Chiari malformation FAQs

Below are answers to questions directly related to Chiari malformation type I (sometimes called Chiari-1 malformation or CM-1 malformation). For more information about Chiari-1 malformation, you can visit the Chiari Medicine blog.

Who gets the Chiari-1 malformation?

Chiari-1 malformation presents in adults, children and occasionally infants. It occurs three times more often in women than in men. There is no particular ethnic or geographic distribution, and the overall cause is not known.

Can it occur in those not born with it?

Yes. This is called secondary Chiari and it can be caused by hydrocephalus, increase of intracranial pressure, tumors that displace the brain structures and trauma. Some people that have a shunt in the lower spinal canal may also develop secondary Chiari. The condition of tethered spinal cord can result in a Chiari malformation.

Can Chiari-1 malformation reoccur?

Some people have recurrent symptoms after a period of good response to surgical treatment. There are various causes for this, and a careful evaluation is needed. Sometimes the cause is not the condition itself, but others, such as raised intracranial pressure, cervical discs causing headaches or occipital neuralgia (pain from the nerves at the back of the head).

Does Chiari-1 malformation cause disability?

Chiari-1 malformation can cause temporary or permanent disabilities. Many troubling symptoms that have prevented working and playing often disappear after surgery. Other symptoms may not be quite as easily resolved, and can take time and effort before you can return to a normal lifestyle.

Is there anything I should avoid?

Pay attention to how you respond to various activities and use this as a guide. For many, lifting weights, straining, bending forward or looking up may worsen their symptoms. Contact sports and activities such as riding high-speed roller coasters may aggravate symptoms.

Why do symptoms show up later in life?

The reason that symptoms in many people do not show up until later in life is unknown. It may be related to the repeated brain pulsations and straining activities resulting in some increased crowding which slowly worsens over time. Many people report symptoms after an injury such as a motor vehicle accident, whiplash and falls.

Why does crowding and poor Cerebrospinal Fluid (CSF) circulation cause symptoms?

The CSF surrounds the brain and spinal cord, circulating constantly and providing protection for the delicate nerves contained within. Most of the symptoms of Chiari-1 malformation can be attributed to obstruction of spinal fluid flow or compression of the lower brainstem and cranial nerves.

Chiari-1 malformation can present in children and infants, and it is important to know how to handle it, both on your end and your child's.

How does Chiari-1 malformation affect children?

Many of the signs and symptoms that occur in adults are also present in children. However, young children may not be able to accurately report their symptoms. Rarely, children may present with cerebellar fits or episodes of paroxysmal rage.

How are children diagnosed?

If you have Chiari-1 malformation and your child has suggestive neurological symptoms, ask your pediatrician about ordering an magnetic resonance imaging (MRI) scan of the brain for a positive diagnosis.

If I have it, will my children have it?

In most instances, Chiari-1 malformation does not run in families. In some cases, however, it can be passed from the mother or father to their children.

How can I help my family and friends understand?

Sometimes it is difficult for family and friends to understand your disease because physical symptoms are usually invisible to others. The best remedy for understanding is education. Consider taking a family member with you when you have an appointment with your neurosurgeon. Have a short list of important questions (about three) that you would like to have answered during your visit.

How can I help protect my unborn child from neural tube abnormalities?

Ask your obstetrician/gynecologist (OB/GYN) about taking the supplement folic acid during your pregnancy.

If pregnant, do I have to have a cesarean section?

No. Many women have normal vaginal deliveries. This decision should be made after a discussion with your OB/GYN.

Is pregnancy possible with Chiari-1 malformation?

Yes. Many women with the condition have normal, uncomplicated pregnancies.

While there are numerous diagnostic imaging scans that are used in medicine to diagnose a wide range of disorders, a Chiari-1 malformation diagnosis primarily comes from an MRI.

What does an MRI scan show?

An MRI creates an anatomical picture of the brain and spinal cord. With this condition, an MRI can show the degree of crowding at the large opening at the bottom of the skull called the foramen magnum. Brain tissue compression and distortion can be analyzed and the shape and changes in the bony structures can be evaluated. An MRI of the spine can also show the presence of syringomyelia.

What is a cine MRI and what is it used for?

A cine study is a special MRI that is used to assess the flow of spinal fluid through the large opening at the base of the skull called the foramen magnum. It is used in some cases to determine if spinal fluid flow is being blocked.

Chiari malformation treatment and diagnosis requires specialized care, so it is important to know where to turn. You should see a neurosurgeon who is experienced in the evaluation and treatment of those with Chiari-1 malformation. A neurosurgeon is a specialized physician that evaluates and treats people with diseases and problems of the nervous system that may require surgery.

We understand that there are alternative therapies out there that some could interpret as beneficial for easing the symptoms of Chiari-1 malformation. However, please be advised that this condition requires specialized treatment from a qualified physician.

Are there any specific herbals that I should avoid before surgery?

Herbal supplements do not have the scientific studies backing their effectiveness or safety that prescription drugs have. The pharmaceutical industry spends millions of dollars developing, testing, running clinical trials and getting FDA approval before a drug is released. The quality and strength of herbal preparations are not regulated in this country. The interactions of many herbal preparations with prescription drugs are not known. Prescription medications interactions with other prescriptions drugs and common foods or drinks are well understood. For example, drinking grapefruit juice while taking blood pressure medication can cause toxicity in some people.

The list below is not inclusive. The herbals listed below may interfere with other drugs or increase the risk of bleeding. They should be eliminated at least two weeks before surgery:

• Chamomile
• Clove
• Dong quai
• Echinacea
• Evening primrose oil
• Fenugreek
• Fever few
• Fish oil
• Garlic
• Ginger
• Gingko biloba
• Ginseng
• Goldenseal
• Horse chestnut
• Kava kava
• Licorice
• Meadowsweet
• Panax
• Poplar
• Prickly ash — northern and southern
• Quassia
• Red clover
• White willow/willow bark

Is it okay for my chiropractor to crack my neck?

High-velocity chiropractic care is not advised in persons with Chiari-1 malformation, as injuries have occurred.

Hydrocephalus is an accumulation of cerebral spinal fluid within the ventricles of the brain, and results from blockage of normal spinal fluid circulation. Hydrocephalus may result from developmental abnormalities, infection, brain injury and brain tumors.

What are the dangers of hydrocephalus?

The dilation of ventricles with spinal fluid places pressure on delicate brain tissues. Symptoms of hydrocephalus may include headache followed by vomiting, nausea, blurred vision, diplopia (double vision), problems with balance and coordination, difficulty walking, urinary incontinence, drowsiness, irritability and other changes in personality or memory changes.

Will surgery make the hydrocephalus better?

In some cases, hydrocephalus will resolve once the blockage of spinal fluid flow has been relieved by Chiari-1 malformation surgery. In others, a shunt may have to be placed in order to remove the excess fluid from the brain ventricles.

Sometimes, it can be difficult to know how to communicate your condition with others, but ultimately, the best answer comes down to each individual.

Should I tell my boss that I have Chiari-1 malformation?

Many people with Chiari-1 malformation or syringomyelia continue to have productive careers long after they are diagnosed. There are many state and federal laws that address work issues for people with disabilities or chronic illness. Check with your state government about available hotlines and information about discrimination in the work place. There are two federal laws that may be useful — the Americans with Disabilities Act (ADA) and the Family and Medical Leave Act (FMLA).

After you know your rights, you may decide if you want your boss to know about your condition.

Symptoms can certainly make life more complicated, but we have solutions for you.

How can I manage my headaches?

Coughing, straining, laughing, bending or lifting often aggravates headaches. Headaches can also be caused by overuse of medications used to treat headaches. Persons suffering from headaches should make sure to drink an adequate amount of water as dehydration can contribute to headaches. Many people report some relief of headaches while resting in a darkened room, occasional use of migraine medication, application of heat or cold to the area and use of certain anti-seizure medications.

Is there anything I can do to help with dizziness?

Abrupt movements of the head and getting up quickly can cause dizziness. It is important to remember that there can be other causes of the symptoms commonly associated with Chiari-1 malformation. A consult with an ear, nose and throat specialist may help to rule out another reason for the dizziness, such as problems with the inner ear. Sudden movements or laying flat may increase dizziness.

What can I take for burning pain in my arms?

As with other types of neuropathic (nerve) pain, narcotic pain pills do not tend to work well. This type of pain is treated in a variety of ways. The most common treatment is with certain anti-seizure medications (gabapentin, pregabalin) that can be used to control neuropathic pain.

What other diseases can cause similar symptoms?

There are many diseases that can cause some of the same symptoms as Chiari-1 malformation. This list does not include all disease states. A partial list of other causes include:

• Alcoholism
• Amyotrophic lateral sclerosis
• Anemia
• Anxiety or panic disorders
• Arachnoid cysts of the cerebellum
• Brain trauma or tumor
• Cerebellar abscess or atrophy
• Depression
• Diabetic neuropathy
• Disorders of the cervical or thoracic spine
• Encephalopathy
• Environmental toxins
• Hypo/hyperthyroid
• Medication toxicity or interactions
• Multiple sclerosis
• Myasthenia gravis
• Neurosarcoidosis
• Polyneuropathy
• Post polio syndrome
• Stroke
• Syringomyelia
• Vitamin deficiency

Why am I depressed and anxious?

Depression and anxiety can be caused by chronic illness, pain and uncertainty. In some instances, these symptoms may be due to the cerebellar cognitive affective syndrome and can improve following decompression surgery. Depending on your personal condition, you may need to be placed on medications for depression and anxiety.

Why am I falling and having trouble walking?

Chiari-1 malformation causes crowding of the cerebellar tonsils. The cerebellar portion of the brain is responsible for the coordination of voluntary muscular movements. The cerebellum interacts with the structures of the brainstem and helps to execute a variety of movements such as balance and walking.