Arnold chiari malformation – causes, symptoms & treatment

Arnold-Chiari malformation

The Arnold-Chiari malformation is a developmental disorder that displaces small brain parts out of the brain. Patients often suffer from the first symptoms only in their teens, which usually correspond to nonspecific symptoms such as dizziness. The therapy should prevent progressive damage in the central nervous system.

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What is an Arnold Chiari Malformation??

Malformations are malformations that are usually innate. The group of Chiari malformations is accordingly also referred to as a group of Chiari malformations and affects the rhombencephalon. This is the unit of myelencephalon and metencephalon, which are affected by malformations of kogenitalen relocation.

The group of Chiari malformations is in the broadest sense a group of [developmental disorders in children | Developmental Disorders]], which shift cerebellar parts into the spinal canal and at the same time go hand in hand with a diminished pelvic pit. Modern medicine distinguishes four types of malformation, known as type I to type IV. The effects and manifestations of the malformation differ depending on the type.

The deformity group is named after the pathologist of Chiari, who first described the malformations in the 19th century. Later, the students of the German pathologist Arnold propagated the malformations, especially the Chiari malformation type II, since then known as Arnold-Chiari malformation.


Depending on the severity and localization, the medicine distinguishes four types of Chiari malformation, which have different causes. In type II, and thus the Arnold-Chiari malformation, there is a caudal displacement of the medulla oblongata. In addition, the lower parts of the vermis cerebelli and the fourth cerebral ventricle are displaced caudally. The cause of the malformation is a malformation of the brainstem.

This malformation is primarily triggered by an embryonic developmental disorder that affects approximately the time between the sixth and the tenth week of pregnancy. The skull undergoes bony malformations. The same applies to the first cervical vertebrae. Under the skull, the posterior parts of the brain such as the cerebellum or the cerebellar tonsils do not find enough space and shift because of lack of space at the transition between the skull and the spine.

For this reason, the brain parts protrude after the displacement of the occipital hole in the spinal canal. Which circumstances exactly cause the developmental disturbance during pregnancy, has not yet been conclusively clarified. Exposure to toxic substances is also considered, as are any bottlenecks in nutrients in the mother‘s maternal diet, trauma or infection. Familial accumulations could be observed, so that also genetic factors are considered causally.

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Symptoms, complaints & sign

Patients with Arnold-Chiari syndrome or Chiari malformation type II suffer from mild to severe cerebellar displacements, which occur in combination with a malformation of the brainstem. The cerebellar tonsils, the cerebellar worm or even the brainstem of the patients dodge because of lack of space in the spinal canal.

The Hinterhauptloch the affected is usually greatly expanded. In the displaced nerve tissue, cell death of the neurons occurs. Patients thus lose portions of their functioning brain mass. In many cases, the ventricles of the brain are also affected by changes in which the so-called cerebrospinal fluid is formed as brain and nerve water. These pathological changes in the ventricles often cause a clogged CSF.

In this way, a water head can be favored. So the skull grows in volume. Not all sufferers suffer from a hydrocephalus. Sometimes there are no symptoms until adulthood. Conceivable symptoms include nonspecific complaints such as neck pain, balance disorders, muscle weakness.

Also, sensory disturbances, visual disturbances or dysphagia. Also ringing in the ears to hearing loss, nausea with vomiting, insomnia or depression and pain may be accompanied by a Chiari malformation.

diagnosis & course

The diagnosis of an Arnold Chiari malformation is usually made by accident. Radiological examinations, computed tomography or magnetic resonance imaging are the common imaging techniques that demonstrate an Arnold-Chiari malformation. In most cases, imaging is not ordered because there is already a suspected diagnosis of the malformation.

However, it is undoubtedly possible to detect the displacements of the brain substance, especially from MRI, and the basis for a diagnosis is given. In most cases, a favorable prognosis for malformation patients applies. This is especially true for people who have remained almost completely symptom-free until diagnosis. The lack of symptoms may persist over a lifetime.


The Arnold-Chiari malformation has various complications and complaints that depend on the type of disease. Type 1 is usually only a headache or pain in the neck. If parts of the brain are affected, there is often a gear insecurity or other balance disorders.

Type 2 Arnold Chiari malformation can cause malformations in the brain. These often lead to speech disorders, thought disorders and other defects. In Type 3 and Type 4 disability occurs due to underdevelopment of the brain. However, the nature of the disability can not be universally predicted.

The Arnold Chiari malformation reduces the life of the patient relatively severely. In many cases, it is no longer possible for the patient to master everyday life alone, so that it depends on the help of family and carers. The treatment itself can only be done if the Arnold Chiari malformation is not innate.

However, in most cases, the brain can not regain its normal state of health and disability continues. However, the severity of these disabilities is different for all people.

When should you go to the doctor?

An Arnold Chiari Malformation is usually detected during a routine examination. A targeted medical examination is useful for external symptoms such as muscle twitching or a water head. In severe external changes, the examination and diagnosis usually takes place directly after birth. In its weak form, an Arnold-Chiari malformation can be relatively symptom-free until adolescence and go unnoticed.

In later life, however, the disease manifests itself by increasing difficulty swallowing, blurred vision, sensory disorders, hearing loss and depression. Sufferers who suddenly notice one or more of these symptoms should talk to the family doctor or a specialist in developmental disorders. Although Arnold Chiari malformations are rare, but the complaints mentioned at least another underlying condition that must be clarified.

If an Arnold Chiari Malformation is present, the medical treatment should not be maintained. Above all, symptoms such as nausea, insomnia and increasing intracranial pressure require immediate treatment, as otherwise severe physical and mental complications threaten. Usually, lengthy aftercare and therapy are required following treatment.

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treatment & therapy

A causal therapy does not exist for patients of Arnold-Chiari malformation. A symptomatic treatment can be done and is especially indicated with increasing intracranial pressure. After a hydrocephal impoundment by herniated cerebellar tonsils, for example, a decompression must be carried out on the basis of a suboccipital access.

If pain persists or symptoms such as nausea, insomnia and depression occur, the symptomatic treatment usually consists of a drug therapy. Functional disorders due to the malformation can only be corrected surgically. Such corrections are indicated, for example, when the damage threatens to progress in the central nervous system. The surgical intervention can take place, for example, at the posterior fossa.

By doing so, the surgeon creates more space for the cerebellum while reducing pressure on the spinal cord. In part, small parts of the skull base are removed for this purpose. All abnormal bone structure can be surgically corrected. In addition, the removal of the back on the first cervical vertebra comes into question. This procedure enlarges the spinal canal and protects the worn nerve tissue from pressure damage.

The most common treatment for the treatment of a water head is the diversion into another body cavity, such as in the abdominal cavity. A subcutaneous tube is placed between the brain and the abdomen. The most important goal of all treatment steps is the pressure relief of the nervous tissue.

Compressions of the neurons can cause the nerves to die in the worst case. This phenomenon should be prevented by the therapeutic steps as far as possible in order to reduce consequential damage to the malformation.

view & forecast

The healing prospects for the Arnold Chiari Malformation are unfavorable. The disease can not be cured with the existing medical options and therapeutic approaches. The treatment of the patient is aimed at reducing concomitant symptoms. In addition, a deterioration of the general state of health should be prevented. Without treatment, developmental consequences can occur.

Within the normal growth process, complications arise that can cause malfunction or system failure. Placed by the lack of space of tissue below the skull abrupt bleeding in cortical regions, threatening a stroke. This is associated with lifelong consequences and can lead to premature death of the patient.

With a treatment, the conditions can be created by surgical interventions, so that the growth process does not lead to any threatening disturbances. The aim of the treatment is to create as much space as possible for organs and vital tissue. Redirections of the vessels are created and pressure damage to muscles and nerve fibers should be avoided.

In addition, the individual sequelae are treated in different therapeutic approaches. It can lead to corrective surgery on the spine or psychological impairments are treated. The disease has a progressive course of disease until the end of the physical growth process. Therefore, this is usually stopped early by a hormonal treatment. Subsequently, there is no further increase in malformations of the body.

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So far, little is known about the factors that cause the Arnold-Chiari malformation. For this reason, there are no preventive measures for the malformation.


The Arnold Chiari malformation must first be examined and treated by a doctor. The options for aftercare are very limited, so that sufferers are primarily dependent on medical treatment. A self-healing can not occur in the Arnold-Chiari malformation, whereby the possibilities of self-help are severely limited.

Increasing pressure in the brain is usually treated by surgery. Those affected should rest after such an intervention. You should refrain from physical exertion and physical activity in order to accelerate healing. Stress should also be avoided in Arnold Chiari malformation. In general, a healthy diet with a balanced lifestyle also has a very positive effect on the course of the disease.

The sooner the disease is detected, the greater the chance of a complete cure of the Arnold Chiari malformation. In some cases, the disease can also reduce or limit the life expectancy of the person concerned. Contact with other people affected by the Arnold-Chiari malformation can also have a positive effect on the course of the disease since information can be exchanged.

You can do that yourself

Patients with Arnold-Chiari malformation suffer from increasing symptoms, especially from puberty, which often delay diagnosis due to their diffuse nature. Symptoms such as dizziness, weak muscles or disorders of the sense of balance affect the quality of life and everyday life of patients.

In addition, there are often complaints of visual function and sensibility disorders. Some of the patients develop sleep problems, which in turn have a negative impact on the well-being and health of the person affected.

Under certain circumstances, the disease causes impairment of speech ability, so that a logopedic therapy makes sense. The treatment either improves the condition or further worsens it to a certain extent.

Basically, the medical treatment of the disease in the foreground, with the drug treatment plays an important role. Periodic medical examinations monitor the condition of the patient to address complications or acute deterioration.

Nevertheless, the illness results from the enormous mental stress in some cases in mental complaints to depression. This not only affects the patients, but may also affect the parents of those suffering from the stress of caring for the sick child. In such cases, a psychological therapist is the correct contact person.

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Christina Cherry
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