19. In the late 1860s, at the end of the century, the German pathologist Chiari described four types of congenital anomalies in which the cerebellum and brain tissues droop, which he named after himself.
Type 1: Cerebellum prolapse is the prolapse of the lower parts of the cerebellum into the spinal canal in the neck.
Type 2: Cerebellum prolapse is the prolapse of the cerebellum itself into the spinal canal along the lower parts of the cerebellum. In this type of sagging, there is a myelomeningocele sac in the lumbar region.
Type 3: In cerebellum prolapse, it is the presence of myelomeningocele sac in the neck region and brain structures in the sac.
Type 4: In cerebellum prolapse, the cerebellum tissues are not developed, as well as the midbrain tissues.
Type-1 Cerebellum Prolapse:
The main feature of this type of prolapse is that the most extreme parts of the cerebellum protrude into the spinal canal. In this disease, after the circulation of the Cerebrospinal Fluid (CSF) in both the brain and spinal cord canal, pressure changes in the brain and intermittent increase in intracranial pressure occur.
Reasons put forward in the formation of sagging;
1:This anomaly occurs due to the pressure difference between the intracranial pressure and the spinal canal.
2: Depending on the adhesions in the cerebellum, the circulation of the brain water is impaired.
3: The cerebellum tissues are elongated as a result of pervasive developmental disorder.
Type-1 Cerebellum Prolapse:
Type-1 cerebellum prolapse is generally more common in youth. The most common age is 20-30 years. It is more common in women than men. It is very rare in childhood.
Clinical Complaints and Findings:
The most common complaints of cerebellum prolapse are pain in the head, neck, neck and shoulders, especially after intermittent increased intracranial pressure. Headache is such that it wakes you up, especially at night. Headache is usually accompanied by nausea and sometimes vomiting. Headaches are followed by pains and stiffness in the nape region, sometimes radiating to the shoulders. The pain happens from time to time, sometimes not at all. The reason for this is intermittent blockage in the circulation of the brain water. In addition, shoulder, low back or leg pains are seen, these pains are not reflected. Pain and pressure or pressure sensations in the neck, shoulders and arms are the most common complaints. These pain complaints are followed by dizziness and imbalance. As a reflection of the increased intracranial pressure, complaints of blurred vision in the eyes, double vision, pain and pressure behind the eyes and not being able to look at the light and the sun are seen. Neurological findings are seen in 70% of cases, especially motor and sensory disorders are seen in the arms or legs, these findings are evident in those with cysts in the spinal nerve (syringomyelia). In addition, an imbalance of 30-40% is an important complaint. Less frequently, 15-25% of patients have dysphagia, speech disorder, nystagmus, and paralysis of the nerves in the cerebellum. Since the cerebellum is a center of balance, especially fine movements or coordinated movements and sometimes tremors in the hands are seen, since the cerebellum is under pressure. In 30% of childhood and adolescence progressive spinal curvature (scoliosis), it is seen with cerebellum prolapse.
Diagnosis
Today’s best diagnostic method for diagnosing this disease is Magnetic Resonance Imaging (MR). In MRI, the lower parts of the cerebellum extend into the spinal canal. However, this amount of sagging is not very important. Because the causes of cerebellar wrapping are different, for example, even a 3 mm prolapse may cause a complaint of cerebellum prolapse, while a 8 mm prolapse may not cause a complaint. Congenital anomalies such as (small posterior fossa, platybase, atlanto-occipital assimilation or basilar invagination) may accompany this disease. For this reason, it should be carefully investigated whether these diseases coexist with 3D Computed Tomography of the neck region. In the brain tomography, the cerebellum and spinal bones are examined in detail. This diagnostic method is very important in surgical planning. In addition, brain CSF dynamics should be reviewed for cerebral water circulation in the brain. In 50-60% of cerebellum prolapses, cysts (syringomyelia) are seen together in the spinal nerve.
Differential Diagnosis
These patients are usually examined and treated in many different clinics in hospitals.
Due to headache, he is examined and treated in neurology outpatient clinics.
Under the heading of dizziness, it is primarily investigated as vertigo.
It is investigated in neurosurgery orthopedics physical therapy and pain outpatient clinics due to neck hernia.
He is treated by psychiatry clinics under the title of depression.
Chest diseases are triggered under the title of sleep apnea and due to respiratory problems.
Sometimes chest pain is investigated by cardiology clinics because of shortness of breath.
He is examined by gastroenterology clinics with the complaint of nausea.
Demyelinating (multiple sclerosis) disease is diagnosed and treated by the neurology clinic.
Treatment
The treatment for this disease is surgery. Unfortunately, there is no drug treatment for this disease. The main question in treatment is whether this disease gives complaints or not. The answer to this question is very important. The aim of surgical treatment is to relieve the cerebellum under pressure in the neck region and to restore the circulation of cerebral water.
Causes of anterior pressure before performing cerebellum surgery; basilar invagination, platibase, C-1 assimilation should be well defined. In this case, surgical intervention on the neck should be considered after removing the anterior pressure by mouth.
Surgical decision should be very careful in childhood cerebellum prolapse. In this age group, the diagnosis of cerebellum sarcophagus is mostly an accidental finding. There is no definite consensus on surgical treatment in this age group.
In the surgical results of adult group cerebellum prolapse, approximately 100% of the patients who underwent suboccipital craniectomy, C-1 laminectomy, and duraplasty, especially in the neck, improved their complaints, and those with cysts (syringomyelia) had 80-90% reduction and reduction in cysts, depending on the surgical technique. Improvements are seen in complaints.
CONCLUSION
Cerebellum prolapse is generally a congenital disease. In its formation, there is pressure in the cerebellum and inhibition of CSF circulation after a mechanical block. Generally, there are different subjective or objective complaints at the young adult age. The most common of these complaints are headache, neck pain and shoulder pain. 60% of patients have cysts in the spinal cord. In the diagnosis, neck MRI is the gold standard, and the lower parts of the cerebellum extended into the spinal canal. Cerebellum prolapse treatment is surgical. Suboccipital decompression and duraplasty are the most common surgical treatments. The results of the surgical treatment are excellent if the surgeon removes the pressure, if any, in the neck area, the bone, or the reason that prevents the circulation of CSF in the cerebellum. If the cause of cerebellar sagging is not eliminated, unfortunately, improvement in complaints should not be expected. Diseases found in the differential diagnosis of such unsuccessful conditions, other cerebellar diseases and / or the surgical technique performed should be reconsidered.
