacles. Similar diseases include Alzheimer's, Parkinson's, and Farah's diseases.

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A group of similar diseases is characterized by manifestations of central nervous system lesions. A certain number of neurons die, which leads to the development of neurological pathologies.

Neurodegenerative diseases can occur for a variety of reasons and also have different clinical manifestations. A characteristic feature of all diseases from this group is their incurability.

All therapeutic actions performed are symptomatic.

That is, therapy is aimed at alleviating the symptoms of the disease, but in no way can affect the regeneration of areas that are damaged. The most effective treatment would be tissue transplantation, but this method has several limitations, primarily technical and moral.

The most famous diseases of this group are Alzheimer's disease, Parkinson's disease, and Fahr's syndrome. Early diagnosis of these diseases is often difficult.

In the diagnostic process, doctors mainly take into account patients' complaints and observe their behavior.

The most modern and effective method for diagnosing diseases of this group is tomography, both computed tomography and magnetic resonance imaging.

Inheritance of Fahr syndrome

Since this disease is a rather rare phenomenon, it is difficult for specialists to provide the correct clinical picture of the syndrome. Symptoms may appear if the patient is over 40 years of age. In younger people, Farah's disease symptoms may be vague and vague. Patients have impaired coordination of movement and parkinsonism.

Also, the development of the disease may be indicated by such signs as tremor, dystonia, rapid and erratic movements of the limbs (chorea), involuntary contractions of the hands and feet. Since the disease affects areas of the brain, performance and mental capabilities decrease. A person’s memory also suffers.

Speech is often impaired. Other neurological signs include pain and mental disorders. There is a juvenile form of the disease, which manifests itself in children and adolescents.

Urinary incontinence may also occur.

The reasons why the disease may develop have not been precisely established. However, it is known that disorders of the thyroid and parathyroid glands have a huge impact on its occurrence. In this case, disruptions occur in the metabolic processes of calcium and phosphorus. Similar neurodegenerative diseases can also occur when the acid-base balance in the body is disturbed.

Alkalosis leads to significant loss of acids. But alkaline compounds are present in excess. There is also an opinion that Fahr syndrome occurs due to genetic disorders. That is, the gene that is responsible for the exchange of an element such as calcium mutates. However, this version has many contradictions.

There are isolated cases where symptoms of the disease appeared after irradiation of the head area, poisoning with poisons, lead, in children with Down syndrome. Calcinosis has also been diagnosed in people who have had rubella (rarely). Another possible cause of basal ganglia calcification is birth trauma.

Fahr's disease is very rare. That is why specialists are not able to draw up a correct clinical picture of the syndrome. Symptoms of the disease appear in patients over 40 years of age.

In younger people, the symptoms of the syndrome are blurred and unclear. Patients suffer from poor motor coordination and develop parkinsonism.

In addition, evidence of the development of the syndrome is the presence of tremor, dystonia, chorea (rapid and erratic movements of the limbs).

These are not all the symptoms of Farah disease.

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The patient may also suffer from involuntary contractions of the feet and hands. Due to the fact that as a result of the disease, areas of the brain are damaged, the patient experiences a decrease in performance, mental abilities, and memory. Speech disturbances occur very often. Other neurological manifestations include the occurrence of pain and mental disorders.

This syndrome is a hereditary disease. Inheritance occurs according to an autosomal dominant pattern.

What does this mean? This type of inheritance involves the transmission of dominant traits located in autosomes. Phenotypic manifestations can vary significantly.

Such a sign can either manifest itself to a significant extent or be weakly expressed.

This type of inheritance has the following characteristic features:

1. The trait will appear in every generation.
2. The ratio of sick and healthy is one to one.
3. If the future husband has Farah disease and a healthy child is born, then his children will also be healthy.
4. The disease can affect girls and boys equally.
5. The disease can be transmitted equally from both men and women.
6. If both parents have the disease, then a child born homozygous for an inherited trait will be more seriously ill than a child born heterozygous.
7. The more a trait affects reproductive function, the greater the likelihood that various mutations will appear.

What will indicate an illness?

Doctors distinguish several forms of Farah disease. Among them:

• The juvenile form of the syndrome manifests itself in children and adolescents. The main symptoms of this form include manifestations of dystonia, chorea, and epileptic seizures.
• Senile form of the syndrome, characteristic of middle-aged and elderly people. Symptoms manifest themselves in the form of parkinsonism, speech disorders, and other problems in the functioning of the nervous system. Urinary incontinence is sometimes observed.

Source: https://nevrolog-info.ru/bolezn-fara/

Research methods for diagnosis

Before the advent of computed tomography, patients underwent X-ray examinations of the brain. Thanks to the advent of modern research methods, specialists received more informative pictures from the affected area. However, it is worth noting that computed tomography rather than magnetic resonance imaging has greater sensitivity for this clinical picture. When analyzing most images, the affected areas are limited to the globus pallidus. They are small in size. Most often, the basal ganglia, thalamus, and cerebellum undergo changes. The level of calcification is approximately the same in young and elderly people. Also, no differences were found between groups in which Farah's disease is asymptomatic and those in which the syndrome is accompanied by pronounced symptoms.

Histological studies

When conducting a postmortem examination, the following picture is observed in the brain: the vessels have a whitish appearance (some branched areas). When touched by a knife, they make a sound similar to a crunch. For histological analysis, material is collected: sections of the cerebral cortex, basal ganglia, and cerebellum. It is in the area of ​​the latter that calcification occurs most often. The samples contain calcium salts. They are also most often detected on arteries (small, medium-sized) and capillaries. Less commonly (in isolated cases), Farah's disease affects the veins. Small calcium conglomerates are found along the entire length of the vessels, as well as in adjacent tissues. The presence of traces of arsenic, aluminum, cobalt, and mucopolysaccharides is also detected in the tissues.

Establishing diagnosis

Depending on whether Farah disease has symptoms, making a diagnosis can be somewhat more difficult. It is often diagnosed accidentally, when a tomography is performed to confirm a completely different disease. First of all, the specialist excludes calcium metabolism disorders and other developmental defects. Then a computed tomography scan (or x-ray examination) is prescribed. Doctors note that one of the problems in making a correct diagnosis is hypoparathyroidism. This is a disease that occurs due to parathyroid hormone deficiency. As a result, the level of calcium in the blood decreases, and the level of phosphorus increases. If calcification of the striopallidodentate structures is observed on a CT scan, then additional tests are necessary to distinguish hypoparathyroidism from a condition such as Fahr's disease. First of all, it is important to determine the level of parathyroid hormone and calcium. Also, when making a diagnosis, parasitic damage to the nervous system is used. Specific reactions occur in the blood and cerebrospinal fluid. Fahr's disease is rarely differentiated from a condition such as Bourneville's sclerosis.

Treatment

Removing calcium deposits that have formed in the brain and its vessels is impossible. As a rule, treatment of such a disease is symptomatic. First of all, it is aimed at improving the metabolism of calcium and phosphorus in the body. If symptoms of Parkinson's disease are observed, the specialist prescribes special medications (levodopa). Also, Farah's disease, treatment of its manifestations, involves taking antioxidants and means to improve metabolism. Therapy with calcium channel blockers is ineffective. It is worth considering the fact that the disease is quite rare and little studied. In most cases, therapy reduces the symptoms of the disease, but does not affect the degree of calcification.

Diagnostics

To diagnose Fahr's disease, MRI or CT is used - the images clearly show calcified areas and changes in the cerebellum. However, calcifications are best seen on CT scans.

Differential diagnosis is carried out, the following diseases are excluded:

1. Helminth infections of the brain: cysticercosis (tapeworm larvae), echinococcosis, calcification also occurs, but secondary.
2. Ttoxoplasmosis (requires cerebrospinal fluid or blood antibody analysis).
3. Wilson-Konovalov disease.
4. Vascular dementia.
5. Tuberous sclerosis.

An analysis is also carried out for the content of parathyroid hormone and calcitonin in the blood. Ultrasound examination of the thyroid and parathyroid glands is indicated.

Polymerase chain reaction analysis is prescribed for the possible detection of neuroinfections: rubella, cytomegalovirus, toxoplasma. In these diseases, secondary calcification of the vessels of the brain and its structures occurs.

It is necessary to determine the levels of the main blood minerals - sodium, calcium, iron, phosphorus. Transcranial Doppler sonography will allow you to assess the condition of the vessels.

Prognosis for Farah disease

Since this condition disrupts metabolism in the brain, and calcium salts have an irritating effect on brain tissue, Farah’s disease has very ambiguous prognosis. The disease progresses slowly, and calcium deposits increase with age. Of course, all this does not happen in one year. Typically, neurodegenerative diseases progress over several decades. The main problem is the lack of information on this condition, as well as the lack of specific treatment. Therefore, therapy is aimed at improving the patient’s quality of life. Another negative point is that the disease is poorly diagnosed in the early stages.

Fahr's disease or syndrome: diagnosis and treatment

There are many human diseases that have a double name: by the clinical syndrome and by the name of the scientist who first described this disease.

In neurology, examples of such well-known diseases are multiple sclerosis (Charcot's disease) or Alzheimer's disease (a form of dementia).

A less well-known disease is Fahr's disease (Far syndrome). What kind of pathology is this and how is it treated?

First of all, you should not be surprised that many hereditary, and even the most common diseases of the nervous system depend on metabolism. For example, in diabetes mellitus, excess glucose permeates all organs and tissues, and so “poisons” the nervous system, which causes polyneuropathy with impaired sensitivity of the “gloves” and “socks” type.

But sugar (aka glucose) is perfectly soluble in water, and, therefore, in blood plasma. But there are substances that, upon reaching a certain point, turn into mineral sediment. These substances include calcium.

Its metabolism is regulated by parathyroid hormone, or parathyroid hormone, and its opposite action, thyrocalcitonin.

In the case of a significant excess of calcium, it undergoes mineralization, that is, calcification of individual small formations in the body occurs (for example, mediastinal lymph nodes). The process of calcification is calcification, and the calcified element itself is called calcification .

What is Farah's disease called?

Fahr's disease (or syndrome) is a fairly rare idiopathic disease (that is, of unknown etiology), in which calcification of brain structures occurs, namely the basal ganglia, dentate nuclei of the cerebellum and cerebral cortex. A more “beautiful” name sounds like this: idiopathic symmetrical intracerebral calcification of subcortical structures.

In this case, calcifications occur in the walls of small blood vessels, primarily arteries.

This nosology is classified as a neurodegenerative disease, since it gradually progresses dysfunction of the nervous system. The disease has been known since 1930, when the first such case was described by the German neurologist Karl Fahr.

What are the basal ganglia and dentate nuclei?

There are many strange formations in the brain: the putamen, the globus pallidus, the fence, the substantia nigra, pyramids, olives... The basal ganglia include structures that manage the extrapyramidal motor system, or the system of unconscious movements.

An example of how this system works is well known to everyone: when a person loses his balance on ice in winter, he waves his arms in a split second and, having “danced” on the ice, restores his balance, completely unconsciously. These movements happened so quickly because control of posture, gait and muscle tone passes by consciousness. It is in these movements that the work of the basal ganglia and cerebellar nuclei consists.

Causes

Why calcareous areas (calcifications) begin to appear in the subcortical parts of the brain is not completely clear.

Mainly, the disease is caused by damage to either the parathyroid or thyroid glands, and a violation of hormonal calcium metabolism.

It is not clear why, in this case, the structures of the brain are mainly affected with such high selectivity, and not, for example, calcareous stones are deposited in the kidneys.

There is an opinion about the genetic nature of Farah's disease. But it has not yet been possible to find the gene that is responsible for mineralization in the brain. In addition, calcifications located in the brain are not uncommon. In a large number of people, especially older people, calcifications can be found in the brain, for example, in the area of ​​the sella turcica, without any clinical manifestations.

The difference between “healthy calcification” is that in Fahr’s disease the basal ganglia are predominantly affected, and the affected areas are symmetrical.