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"When symptoms such as headaches, dizziness, and fatigue persist, they often rob a person of their quality of life. Though not everyone experiencing these symptoms is suffering from Chiari I Malformation, for those that are, we're here to provide answers and options." - John Oró, M.D.
CHIARI I MALFORMATION
The Chiari malformation (CM) was first identified by Dr. Hans Chiari in 1891. It is an uncommon, complex, neurologic deformity which is present at birth.The brainstem normally sits in a funnel-like cavity just above the spinal cord. The problem occurs when the posterior fossa is not formed properly. Instead of the brainstem sitting in its proper space, the cerebellar tonsils are displaced downward into the funnel, thus causing pressure on this part of the brain and spinal cord (Figure-1).
Chiari_Overview_Fig1.jpg
There can also obstruction of the normal flow of CSF through this area because the tonsils of the cerebellum are “stuffed” into the funnel. Sometimes the brainstem appears atrophic (or shrunken), or elongated (stretched). This downward displacement of the cerebellar tonsils is called the Chiari malformation. Although there is a form of Chiari malformation which occurs in children with meningomyelocele, the Chiari I malformation more frequently causes problems in adults.
The exact cause of the Chiari malformation is unknown. It has been suggested that the Chiari malformation occurs during early embryo development of the brainstem and spinal cord. An abnormally small posterior fossa forces the brainstem downward. Persons with Chiari I malformation often have no symptoms until the third or fourth decade of life.The Chiari I malformation occurs more often in women than men. There is no particular ethnic or geographic incidence of this disorder. The Chiari malformation does seem to run in some families, but a specific gene has not been identified.
Some people with Chiari I malformation also have a syrinx (se-ri-nx) or syringomyelia (Figure 2).
CM-I Symptoms
Symptoms from the Chiari I Malformation often develop in a slow and insidious manner. Frequently, the symptoms are vague and progress over a number of years. Many people have symptoms for years before they are properly diagnosed. A few patients develop symptoms after a mild or moderate trauma. Examples include falling off a trampoline, falling off a porch or a blow to the back of the head.
Headache
The most common symptom of the Chiari I Malformation is headache. It usually begins at the back of the head and radiates behind one or both eyes, to the top of the head or to the temples. The headache is often described as a pressure sensation but can be heavy, sharp or stabbing. For many, the pain varies between these and other sensations.
The headache is often made worse by straining activities (known as valsalva maneuvers) such as coughing, sneezing or even laughing or singing. Some patients report feeling as though "my head will explode" or "my head will pop off." Bending forward or looking up can worsen the headache. When the headache is intense, some patients note dizziness, blurring of vision, nausea or a feeling of being in a "mental fog."
Visual Symptoms
A variety of visual symptoms can occur with CM-I including double vision, decreased peripheral vision, seeing flickers of light ("fireflies"), visual loss, blind spots, photophobia (sensitivity to bright lights), spasm of the eyelids (blepharospasm) and jerking of the eyes (nystagmus).
Dizziness & Auditory Symptoms
Dizziness is frequently seen in persons with CM-I. It is usually intermittent, often worse with the headache, and more noticeable when changing position. A sensation of spinning (vertigo), ringing in the ears (tinnitus), poor balance (disequilibrium) and decrease or loss of hearing may also occur.
Difficulty Swallowing & Hoarseness
People with CM-I may develop difficulty swallowing (dysphagia), either with liquids or solid foods, or a feeling of "catching" in the throat when attempting to swallow. The progression of dysphagia may be rapid and result in aspiration (inhaling fluid or food products into the lungs). Change in voice character and timber are common complaints. Hoarseness is often noted first by a family member or friend. Some patients report the inability to modulate voice when singing or speaking loudly.
Pain
Persons may complain of neck or arm pain. Often the pain is worse with exertion, fatigue, or lifting. The pain may be dull and aching, or shooting and stabbing. It is usually present or worse on one side. Along with pain, some patients experience weakness of the hand or arm, or difficulty with fine movements of the fingers. If syringomyelia is present, the pain may be more widespread.
Numbness
Tingling, numbness, or pins and needles sensation often occur in the same areas as the pain. Numbness can progress over months to years and involve the lower extremities and trunk. These symptoms are usually more noticeable if a person has a syringomyelia (also called syrinx).
Problems Walking
Problems walking may be described as unsteadiness or listing to one side. Persons may report falling or bumping into walls or doors. Infrequently, patients may have spasticity (stiffness and jerkiness) of the legs, which occurs more commonly with syringomyelia.
Respiratory, Heart & Abdominal Symptoms
Effects of the CM-I on the respiratory and cardiovascular systems can cause shortness of breath, chest pain, episodes of rapid heart rate (tachycardia), black out spells, and hypertension. Abdominal symptoms may include nausea, abdominal pain, or vomiting.
General & Cognitive Symptoms
These include poor sleep (insomnia), fatigue and depression. Some note problems with memory, thinking, speech and difficulty with word finding. People may report the feeling of a "brain fog" with difficulty in thinking and concentration. Difficulty in finding the right word may occur.
Neurologic Examination
While being evaluated for Chiari malformation (CM-I), it is important to have a thorough neurologic examination. All of some of the following tests may be done during the clinic visit.
1- Cognitive evaluation- asking such questions as the date, place and your full name helps to determine if there is a problem with thinking and memory.
2- Reflexes- Using a small reflex hammer, the areas of the arms, knees, and ankles will be lightly tapped to determine if the reflex are normal and symmetric.
3- Cerebellar function- Because the cerebellum is the area of the brain that controls coordination of movement, tests will be done to determine if this is functioning properly. You may be asked to walk, touch finger to nose, stand with eyes closed and maintain balance, and perform certain movements of the hands and fingers.
4- Cranial nerves- There are nerves within the brain that control a variety of movements and and sensation. Shining a light in each eye determines if the pupils react properly. Hearing, speech, tongue movement, eye movement and facial sensation/movements will also be checked.
5- Sensory evaluation- Sensation is checked using light touch and pinprick (gently with a small sterile pin). This will determine if there are any areas with numbness or decreased sensation.
6- Motor examination- The motor examination tests strength in the face, arms and legs. Walking, standing, sitting, and other muscle movements will be tested.
Imaging Studies
An MRI (Magnetic Resonance Image) of the brain and/or cervical spine is the test of choice for diagnosis of Chiari malformation (CM-I). An MRI scan creates an anatomical picture of the brain and spinal cord. In CM-I it can show the degree of crowding at the large opening at the bottom of the skull (called the foramen magnum). The MRI can show compression and/or distortion of the cerebellar tonsils, and can also analyze the shape and changes in the bony structures skull and upper cervical spine.
An MRI of the spine can also show the presence of syringomyelia (the collection of abnormal fluid in the spinal cord). Additional information about syringomyelia can be found under the realted disorder section. Tethered cord can be diagnosed with an MRI of the lumbar spine (read more under related disorders section).
A CINE flow study is an MRI study that evaluates the flow of spinal fluid through the area of the foramen magnum. The CINE MRI is performed in the MRI scanner- the same as a regular MRI. The test takes about 30 minutes. In some cases, the CINE shows obstruction of flow through the area.
Additional studies may be recommended, such as plain x-rays of the spine to show bone structure, or a 3-dimensional CT scan of the skull.
After the tests have been completed, Dr. Oró will meet with you, and your family to review the imaging studies. The results of the test will be explained and treatment options will be discussed. If one or more of these conditions are present, and if the quality of your life is significantly affected, surgical treatment may be an option. If surgery is an option, the decision to have surgery is made by you. Dr. Oró's goal is to explain the condition, its relation (or lack of relation) to the symptoms, and the nature of the surgery, the possible benefits, and the associated risks.
If surgery is an option, you are encouraged to have all your questions answered, make your decision carefully, and take time in making that decision.
Healing after surgery is a gradual process.
You will experience some good days and some days when things seem achy or sore. This is normal. Slowly increasing activity, eating healthy, avoiding strenuous lifting, adhering to your doctor's advice/instructions and maintaining a positive attitude are the best ways to allow your body to recover. Concentrate and focus on the symptoms that have improved, instead of what symptoms remain. The goal is not to recover fully in the first two weeks but to generally progress and recover over the months following surgery.
Activities
After discharge from the hospital, you may be up and walking around the house the first week. This is a time of healing, so it is important to remember to not overdo the activity. The body needs time to rest; therefore, some patients take a nap in the afternoon. Activities such as washing dishes, fixing light meals and dusting are fine. Avoid activities such as vacuuming, lifting, carrying or anything that requires stretching of the neck muscles. Do not lift anything heavier than a gallon of milk (about 10 pounds) during the first month after surgery. If you are recovering well, the second month you can increase your lifting by five pounds per week until you reach what is comfortable for you.
Hygiene
Do your best to keep the incision clean and dry at all times. You may shower about 48 hours after surgery, but you should try to keep the incision dry and be sure not rub or soak the incision.
It is best to use very light shampoos such as baby shampoo for the first three weeks after surgery. In addition, be sure not to use any dye, hair color or other hair treatments like perm solutions until your follow up appointment.
Driving
You should avoid driving for the first few weeks after surgery - and especially if your are using pain medications. Movement of the neck may feel tight, and it is difficult to see in all directions when driving. DO NOT DRIVE if taking narcotic pain medications.
Nutrition
Good nutrition is an essential part of healing and recovery. Eating a balanced diet each day, including fruits and vegetables, dairy products and protein will aid in the healing process. A multivitamin is not necessary, unless one is taken normally. Remember to drink plenty of water.
Medications
The neck incision may be tender and stiff for several days. This is normal. Avoid rubbing or scrubbing the area until the staples are removed. If any redness, swelling, heat, or drainage is noticed around the incision, the neurosurgeon needs to know immediately.
Most people resume their normal medications after surgery. You will also receive a prescription for pain medication after discharge. The pain medication works best if taken every 6-8 hours before the pain worsens. To reduce upset stomach, take the medication with food. Pain medication should not be necessary after the second week. If a refill is needed on pain medication, do not wait until the bottle is empty - call our office at least two days before the prescription runs out. DO NOT DRIVE if you are taking narcotic pain medications.