Cerebellar sagging is a condition in which the lower end parts of the cerebellum, called the tonsil, herniate from the skull downwards into the spinal canal. Also called Chiari malformation, the disorder is named after Austrian pathologist Hans Chiari, who identified and categorized abnormalities associated with the disease in the 1890s. It is also known as Arnold-Chiari malformation. If cerebellar sagging occurs during fetal development in the womb, it is called primary or congenital chiari malformation. In adulthood, it gets the name secondary CM if it occurs as a result of accidents or infections that cause the cerebrospinal fluid to drain.
Ingredients
What is cerebellar sagging?
What are the types of cerebellar sagging?
What are the symptoms of cerebellar sagging?
Symptoms of cerebellar sagging in infants
What are the causes of cerebellar sagging?
Does cerebellar sagging progress?
How is cerebellar sagging diagnosed?
How is cerebellum sagging treatment performed?
How is cerebellar sagging surgery performed?
How is the course and life expectancy in cerebellum sagging?
What is cerebellar sagging?
Cerebellar sagging is a condition in which the lower end parts of the cerebellum are displaced downwards from the opening called the foramen magnum, located in the neck part of the skull. Herniated tissue blocks the normal flow of cerebrospinal fluid (CSF). Instead of cerebrospinal fluid moving easily through the opening, it pushes the cerebellum further down. The blockade can cause fluid to accumulate in the spinal cord (syringomyelia) or in the brain (hydrocephalus).
Chiari syndrome is often misdiagnosed because it causes a wide range of symptoms, such as headache, neck pain, dizziness, arm numbness, sleep problems. Headache worsened by coughing, sneezing or leaning forward in the back of the head is an important sign for differential diagnosis.
Treatment options depend on the type of chiari and the severity of the symptoms. If the symptoms are mild, the patient is monitored at regular intervals December and medications are used to control complaints. If the symptoms are severe or getting worse, surgical treatment is required. The surgical procedure is performed by removing part of the skull bone in order to make room for the cerebellum and brain stem.
What are the types of cerebellar sagging?
Type 1: This is the most common type. It can be seen in both children and adults. In this type, the back part of the skull is small or deformed, so the brain stem and cerebellum are squeezed. The cerebellar tonsils hang down from the skull, preventing the flow of CSF. Chiari 1 can cause a fluid-filled cyst (syrinx) inside the spinal cord.
Type 2: Present at birth and affects babies. It is accompanied by a birth defect called spina bifida, which is a spinal cord anomaly. In spina bifida, the spine does not close before birth, and part of the spinal cord protrudes like a pouch from this opening in the baby's back.
Type 3: This is a rare but serious herniation of the cerebellum that occurs in infants. It occurs together with a birth defect called encephalocele. Encephalocele is the formation of a fluid-filled sac at the back of the baby's neck.
Type 4: This is a rare and often fatal malformation seen in infants. It occurs when the cerebellum does not develop properly.
Type 0: It is a newly defined Chiari form. There is no herniation in the cerebellum in this form. But there are some symptoms of the disease.
What are the symptoms of cerebellar sagging?
In general, the more brain tissue that sags into the spine, the more severe the symptoms will be. Symptoms vary depending on the type of disease, excessive fluid accumulation in the brain or spinal cord, and the amount of pressure on tissues or nerves. Since the disease mainly affects the cerebellum, symptoms typically include balance, coordination, vision and speech. The most common symptom is a headache that increases in severity with activities such as exercising at the back of the head, leaning forward, sneezing, pushing. Some of the other common symptoms are:
Dizziness
Neck pain
Numbness or tingling in the hands and feet
Difficulty swallowing
Weakness, pain and loss of temperature sensation in the upper part of the body
Hearing loss
Loss of balance or difficulty walking
Other less common symptoms include general weakness in the body, ringing in the ears, curvature of the spine, Decelerated heartbeat and abnormal breathing.
Symptoms of cerebellar sagging in infants
If babies have sagging cerebellum, the following symptoms may be seen:
Swallowing problems
Restlessness during eating
Burpees
Vomiting
Respiratory disorders
Neck containment
Developmental delays
Difficulty gaining weight
Loss of strength in the arms
Symptoms that may occur if type 2 is accompanied by excessive fluid collection in the brain (hydrocephalus);
Big head
Vomiting
Epileptic seizures
Irritability
Delay in development
Sometimes, the symptoms appear suddenly and quickly and require urgent treatment.
What are the causes of cerebellar sagging?
If cerebellar sagging is caused by prenatal causes, it is called primary, if it has developed due to a condition that causes cerebrospinal fluid to drain, such as an accidental infection, it is called secondary brain sagging.
Various factors can affect the development of the fetus during pregnancy, causing primary brain sagging. Some of these factors are;
Genetic mutations
Deficiency of important vitamins and nutrients such as folic acid during pregnancy
Infection or high fever during pregnancy
Exposure to dangerous chemicals, illegal drugs or alcohol during pregnancy can be sorted in such a way.
Does cerebellar sagging progress?
If cerebellar sagging blocks the flow of cerebrospinal fluid, it may sag more downward due to the effect of pressure caused by excessive amounts of fluid accumulated in the brain. An increase in herniation can lead to new complaints and exacerbation of acute symptoms. In this case, the disease should be treated with a surgical procedure.
How is cerebellar sagging diagnosed?
Sagging of the cerebellum is often diagnosed during ultrasound tests performed in the womb or during childbirth. In individuals who do not have any symptoms, they may be diagnosed by chance during tests performed for other purposes. Physical examination of patients suspected of cerebellar sagging may be affected by the disease;
Balance
Cognitive functions
Memory
Motor skills
Reflexes
The senses are evaluated.
Imaging methods such as computed tomography, magnetic resonance imaging and direct radiographs can also be used to help with the diagnosis. The resulting images help in the search for abnormalities in bone structure, brain tissue, organs and nerves.
How is cerebellum sagging treatment performed?
Treatment differs according to the type, severity and symptoms of sagging. Some medications may be prescribed for mild pains that do not affect daily life much. If the disease causes serious symptoms or damages the neural tissues, surgical treatment is required. The type and number of surgeries needed depends on the patient's condition.
How is cerebellar sagging surgery performed?
Brain sag surgery in adults is performed in the form of cutting and removing part of the skull bone. The exposed part of the brain is then sealed with tissue taken from another part of the body. This process relieves the pressure on the spine by allowing more space for the cerebellum and brain stem. If necessary during surgery, the cerebellar tonsils are reduced using an electric current. It may also be necessary to remove a small part of the spine to provide more space.
Congenital defects such as spina bifida and encephalocele that accompany cerebellar sagging are treated with surgery performed for infants and children. The surgery is performed to reposition the spinal cord and close the openings in the neck or back. If you have hydrocephalus, a tube is inserted to drain excess fluid from the brain to reduce intracranial pressure. The surgical procedure is performed to alleviate the severity of the symptoms.
How is the course and life expectancy in cerebellum sagging?
Surgical treatment can help alleviate the symptoms, but the evidence from research is not sufficient as to how effective the treatment is. According to research, some complaints are more likely to recover after surgery than others. The result is different for each case and more than one surgery may be required depending on the situation. In the long term, people who have undergone surgery to treat chiari need to be monitored frequently and tested repeatedly to check for changes in their symptoms and function. The course of the disease and the expected life expectancy vary depending on different factors such as age, type of sagging, general health status, response to treatment.