Basal Ganglia Diseases

Definition

Definition of Basal Ganglia Diseases
Basal ganglia disease refers to a group of physical dysfunctions that occur when the group of nuclei in the brain known as the basal ganglia fail to properly suppress unwanted movements or to properly prime upper motor neuron circuits to initiate motor function. Research indicates that increased output of the basal ganglia inhibits thalamocortical projection neurons. Proper activation or deactivation of these neurons is an integral component for proper movement. If something causes too much basal ganglia output, then the thalamocortical projection neurons become too inhibited and one cannot initiate voluntary movement. These disorders are known as hypokinetic disorders. However, a disorder leading to abnormally low output of the basal ganglia leads to relatively no inhibition of the thalamocortical projection neurons. This situation leads to an inability to suppress unwanted movements. These disorders are known as hyperkinetic disorders. Currently, reasons for abnormal increases or decreases of basal ganglia output are poorly understood. One possible factor could be the natural accumulation of iron in the basal ganglia, causing neurodegeneration due to its involvement in toxic free-radical reactions. Though motor disorders are the most common associated with the basal ganglia, recent research shows that basal ganglia disorders can lead to other dysfunctions such as Obsessive Compulsive Disorder (OCD) and Tourette syndrome.

Symptoms

Symptoms of Basal Ganglia Diseases
Damage to the basal ganglia cells may cause problems with one's ability to control speech, movement, and posture. A person with basal ganglia dysfunction may have difficulty starting, stopping, or sustaining movement. Depending on which area is affected, there may also be problems with memory and other thought processes.

In general, symptoms vary and may include:

1. Movement changes, such as:
2. Involuntary movements
3. Slowed movements
4. Increased muscle tone
5. Muscle spasms and muscle rigidity
6. Memory loss
7. Problems finding words
8. Tremor
9. Uncontrollable, repeated movements, speech, or cries (tics)
10. Walking difficulty

Causes

Causes of Basal Ganglia Diseases
Conditions that cause injury to the brain can damage the basal ganglia. Such conditions include:

1. Drug overdose
2. Head injury
3. Infection
4. Liver disease
5. Metabolic problems
6. Multiple sclerosis
7. Side effects of certain medications
8. Stroke
9. Tumors

Many brain disorders are associated with basal ganglia dysfunction. They include:

1. Dystonia
2. Huntington's disease
3. Multiple system atrophy
4. Parkinson's disease
5. Progressive supranuclear palsy
6. Wilson's disease

Diagnosis

Diagnosis of Basal Ganglia Diseases
The health care provider will perform a complete physical and neurological exam. Depending on the results, blood tests and imaging studies of the brain may be needed. This may include:

1. CT and MRI of the head
2. Magnetic resonance angiography to look at the blood vessels in the neck and brain
3. Positron emission tomography (PET) to look at the metabolism of the brain
4. Blood tests to check blood sugar, thyroid function, liver function, and iron and copper levels

Treatment

Treatment of Basal Ganglia Diseases
Treatment depends on the cause of the disorder.

Prognosis

Prognosis of Basal Ganglia Diseases
How well a person does depends on the cause of the dysfunction. Some causes are reversible, while others require lifelong treatment.

Prevention

Prevention of Basal Ganglia Diseases
Consult with your doctor.