Disambiguation is also known as Ataxia. This can be described as a condition in which there is a lack of muscle control or coordination of voluntary movements, Examples of these voluntary movements include walking, buttoning your shirt, tying shoes. It is a neurodegenerative disease, in which the control of voluntary muscle movement is lacking. In other words, ataxia is their inability to control muscles associated with movement properly, or entire lack of control. Ataxia is a disease of the nervous system, especially the cerebellum. The cerebellum is the part of the brain that is responsible for locomotion and the movement of the muscles. This disease can affect just one side of the body; this condition is known as hemiataxia. People suffering from ataxia usually have an abnormal gait. There are many factors that are responsible for ataxia, they include stroke, chronic alcohol abuse, some drugs, degeneration of the brain and multiple sclerosis. Stem cells are being used in regenerative medicine to treat brain disorders such as ataxia. Other genetic materials and signaling cells such as exosomes are used in treatment because of its autoregenerative property.
What Are The Signs And Symptoms Of Ataxia?
Ataxia can occur at any time, and can also develop over time. Below are some of the signs and symptoms of ataxia:
- Poor coordination
- Inability to walk, and a tendency to fall
- Problems with speech.
- Difficulty in swallowing.
Types of Ataxia
Ataxia can be categorized based on what part of the brain the defect lies;
- Cerebellar ataxia: Cerebellar ataxia is the term used to describe the ataxia that is due to the abnormal functioning of the cerebellum. The cerebellum is the part of the brain that controls and integrates the brain signals that coordinate locomotion, and body movements. Individuals suffering from cerebellar ataxia may have difficulty with activities that concerns direction, acceleration, speed, movement and so on. This condition results in abnormal and irregular movement. They can also show symptoms like wrong gait, problems with focusing the eyes, problems with the digestive system, muscle tonicity and so on. The early signs of cerebellar ataxia could present as the inability of the patient to stand on one leg, however, the symptoms of the disease get worse, as it progresses. In the later stage, persons affected might require help to stand properly and to walk. In addition to this, patients also have difficulties with speech. They speak slower, as the disease progresses. It would become increasingly difficult to make sense of what they’re saying.
- Dysfunction of the cerebrocerebellum: The cerebrocerebellum area of the brain is the part that controls voluntary movement. Dysfunction of the cerebrocerebellum would lead to the following
- Body trembling- This occurs at rest, but becomes more pronounced when the person to make a voluntary movement.
- Speech problems: Patients with this kind of brain dysfunction usually have problems with speaking. Their speech becomes slurred and irregular.
- Hand tremor: Patients find it difficult to control their hand, especially when it comes to writing. They are not capable of writing legibly, even if they had one before the disease. Also, they would lack the ability to draw straight lines, and also would not be able to write on a line.
- Patients usually have the condition known as dysmetria. This is the inability of the patient to estimate distance. They find it difficult calculating and hitting their target. This comes into effect when making calculated steps, or trying to avoid an obstacle in their way.
- Dysfunction of the spinocerebellum: The dysfunction of the spinocerebellum part of the cerebellum affects the gait of the individual. Patients with this disorder usually walk like someone drunk. Falling is predominant among the people suffering from this disorder. According to statistics, over seventy per cent of this group of people falls at least once a year.
- Dysfunction of the vestibulocerebellum: This has to do with the control of the eyes. Patients with this kind of cerebellum dysfunction find it difficult to control the movement of their eyes. This is also associated with postural imbalance. People with this disorder are not capable of maintaining balance with their legs together. They try to avoid falling by putting a large space between their legs. This can be detected by having them undergo the Romberg’s test. People with this disorder cannot pass this test, because of the fear of falling down.
- Sensory Ataxia
Sensory ataxia can be described as the ataxia that has to do with the loss of proprioception. Proprioception basically means the ability of an individual to be sensitive to the position of the body joint and parts. It is due to the abnormal functioning of the dorsal column of the spinal cord. The dorsal column carries proprioceptive stimulus form the spinal cord to the brain. Any damage or disorder to the track or the dorsal column leads to the inability of the patient to identify the position and his body parts. The Romberg test can also be used to test for this. Patients lack the ability to maintain stability when their legs are together. They tend to widen their legs so they won’t trip for fall.
- Vestibular Ataxia
Vestibular ataxia is used to describe the dysfunction of the vestibular system. Symptoms of vestibular ataxia include vertigo and vomiting, for the acute cases.
Causes of Ataxia
The degeneration or destruction of the neurons in the cerebellum leads to ataxia. The cerebellum is divided into two parts. The left side is responsible for the coordination of the right side, while the right side is responsible for coordination of the left side. The disorders that affect the track that links the spinal cord to the brain can also cause ataxia. Below are some of the common causes of ataxia
- Injury: Injury of different severity can lead to ataxia. This ranges from home accidents to road traffic accidents and so on. Injuries that affect the cerebellum are capable of causing ataxia.
- Diseases due to autoimmunity: Sometimes, the body immune system fails to recognize the body tissues, so they attack them. This is what happens in some conditions such as multiple sclerosis when the T cell antibodies of the body attack the nerve cells. Such condition can lead to ataxia.
- Infections
- Tumor growth: A tumor growth in the brain, benign or malignant would compress the brain parts and vessels. This can also lead to ataxia.
How Is Ataxia Currently Treated?
Ataxia currently has no treatment, however treating the cause of the disorder can stop the disease. For example, in cases where it’s caused by an infection, eliminating the infection would also resolve the problem.
Stem Cell Therapy
Stem cells are cells that can differentiate and proliferate into specialized cells, such as the cells of the cerebellum. Stem cells are being used in regenerative medicine to treat brain disorders such as ataxia, Parkinson disease and so on. The stem cells are infused into the body, where they migrate to the damaged part of the body to repair and replace the badly damaged or dead cells.
References
Gwinn-Hardy, K. (2004). When Is Ataxia Not Ataxia? Archives of Neurology, 61(1), p.25.
Hansen, S., Borland, H., Hasholt, L., Tümer, Z., Nielsen, J., Rasmussen, M., Nielsen, T., Stummann, T., Fog, K. and Hyttel, P. (2016). Generation of spinocerebellar ataxia type 3 patient-derived induced pluripotent stem cell line SCA3.B11. Stem Cell Research, 16(3), pp.589-592.
Ku, S., Soragni, E., Campau, E., Thomas, E., Altun, G., Laurent, L., Loring, J., Napierala, M. and Gottesfeld, J. (2010). Friedreich’s Ataxia Induced Pluripotent Stem Cells Model Intergenerational GAA⋅TTC Triplet Repeat Instability. Cell Stem Cell, 7(5), pp.631-637.
Pandolfo, M., Chintawar, S., Wattenhofer-Donze, M. and Puccio, H. (2012). An Induced Pluripotent Stem Cell Model for Friedreich’s Ataxia (IN8-1.007). Neurology, 78(Meeting Abstracts 1), pp.IN8-1.007-IN8-1.007.
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