Treatment of Basal Ganglia Disorders

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What is the Basal Ganglia

The basal ganglia are a group of nuclei located in the forebrain. There are four key areas in the basal ganglia that communicate with each other and the rest of the brain. These are the striatum, subthalamic nucleus, substantia nigra, and the pallidum.

The exact mechanisms and purpose of the complicated circuitry within the basil ganglia are not fully understood; however, it is known that they play an important role in coordinating and controlling movement. In current theory, the basal ganglia do not generate movement but are part of the process of selecting or repressing possible movement options. As such, they help maintain purposeful movement and repress unwanted movements. The basal ganglia also inhibit muscle tone in the body. Muscles need to be in a constant state of alert to respond to instructions and to help maintain body posture. This is achieved through a balance of inhibitory and excitatory inputs.

What Causes Damage to the Basal Ganglia

Damage or dysfunction of the basal ganglia cause a range of movement-related disorders. A person suffering from one of these disorders will have difficulty starting, stopping or maintaining movement. The individual may have problems with posture or controlling speech. The symptoms may include involuntary movements or an inability to initiate movement. Muscle spasms may occur, or there may be an increase in muscle tone or rigidity. Different disorders arise from damage to different areas of the basal ganglia. Some common basal ganglia disorders include Parkinson’s disease, Huntington’s disease, and Wilson’s disease. Other disorders that are associated with the basal ganglia include ADHD, Tourette’s syndrome, obsessive compulsive disorder and schizophrenia.

Parkinson’s Disease

Sufferers of Parkinson’s have difficulty in initiating movements. They exhibit tremors, rigid muscles, difficulty making quick, smooth movements and difficulty standing and walking. Parkinson’s develops due to cell death in the substantia nigra, the dopamine producing area of the basal ganglia. The dopamine in these cells innervate the striatum; thus, a loss of these cells results in a loss of innervation and hence leads to a hypokinetic disorder, i.e., one characterized by a lack of movement. Parkinson’s is not a fatal disorder, and with treatment, the symptoms can be alleviated.

Huntington’s Disease

Sufferers of Huntington’s disease make unwanted movements and suffer from memory loss. Huntington’s develops due to cell death in the striatum and produces a hyperkinetic disorder, i.e., one characterized by too much movement. Huntington’s is a fatal disorder, though death usually arises due to complications in the patient.

Treatment of Basal Ganglia Disorders

The treatment of basal ganglia disorders largely depends on the disorder and where the damage occurs. Medication can be used in both Parkinson’s and Huntington’s disease to relieve their symptoms. These medications increase the dopamine available in the brain and help to reduce the jerky, unwanted movements of Huntington’s and the lack of smooth movement with Parkinson’s. However, in both diseases, the medication loses its efficacy over time.

Other treatments are available to those suffering from Parkinson’s disease. These include neurosurgical treatments, for example, where lesions are burned in various areas of the basal ganglia or deep brain stimulation. The targets for the lesions are the thalamus, the pallidum and the subthalamic nucleus. The lesions work by disrupting the direct pathways to the thalamus, which is inhibitory, which in turn increases movement in the patient.

Deep brain stimulation (DBS) can be thought of as reversible or nondestructive lesions as no brain tissue is destroyed. Electrodes are placed inside the target area of the brain and electrical impulses are sent to that area. As with lesions, these impulses disrupt the abnormal signals that cause tremors and other movement-related difficulties.

Current Research about Basel Ganglia Disorders

Current research focuses on stem cell therapy for both Parkinson’s and Huntington’s disease. Stem cells are generic cells that have the ability to differentiate themselves and develop into different specific cells in the body. In the case of Parkinson’s disease, these stem cells would be introduced into the substantia nigra to become dopamine-producing cells. In the case of Huntington’s disease, these cells would become striatal cells. This technique is a reparative therapy designed to halt or slow the progression of the disease. At this stage, there are only trials of this therapy taking place, and it is not a therapy used in practice.

Resources about Basal Ganglia Disorders

Information about Basal Ganglia Disorders in children: Basal ganglia bleeding and Grip disorders in children

Dr. C. George Boeree explains The Basal Ganglia along with an image of the 4 key areas

nih.govBasal ganglia dysfunction: Basal ganglia dysfunction is a problem with the basal ganglia, the deep brain structures that help start and control movement.

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