What is cerebellar prolapse, what is done in diagnosis and treatment?

Although we do not know exactly why it can be seen at any age, its treatment has come a long way.

Although we do not know exactly why it can be seen at any age, its treatment has come a long way. Congenital cerebellar prolapse, which manifests itself with symptoms such as persistent headaches, difficulty swallowing or dizziness, can be solved with today’s surgical practices. Anadolu Health Center Brain and Nerve Surgery Specialist Prof. Dr. Selçuk Göçmen said, “Cerebellum prolapse is a serious problem that affects patients and their families. “In all post-diagnosis processes, patients should understand their disease, educate themselves and cooperate with their doctors in managing their health care,” he says. Prof. Dr. Selçuk Göçmen gave important information on the subject.

Shape What is cerebellum prolapse, what is done in diagnosis and treatment? What is cerebellum prolapse and what are its causes?

Cerebellar prolapse (Arnold-Chiari disease) is a disease that occurs as a result of the structures of the cerebellum, which we call tonsil, advancing towards the junction of the spinal cord and the spinal cord and compressing the nerves passing through there. Therefore, the anatomical structure and shape of the tonsils also play an important role in the diagnosis of cerebellum prolapse. He underlines that, according to research, cerebellar prolapse, which is seen in 1-3 percent of the population, is, first of all, a congenital problem. So, this is a developmental anomaly that occurs in the womb. There are many reasons for cerebellar prolapse, such as genetic factors (such as the smallness of the posterior fossa where the cerebellum is located), some syndromes (Klippel-Feil syndrome, etc.), bone anomalies, tumor or cyst in the brain, folic acid deficiency during pregnancy, exposure to drugs or chemical agents. There might be a reason. This problem, which can also be seen in children, usually occurs between the ages of 20-30 and is more common in women. Why it occurs at any age is a complete black box.

Due to the effect of cerebellum prolapse, symptoms may occur due to compression of the spinal cord and cerebellum. In addition, symptoms may occur due to increased pressure in the canal located in the middle of the spinal cord, causing an obstruction in the circulation of the cerebrospinal fluid (CSF). For example, clinical conditions called “Hydrocephaly” may occur as a result of increased CSF pressure in the brain, and “Syringomyelia” may occur due to expansion of the spinal canal as a result of increased pressure. While these two diseases can be seen with other diseases, it is also possible for them to occur alone.

Connective tissue disease (excessively stretchy skin, joint tissue) called Ehler-Danlos syndrome is considered a subgroup of cerebellum prolapse. “Pseudotumor Cerebri” is seen in 10 percent of adult patients with cerebellum prolapse. Their complaints are; Headache that causes pressure in the eyes, nausea/vomiting, dizziness, blurred vision, neck, shoulder or back pain.

How is cerebellum prolapse diagnosed?

Cerebellar prolapse can be detected by ultrasonography performed in the womb. However, in the years after birth, it can often be confused with other diseases, depending on the complaints. There is no single test that can detect the disease. Sometimes patients may see many doctors from different specialties due to their complaints; This can cause the diagnosis process to extend over many years. After listening to the patient’s story, the first thing to do when the examination process begins is to take MRI and tomography images. During this process, some electrophysiological studies and vision and hearing tests are also performed to clarify possible suspicious situations.

How is cerebellum prolapse treated?

Since the diagnosis of the patients is difficult, surgical treatment is performed in half of the symptomatic and diagnosed patients. In this process, where each patient must be evaluated individually, surgical experience is undoubtedly of great importance. Patients with mild problems should first be followed with the “Wait and See” principle. These are generally patients who were diagnosed incidentally during an MRI performed for other reasons (such as head trauma). Since some patients are reluctant to undergo surgery, drug therapy may also be applied. In such patients, their lifestyles are rearranged and medications are given for pain. In patients who only complain of headache, long-term follow-up and medication may be required to decide on surgery. If the patient does not benefit from the “Wait and See” phase and wants to improve his quality of life by getting rid of his complaints, this time the first option will be surgery.

After the surgery, which takes approximately 2-4 hours, patients are rarely monitored in intensive care for 1 night and are usually discharged after staying in the hospital for 3-4 days. While the healing of the stitches takes between 7-10 days, it may take between 3 weeks and 3 months for patients to return to their daily lives. In seriously ill patients and patients with post-surgical complications, return to normal may be later or certain disorders may remain as sequelae.

Who is operated on and what surgical methods are used?

Surgical treatment is performed in patients whose examination reveals problems, who have syrinx and hydrocephalus (water collection in the brain), and whose congestion increases or worsens. In patients with accompanying syrinx, the posterior fossa is usually enlarged by shaving (decompression) and the atlas bone is removed from the back to release the compressive connective tissues there. Although some surgeons can use the patch technique by opening the brain membrane, this method is a controversial issue in the world. In appropriate cases, it is also possible to reduce the size of the tissue causing congestion, called tonsil, by burning it, if necessary. In some severe types of cerebellar prolapse, other than “decompression surgery”, there are also surgical treatment methods such as microsurgery or “Ventriculoperitoneal Shunt”.

We should also point out that screwing surgeries can be performed on patients who may have cervical instability (a neurological problem) after decompression. While the degree of spinal curvature seen in these patients can be monitored and surgery can be performed if necessary; If there is serious pressure from the front, it is also possible to perform decompression surgery by entering through the mouth.

Expansions due to CSF ​​accumulated in the spinal canal can also be followed after decompression surgery. These cystic enlargements resolve within 1-2 years as the cerebellar prolapse is relieved and the CSF circulation returns to normal. While the patients return to their daily lives after the surgery, the complaints decrease and disappear over time in almost all of the patients. Complications related to the surgery are; It may occur in the form of meningitis due to CSF ​​leak, pseudomeningocele, bleeding or pain in the surgery area, and paralysis problems may occur due to injury to the spinal cord. In decompression surgeries performed without opening the brain membrane (dura), the risk of complications is very low (1-3%).

Quick Facts

• Cerebellar prolapse is a serious disease that affects patients and their families.

• It can cause many symptoms.

• The real treatment for symptomatic, suitable patients is surgery. The aim is to ensure circulation of cerebrospinal fluid (CSF).

• Hydrocephalus, Syringomyelia, Pseudotumor Cerebri and Ehler Danlos Syndrome can often accompany this problem.

• After surgery, complaints improve by 80 percent in patients who are not very severe.

• Surgical outcomes in children are better than in adults.

• Chronic pain, in particular, can cause permanent problems in some patients.

• More than one surgery may be required in a very few patients.

• The unknowns about the disease continue to become clearer as research progresses.

• Patients with cerebellar prolapse must understand their disease, educate themselves, and collaborate with their doctors in managing their healthcare.

What types are there?

• Type 1: The most common mild form. It may be accompanied by syringomyelia. According to clinical findings, it is seen at the age of 20 or later.

• Type 2: It occurs with the problem we call spina bifida when the baby is born. It has a pouch on its neck. Hydrocephalus is common.

• Type 3: It is rare. The cerebellum and brain tissue protrude from the gap formed at the back of the head.

• Type 4: It is very rare and fatal.

Two more types have been added.

In the latest updated information, 2 new types have been identified: Type 0 and Type 1.5. These are also mild types.

Cerebellar prolapse symptoms

Complaints may vary depending on the degree of pressure and age group:

1. Headache that usually starts from the nape of the neck (may also be due to hydrocephalus). Pain may intensify in situations that increase intracranial pressure (such as straining, sneezing, coughing, exercise). Pain may sometimes be accompanied by nausea and vomiting.

2. Dizziness, loss of balance (due to cerebellum involvement), gait disturbance.

3. Spinal cord symptoms such as vision problems (involuntary movements of the eye, which we call Nystagmus), hearing problems (tinnitus, hearing loss), difficulty swallowing, speech and breathing problems, and sleep apnea.

4. Pain in neck and shoulders, feeling of electric shock.

5. Due to syringomyelia, weakness in the arms and legs, numbness, inability to feel hot and cold and pain, and weakening of bladder (urinary bladder) and bowel control.

6. Rarely, in some adults, psychiatric conditions such as self-harm or suicidality.

7. In babies, tension in the fontanelles (fontanelles, enlargement of the head), vomiting, difficulty in swallowing, developmental delay, frequent restlessness and weak crying, epileptic seizures, vision problems.