The problem of “ cerebellum prolapse
While we don't know exactly why it can occur at any age, we have come a long way in its treatment. Congenital cerebellum prolapse, which manifests itself with symptoms such as headaches that do not go away, swallowing difficulties, or head dizziness, can be eliminated by surgical applications.
However, the cause is not completely clear.”
“prolapsed cerebellum is a serious problem that affects patients and their families. Patients should understand their disease, educate themselves, and collaborate with their doctors in managing their health care throughout all post-diagnosis processes.”
Cerebellar prolapse (Arnold-Chiari disease) is a disease in which the structures of the cerebellum called tonsils move towards the junction of the spinal cord and the spinal cord and compress the nerves passing through it. Therefore, the anatomical structure and shape of the tonsils play an important role in the diagnosis of cerebellar prolapse. According to researches, cerebellum prolapse, which is seen in 1-3 percent of the society, is above all a congenital problem. In other words, it is a developmental anomaly that occurs in the womb. Genetic factors (such as a small posterior cavity where the cerebellum is located) and some syndromes (Klippel-Feil syndrome, etc.), bone anomalies, the presence of a tumor or cyst in the brain, folic acid deficiency during pregnancy, exposure to drugs or chemical agents, and many other causes can be the cause of cerebellum prolapse. This problem, which can also be seen in children, usually occurs between the ages of 20-30 and more frequently in women. Why it occurs at any age is a complete black box.
Symptoms related to compression of the spinal cord and cerebellum may occur due to the effect of cerebellar prolapse. In addition, symptoms can also be seen due to increased pressure in the cerebrospinal fluid (CSF) circulation in the canal located in the middle of the spinal cord, causing obstruction. For example, clinical pictures called "Hydrocephalus" may occur as a result of increased CSF pressure in the brain and "Syringomyelia" may occur as a result of increased pressure in the spinal canal. These two diseases may be associated with other diseases, or they may occur alone.
Ehler-Danlos syndrome, a connective tissue disease (excessive stretching of skin and joint tissue), is considered a subgroup of cerebellar prolapse. Psödotümör Cerebri” is seen in 10 percent of adult patients with prolapsed cerebellum. Complaints include headache causing pressure in the eyes, nausea/vomiting, dizziness, blurred vision, neck, shoulder or back pain.
What is being done for a definitive diagnosis?
Sagging cerebellum can be detected by ultrasonography 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, which may cause the diagnosis process to spread over many years. After listening to the patient's history, the first thing to do when the examination process starts will be to take MRI and CT scans. In this process, some electrophysiological studies and vision and hearing tests are also performed to clarify possible suspicious situations.
Diagnosis is difficult, so what about treatment?
Surgical treatment is performed in half of symptomatic and diagnosed patients, as the diagnosis is difficult. In this process, where each patient needs to be evaluated individually, the importance of surgical experience is undoubtedly very important. Patients with mild problems should first be followed with the "wait and watch" principle. These are usually patients who have been diagnosed incidentally on MRI for other reasons (such as head trauma). Some patients may also be treated with medication because they are recovering from surgery. In such patients, their lifestyle is reorganized and they are given medication for pain. Long-term follow-up and medical treatment may be required to make a surgical decision in patients with only headache complaints. If the patient does not benefit from the "wait and see" phase and wants to improve his/her quality of life by getting rid of his/her complaints, surgery will be the first choice.
After approximately 2-4 hours of surgery, patients are rarely followed up in intensive care for 1 night and are usually discharged after 3-4 days of hospitalization. While the union of the stitches is completed in 7-10 days, the patients' return to their daily life may vary between 3 weeks and 3 months. In severely ill patients and patients with post-surgical complications, the return to normal may be delayed or certain conditions may remain as sequelae.
Surgical Treatment in Patients with Severe Progression
Surgical treatment is performed in patients with syrinx and hydrocephalus (water retention in the brain) who are found to have a problem during the examination, and in whom the compression has increased or worsened. In patients with syrinx, the posterior cavity is usually enlarged by shaving (decompression) and the atlas bone is removed from the posterior side to release the compressing connective tissues. Although some surgeons can use the patch technique by opening the meninges, this method is controversial worldwide. In appropriate cases, if necessary, it is also possible to burn the tissue that causes the compression, called tonsil, and reduce it. In some severe types of cerebellum prolapse, there are also surgical treatment methods such as microsurgery or ventricular operitoneal shunt in addition to decompression surgery.
It is also worth noting that screw fixation surgeries can be performed in patients who may have cervical instability (a nöroological problem) after decompression. In these patients, the degree of curvature of the spine can be monitored and surgery can be performed if necessary; if there is severe compression from the other side, it is also possible to perform decompression surgery by entering through the mouth.
Cystic enlargements due to CSF accumulated in the spinal canal can also be monitored after decompression surgery. These cystic enlargements are reduced within 1-2 years as a result of normalization of CSF circulation by relieving the sagging of the cerebellum. After the surgery, patients return to their daily lives and complaints decrease in almost all patients over time. Complications related to the surgery may be meningitis due to CSF leakage, pseudomeningocele, bleeding or pain in the surgical area, paralysis due to spinal cord injury. The risk of complications is much lower (1-3%) in decompression surgeries performed without opening the cerebral cortex (dura).
Short …
- Sagging cerebellum is a serious disease that affects patients and their families.
- Can cause many symptoms.
- Surgery is the treatment of choice in symptomatic, appropriate patients. The purpose is to maintain the circulation of cerebrospinal fluid (CSF).
- Hydrocephaly, Syringomyelia, Psödotümör Cerebri and Ehler Danlos Syndrome can often accompany this problem.
- Postoperatively, in patients who are not very severe, complaints decrease by 80 percent.
- Surgical outcomes in children are better than in adults.
- Öespecially chronic pain can cause permanent problems in some patients.
- Multiple surgical procedures may be required in very few patients.
- The unknowns about the disease continue to become clearer as research progresses.
- Patients with prolapsed cerebellum should understand their condition, educate themselves, and cooperate with their physicians in managing their health care.
What Types Are There?
- Type 1: The most common mild form. Syringomyelia may accompany. According to clinical findings, it is seen in patients aged 20 years or older.
- When the baby is born, it is born with a problem called spina bifida. It has a sac in the neck. Hydrocephalus is often seen.
- Type 3: It is rare. The cerebellum and brain tissue protrude from the cavity at the back of the head.
- Type 4: Çvery rare and ölümcül course.
TWO MORE TYPES ADDED!
In the latest information, 2 new types have been identified: Type 0 and Type 1.5. These are also mild types
Symptoms of Cerebellar Prolapse
Complaints may vary depending on the degree of pressure and age group:
1 Headache, usually in the nape of the neck (may also be due to hydrocephalus). The pain can be exacerbated in situations that increase intracranial pressure (such as straining, sneezing, sneezing, exertion, exercise). Pain can sometimes be accompanied by nausea and vomiting.
2 Dizziness, loss of balance (due to cerebellum involvement), impaired walking.
3 Symptoms of the spinal cord bulb, such as problems with vision (involuntary movements in the visual cortex called nystagmus), hearing problems (noise, hearing loss), swallowing difficulties, speech and breathing problems, and sleep apnea.
4 Pain in the neck and shoulders, electric çpulsating sensation.
5 Gloss, numbness, inability to feel pain with heat and cold, weakness in bladder and bowel control due to syringomyelia.
6 Rarely, in some adults, psychiatric conditions such as self-harm or suicidal tendencies.
7 In infants, tension in the fontanelles (fontanelles, the bulging of the head), vomiting, swallowing difficulties, growth retardation, frequent restlessness and weak crying, epileptic seizures, vision problems.
