What Is Basal Ganglia Calcification?

IBGC is idiopathic basal ganglia calcification, also known as Fahr disease. It was first reported by Fahr (1930).

IBGC

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IBGC is idiopathic basal ganglia calcification, also known as Fahr disease. It was first reported by Fahr (1930).

nickname

Fahr disease

TCM disease name

Idiopathic base nuclear calcification

English name

idiopathic basal ganglia calcification

Visiting department

Neurology

Confirmation basis

According to the symptoms of dyskinesia, accompanied by mental disorders, mental retardation, etc., CT and MRI can show bilateral basal nucleus symmetrical calcification plaques. Family history is more supportive of diagnosis

Whether it is contagious

no

Whether inherited

May be relevant

Disease characteristics

Mental disorders, etc.

Inducement

Hypoparathyroidism and pseudohypoparathyroidism, intracranial infection in children, neonatal birth injury, etc.

Precautions

Early diagnosis, early treatment, and enhanced clinical care

Can it be cured

No-effect therapy

Bilateral basal calcification is called bilateral basal calcification syndrome or Fahr syndrome. Pale globules and caudate nucleus calcification are more common in older age, and can also appear in normal people. Those who have calcium plaque after 40 years of age are mostly considered physiological and have no clinical significance. . In addition to basal nucleus calcification, there is still cerebellar calcification.

With the popularization of CT scans in China, the discovery of calcification of basal ganglia has increased significantly. Some are calcified on one side of the basal nucleus, but most are on both sides

IBGC

basal ganglia calcification

Fahr disease; Fahr disease; Basal nucleus calcification; Basal nucleus calcification; Basal ganglia calcification; Basal ganglia calcification; Idiopathic basal ganglion calcification

Neurology> Dyskinesia

G23.8

There have been many reports recently in China, but the actual incidence of the disease is unknown.

The cause of IBGC is unknown, and it is currently believed to be mainly related to the following factors:

1. Genetic factors are mostly sporadic, and there are also reports of familial disease, which are autosomal recessive or dominant.

2. Exogenous toxins activate glutamate receptors in the brain, produce neurotoxic effects, and lead to calcium deposition.

3. The abnormal metabolism of iron and calcium phosphate plays an important role in the pathogenesis of Fahr disease.

4. Immune factors.

IBGC is currently considered to be a neurodegenerative disease. The underlying mechanisms are as follows:

1. Familial cases are more frequent than onset in adolescence or early adulthood, with early genetic findings. Some patients are associated with rare genetic diseases, such as pseudohypoparathyroidism type 2, refractory anemia, and many autoimmune endocrine gland diseases. Main manifestations of various movement disorders, such as torsional spasm, unilateral or bilateral hand and foot asthma, tremor and ataxia. It can be seen that Parkinson syndrome with tonicity as the prominent manifestation, torsion spasm, hand and foot asthma, hand and foot asthma can completely disappear with the course of the disease, leaving only the symptoms of Parkinson syndrome.

2. Hypoparathyroidism or pseudohypoparathyroidism associated with abnormal calcium and phosphorus metabolism accounts for about two-thirds of basal calcification cases.

Fahr syndrome caused by primary hypoparathyroidism, has a long course, has multiple episodes of hand-foot convulsions, has dance, hand-foot movement or Parkinson's disease-like manifestations, cerebellar ataxia, or a few patients have bilateral Positive pyramidal tract signs. The diagnosis of primary hypoparathyroidism should be consistent with no history of trauma or thyroid surgery, hypocalcemia, hyperphosphatemia, and chronic seizures of hands and feet.

3. Some patients have mental disorders, such as depression, mania, compulsive behavior, aggressiveness, irritability, apathy, gender inversion, delirium, etc. Dementia is one of the most common clinical manifestations of the disease, but the type of dementia in Fahr disease is different from Alzheimer's disease and Pick's disease, and it is a mixed type of the two. Early signs of mental decline, mostly occult, followed by decreased memory, language, time, and spatial orientation.

Mental disorders occur in some patients. Dementia is one of the most common clinical manifestations of the disease. The early manifestations of mental retardation are mostly insidious. Later, memory, language, time, and spatial orientation decline.

1. Serum calcium content is normal, but patients with hypoparathyroidism or pseudohypoparathyroidism have decreased serum calcium and have low calcium clinical manifestations.

2. Routine blood, biochemical, and cerebrospinal fluid tests are not specific.

3. Drugs, trace elements, and biochemical tests are helpful for the diagnosis of the cause.

According to the symptoms of dyskinesia, accompanied by mental disorders, mental retardation, etc., CT and MRI showed bilateral basal nucleus symmetrical calcified plaques. A family history is more supportive of diagnosis.

The cause must be actively searched for, and it is clear that it is idiopathic or some special causes lead to Fahr syndrome. Moskowitr (1971) proposed new diagnostic criteria for Fahr disease: CT or X-ray films have bilateral basal nucleus symmetrical calcification; clinical manifestations without pseudohypoparathyroidism; serum calcium and phosphorus are within the normal range; renal tubules Normal response to thyroxine; no infection, poisoning and other causes; with or without family history.

Basal nucleus calcification that can be found for the cause is Fahr syndrome.

IBGC should pay attention to differentiating Fahr syndrome caused by various reasons (Table 1).

1. Hypoparathyroidism and pseudohypoparathyroidism are the most common causes of Fahr syndrome. The patient's serum calcium content is reduced, and hand and foot convulsions and convulsions are manifested. Pseudohypoparathyroidism type 2 is a rare familial genetic disease. In addition to the symptoms and signs of hypoparathyroidism, there are still obvious bone and physical development disorders.

2. Childhood intracranial infection can cause basal calcification. Bobek et al. Reported two cases of childhood meningoencephalitis, and later a series of clinical symptoms of basal calcification.

3. The main causes of Fahr syndrome in newborns are birth trauma, severe ischemic hypoxic encephalopathy, intrauterine asphyxia, etc., which can cause a series of manifestations of Fahr syndrome within a few months, and the medical history can be identified.

There is no specific treatment for IBGC, mainly for the cause and symptomatic treatment. Antipyramidal symptoms can be treated with anti-Parkinson's disease drugs and hand, foot and agitation drugs, and antipsychotic drugs can be used for mental symptoms.

There are reports of trials of platelet aggregation inhibitors or cerebral vasodilators, such as compound sodium ferulate capsules (Limai capsules), flunarizine (brainizine), nimodipine, Yilixun, etc .; use levodopa / Carbidol / Carbidopa (Divine Enzyme) or Levodopa / Bencerazil / Benserazide (Medopa) for tremor and palsy symptoms; Tibride (Tepiri) or Haloperidol for dance symptom.

The course of IBGC is long. It has been reported to live above 70 years.

IBGC is currently considered a neurodegenerative disease, and prevention is related to the cause. For those with a genetic background, preventive measures include avoiding the marriage of close relatives, the implementation of genetic counseling, carrier genetic testing and prenatal diagnosis and selective abortion to prevent the birth of children.

Early diagnosis, early treatment, and enhanced clinical care are of great significance for improving the quality of life of patients.

Methotrexate, glutamic acid, sodium ferulate, flunarizine, cinnarizine, nimodipine, levodopa, levodopa / carbidopa, carbidopa, benzylhydrazine, sulfur Biley, Haloperidol