Diseases that affect the brain are rare but serious and often have life-long complications. Different parts of the brain can be affected and one such susceptible part is the cerebellum. Read on, to learn about some various disorders that affect this brain area.
The way you walk, stand upright, balance yourself while moving, move your limbs and even speak, is all thanks to the cerebellum, a key part of the brain, located in the lower half of the brain. The cerebellum (Latin for little brain) is in charge of the body’s motor functioning. It is not responsible for movement but can be thought of as a quality checker of the brain’s output, with respect to movement. That is why, if damaged or injured, the body will not be paralyzed or lose the ability to perform movement but rather, the quality and form of movement is damaged. In this article, learn about some cerebellar disorders and how they manifest themselves in an individual.
Various Cerebellar Disorders
Dandy-Walker Syndrome
This is a relatively rare brain malformation disease, which is genetic in occurrence. The brain organ itself is malformed or defected. An entire area of brain mass between the cerebellar vermis (the 2 separate hemispheres of the cerebellum) is missing or maybe partially formed. Cysts or boils are present towards the base of the skull. A ventricle which allows fluid to flow between the brain and spinal cord, is abnormally large.
Due to such an abnormality in structure, pressure within the brain is increased or the regions of fluid, which surround the brain are increased in size. A key physical symptom of this disorder is a malformed head structure. The skull may bulge at the back and the individual’s head is unusually large in size. It is hereditary in occurrence with an estimated 1 case occurring in 25,000 births.
Joubert Syndrome
This is another disorder which is genetic in contraction. The cerebellar vermis is a small area or mass of flesh, which separates the 2 hemispheres of the cerebellum. With this syndrome, the cerebellar vermis is deformed or completely absent. The stem or bone connecting the brain to the spinal cord is also absent or partially formed. Hence motor signals and messages from the brain are incomplete and cannot even be communicated properly to the rest of the body.
Individuals suffering from this disease may have extra digits and a deformed lip (cleft lip). Being hereditary in infection, Joubert syndrome is caused by multiple gene mutations. Both parents must have a copy of the mutated gene for the syndrome to affect their child. But some cases of this syndrome are not inherited. It is quite rare in occurrence, with 1 case in approximately 100,000 births.
Cerebellar Hypoplasia
This disorder is characterized by an underdeveloped or small-sized cerebellum. Different parts of the cerebellum may be missing or deformed and will not function normally. Such a disturbance in development can be a symptom of other disorders like Williams syndrome or Walker-Warburg syndrome. This disorder can be inherited or occur sporadically due to other factors like substance abuse, viral infections and thyroid imbalance. Its extent can also differ based on individual cases. It can worsen in effect over time or remain static.
Von Hippel-Lindau Disease
This disorder is named after the mutated gene responsible for its cause. The Von Hippel-Lindau suppressor gene is present on chromosome 3. The absence or defective copies of this gene will cause numerous tumors and cysts to grow in parts of the body with a lot of blood vessels. Areas include the kidneys, retina, genital tract and the cerebellum.
Due to the different body areas affected and the tumor size, symptoms can vary in occurrence and behavior. This condition is genetic in nature with an autosomal characteristic, where only parent needs to have the gene for the disease to affect the child. So with an affected individual, a child has a 50% chance of inheriting the disease. It is also rare in occurrence, with an approximate number of 1 case in every 32,000 births.
Cerebral Palsy
This disease is actually a group of various disorders, with different areas of the body being affected. But the cause and the expected behavior of the disorder are the same. When the cerebellum is damaged or injured in the early stages of its development (fetal stage, during childbirth, infancy), then the motor skills and physical movement system of an individual is compromised. There are different sub-types and classifications of this type of disorder and it is one of the most common cerebral illnesses, with an incidence of 2 cases in every 1,000 births.
Cerebellar Disorder Symptoms
Due to the sensitivity of the affected area, the signs and indications of this disease are very severe and mostly noted in the motor skills of the individual. Some of them are:
- Ataxia – muscular movements are uncoordinated and jerky
- Difficulty in balancing the body
- Abnormal walking gait
- Tremors and shakes
- Poor or lack of muscle tone
- Severe speech problems
- Cannot react or move quickly or change movements
- Cannot control extent of movement
- Rapidly twisting and rolling of the eyeballs which is completely involuntary
Treatment of cerebellar disorders is restricted to physical therapy and learning to live with the lack or defect in motor skills. With some disorders or nervous system, the damage done can cause an individual to require assisted living. Medications are prescribed only when there are painful symptoms. Disorders of the cerebellum are rare in occurrence but their impact can be very damaging and severely affect the individual’s quality of life.
