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Conditions Related to Chiari

Related Conditions to Chiari 1 Malformation

Basilar Migraine

Also Known As (AKA): Bickerstaff syndrome

Basilar migraine is a “headache with dizziness, ataxia, tinnitus, decreased hearing, nausea and vomiting, dysarthria, diplopia, loss of balance, bilateral paresthesias or paresis, altered consciousness, syncope, and sometimes loss of consciousness.” It is most frequent in adolescent girls and young women. The headache is thought to occur due to temporary brainstem and cerebellar decreased blood flow (ischemia) from constriction of brain blood vessels (vasoconstriction) in the posterior fossa area.

It is not known how many persons with basilar migraine also have the Chiari malformation.

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Cervicogenic Headache

Occipital headaches (headaches in the back of the head) may occur from problems in the neck, especially the upper part of the neck. Although the pain originates in the neck, it can radiate to the head and cause "cervicogenic" headache.

The headache usually radiates from the neck to the occipital area, to the temples, or around the eyes. It can be on one side of the head (unilateral) or both sides (bilateral). The pain is often dull and is worse with neck movement and poor posture. It is estimated that 2.5% of the U.S. population have cervicogenic headache.

The pain can be due to problems in the cervical discs, facet joints, ligaments, or from muscular spasm. It commonly originates from the C2-3, C3-4 joints or discs, but it can also occur from the upper two joints (atlantooccipital and atlantoaxial joints) or from below C3-4. In studies using discography (injecting contrast material in the discs), injections into the C4-5 and C5-6 disc resulted in pain radiating to occipital region in some persons.

Degenerative changes: The most common cause of cervicogenic headache is degenerative changes in the spine. These are natural aging changes that cause arthritis, bony spur formation, nerve compression, and loosening of the ligaments.

Reversal of cervical curvature or kyphosis: We normally have a C-shaped curve to our neck that curves slightly backwards. This normal position keeps the head in balance and reduces neck strain. As our discs dry out, they lose some of their height and our neck straightens, and in some cases, curves forward instead of backwards. This causes strain on the muscles at the back of the neck and results in occipital headache.

“Whiplash”: Headaches following whiplash are very common and have been reported in 80% of persons at 2 months following whiplash. Even 2 years after a whiplash injury, 20-25% will still have headaches.

Nerve compression: A common cause of nerve compression is degenerative discs as discussed above. However, in a few cases, nerve compression may be due to other causes such as tumors.


  • Neurological examination
  • Head pain or tenderness with digital compression pressure at base of skull or upper cervical facets
  • Axial compression on neck
  • Spurling’s sign
  • L’Hermitte’s sign

Radiology studies may be needed to evaluate for cervicogenic headache including:

  • Cervical spine films to asses the overall degree of spinal osteoarthritis and the spinal curvature.
  • Flexion/extension cervical spine films to determine if there is instability.
  • CT scan to evaluate the bony portions of the spine in greater detail.
  • MRI to evaluate the nerves, spinal cord, and other soft tissues.
  • Discography (injection of dye into the disc) to look at the degree in internal degeneration of the disc may be needed in a few cases.


  • NSAIDs
  • Physical therapy including exercises to strengthen the cervical extensor & flexor muscles, postural adjustments, traction, and work and activity modifications
  • Local anesthetic block (injection) 
  • Intra-articular (facet-joint) corticosteroid injections
  • Radiofrequency neurotomy
  • Surgery: anterior or posterior (from the front or from behind the neck) 

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Chronic Fatigue Syndrome

The chronic fatigue syndrome (CFS) is defined as a “debilitating and complex disorder characterized by profound fatigue that is not improved by bed rest and that may be worsened by physical or mental activity.” Persons with CFS function at a substantially lower level of activity.

The cause of CFS is unknown though suspicions include dysfunction of the immune, endocrine, and the nervous system. No specific diagnostic tests are available to prove the presence of the disorder.


  • Malaise after exertion
  • Sleep is not refreshing
  • Difficulties with memory and concentration
  • Muscle pain
  • Pain in multiple joints
  • Headaches of a new type, pattern or severity
  • Sore throat
  • Tender neck or armpit lymph nodes

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Empty Sella Syndrome

The empty sella syndrome (ESS) is due to a spinal fluid pouch pressing down on the pituitary gland. Spinal fluid is held inside the arachnoid membrane. In some people, a pouch of arachnoid develops and can press on the pituitary gland sitting in a small bony recess called the sella turcica just below the brain. (On early studies, the sella, when filled with fluid, looked empty and thus the name empty sella syndrome. However, today’s CT and MRI scans show the pituitary gland is still present but is pressed flat at the bottom of the sella.) ESS can also affect the nearby optic nerves. It is 7 times more common in women than men and 75% of those affected have increased intracranial pressure.

Signs & Symptoms
Headache occurs in 60-80% of persons with ESS. The headache is often on one side of the head and more often in the frontal area. The intensity of the headache is usually moderate. Some people have a decrease in their field of vision and some may have problems with hormonal function including:

  • Increased secretion of prolactin (hyperprolactinemia) (11%)
  • Growth hormone deficiency (6%)
  • Dysfunction of other hormones in (1-2%)
  • Patients with severe ESS may develop spinal fluid leakage from nose noticed as a clear watery fluid drainage.

Associated Conditions
Patients with an empty sella may also have:

  • Hydrocephalus
  • Brain tumors
  • Chiari malformation
  • Idiopathic intracranial hypertension (pseudotumor)
  • Obesity

An MRI of the brain is the test of choice to determine if ESS is present. The radiologist evaluates the distance from the diaphragm above the pituitary to the gland itself to determine if the pituitary is being compressed. ESS is present if the distance is 5 mm or more.

On a frontal (coronal) MRI view, the radiologist may find an anchor sign, another indication of ESS.

Medical treatment of ESS includes analgesics, amitriptyline if there is associated depression, and dopaminergic agonists.

Surgical treatment may include:

  • Removal of brain tumor, if present
  • Decompression of a Chiari malformation, if present
  • Shunt if spinal fluid pressure is high (pseudotumor)
  • Repair of CSF leak from nose, if present
  • Transphenoidal elevation of sellar content (chiasmapexy) if vision worsens

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Fibromyalgia is a “chronic pain illness characterized by widespread musculoskeletal aches, pain, and stiffness, soft tissue tenderness, general fatigue, and sleep disturbances.” Pain is most common in the neck, back, shoulders, pelvic girdle, and hands. Symptoms vary in intensity and wax and wane over time.

Associated Symptoms
Those affected may have a number of associated symptoms including “irritable bowel and bladder, headaches and migraines, restless legs syndrome (periodic limb movement disorder), impaired memory and concentration, skin sensitivities and rashes, dry eyes and mouth, anxiety, depression, ringing in the ears, dizziness, vision problems, Raynaud's Syndrome, neurological symptoms, and impaired coordination.”

While no laboratory test is available to determine if fibromyalgia is present, the American College of Rheumatology (ACR) has developed criteria for the diagnosis. Symptoms are typically managed by rheumatologists or neurologist.

Obesity has been shown to increase the symptoms of Fibromyalgia. Weight reduction is an important intervention in the management of Fibromyalgia.

The goals of treatment are to reduce pain and improve activities of daily living. Treatments can include medications, physical therapy, exercises, acupuncture, relaxation techniques and stress reduction techniques (such as biofeedback).

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Idiopathic Intracranial Hypertension (IIH)

AKA: Pseudotumor cerebri, benign intracranial hypertension

Definition: increased intracranial pressure of unknown cause that results in a variety of neurological symptoms, especially headache and visual problems

Symptoms of IIH (Pseudotumor)
Headache: 99% of patients with IIH seen by neurologists complain of headache. The headache is most often diffuse, worse in the morning, and worse with Valsalva maneuvers such as coughing, sneezing or straining.

Visual Symptoms: Transient visual difficulties are frequent and lasts 1-5 seconds. The episodes are described as a graying out of vision and can occur with standing up or bending over. Double vision when looking side to side is much more frequent than double vision when looking up or down.

Other Symptoms: Patients with IIH may also have dizziness, nausea, vomiting, and ringing in the ears (tinnitus).

Suspected Causes
The cause of IIH (pseudotumor) is unknown. Among the possibilities are decreased absorption of cerebrospinal fluid (CSF), thus the brain contains more spinal fluid. Another possibility is abnormal cerebral circulation resulting in increased brain water. The increased fluid within the brain tissue causes the pressure within the cranium (intracranial pressure) to increase. 
The increased pressure affects structures within cranium, including the optic nerves and cause the complaints of vision reported by many.

Pseudotumor is more common in obesity, especially in overweight women. As the abdomen enlarges, the cardiac filling pressures increase. The elevated pressure in the heart decreases venous blood return from brain and causes an in increase in intracranial venous pressure.

Pseudotumor can affect persons of any age but is most common in the third decade of life. For unknown reasons children with pseudotumor are usually not obese.

Neurological Findings
On neurological examination, the doctor may find:

  • Swelling of the optic nerves (papilledema; although rarely, the optic nerves normal
  • Decreased vision; this is evaluated by vision test and test of visual fields
  • Double vision (diplopia)
  • Weakness of nerves that move the eyes

Risk Factors for Pseudotumor
Pseudotumor has been found to be more common when these following factors are present:

  • Medications: antibiotics, steroids, lithium, oral contraceptives, phenytoin, vitamin A (>100,000 U/day)
  • Diseases: anemia, chronic respiratory insufficiency, hypertension, multiple sclerosis, renal disease, sarcoidosis, Lupus
  • Disorders of venous drainage: tumors affecting venous sinuses, sinus thrombosis, radical neck dissection surgery

Relation to Chiari I Malformation
MRI scans in 68 patients with IIH showed that 10% had Chiari I malformation (5 mm or more), 13% had tonsillar herniation of 2-4 mm (tonsillar ectopia). Spinal taps were performed to measure the intracranial pressure in 36 patients who had failed to improve following decompression surgery for the Chiari malformation. Of these, 42% had were found to have both Chiari and psuedotumor. Thus, pseudotumor is a cause of failure following posterior fossa decompression for Chiari.

The evaluation of a patient suspected of having IIH (pseudotumor) may include:

  • Visual exam including evaluation of the optic nerves and visual fields
  • MRI & MRV (venography) of the brain
  • Lumbar puncture (spinal tap) to measure the intracranial pressure. (This can help relieve the symptoms as well.)

Medical Treatment
Weight loss is the most important treatment in persons that are obese. Just a 6% loss of weight can be effective in resolving the condition. Patients on medications that increase the chances of developing pseudotumor should consult with their doctor to determine if they can come off of the suspected medication. If any of the diseases noted above are present, they should be treated as best as possible.

Medications that are used in the treatment of pseudotumor include:

  • acetazolamide (Diamox)
  • furosemide (Lasix)
  • course of steroids

Surgical Treatment
The most common surgical treatment for pseudotumor is a cerebrospinal fluid shunt. Usually this is a shunt running from the spinal canal into the abdominal cavity and is called a lumbo-periotoneal shunt. Some patients may need a shunt from the cerebral ventricles to the peritoneum, a ventriculo-peritoneal shunt.

Optic nerve sheath fenestration is a surgical procedure performed by ophthalmologists that can reduce the pressure around the optic nerve. However, this treatment is usually reserved for severe cases where the risk to vision is high and a shunt is not effective.

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Multiple Sclerosis

Multiple sclerosis is a chronic, inflammatory disease of the nervous system that affects the lining around the nerves in the brain and spinal cord. The lining is made of myelin and is called the myelin sheath. The disease can cause widespread neurological symptoms including changes in sensation, vision, muscle weakness, depression, difficulties coordination and balance, speech, severe fatigue, cognitive impairment, problems with overheating and pain.

It is believed that MS is an autoimmune disease. A neurologist makes the diagnosis and recommends treatment. The condition has become more treatable since the development of new medications.

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Occipital Neuralgia

One of the most common pains, similar to the headache seen in the Chiari malformation, is occipital neuralgia. The occipital nerves originate from cervical nerves in the upper neck and run in the scalp in the back of the head. The nerves can become compressed or injured and cause a number of symptoms.

Persons with occipital neuralgia often note a prolonged ache or feeling of tightness in the back of the head. The pain is often a throbbing or electric-shock-like pain that starts in the upper neck and back of head and radiates behind the ears, temples, forehead and behind the eyes. The shape of the pain radiation has been described as a ram’s horn. Occipital neuralgia is usually present on one side (unilateral), but can be on both sides at the back of the head (bilateral). The scalp may be tender to the touch. The eyes may be sensitive to light.

Occipital neuralgia can be caused by a number of factors including:

  • Blunt injury to the back of head or neck
  • Pinching of the nerves by tight neck muscles
  • Osteoarthritis of the neck pressing on the cervical 2 spinal nerve
  • Tumors or other lesions in the neck
  • Localized inflammation or infection such as with gout, diabetes, blood vessel inflammation (vasculitis)
  • Frequent lengthy periods of keeping the head in a downward and forward position

In many cases, the cause is not found.

Initial treatment of ON usually includes heat, rest, anti-inflammatory mediations, and muscle relaxants. If these treatments do not help, medications for nerve pain (such as Gabapentin) may be recommended.

If medications do not work or are not tolerated, injections in the region of the nerve, called occipital nerve blocks, can be performed. If nerve blocks are effective but are not lasting, other procedures can be used. Each of these treatments has advantages and disadvantages that should be discussed with the pain specialist:

  • Cryotherapy - freeze nerve
  • Rhizotomy – cutting of the occipital nerve
  • C2 ganglionectomy
  • Occipital nerve stimulation

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Rebound Headache

AKA: Analgesia Rebound Headache

Long-term use of narcotics can cause headaches to increase in frequency and severity. The chronic use of narcotic pain medications has been shown to make the brain and spinal cord more sensitive to pain. Thus, as the pain medication is increased, the pain increases further. This condition is difficult to manage and requires a pain specialist familiar with management of narcotic withdrawal.

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Spontaneous Intracranial Hypotension

Spontaneous intracranial hypotension (SIH) is a condition of low spinal fluid pressure. Most are due to a hidden spinal fluid leak, although some are due to other causes (noted below). In some cases, the cause may not be found.

Headache: Persons with SIH usually have headaches that occur or worsen when upright and improve when lying down. The headache can be diffuse or localized to the frontal, temporal, occipital or suboccipital regions. The pain is throbbing or non-throbbing, usually occurs on both sides of the head, but can rarely  occur on only one side.  It is worse with Valsalva maneuvers such as laughing, coughing, and sneezing.

Some persons complain of chronic headaches both when upright and lying down. A few cases without headache have been described, the condition being diagnosed by typical findings on MRI and by measurement of the spinal fluid pressure. Patients with SIH may report a variety of other neurological symptoms including:

  • nausea, vomiting, anorexia
  • neck pain, dizziness, problems with balance
  • double vision
  • photophobia (sensitivity to light)
  • phonophobia (sensitivity to noise)
  • facial numbness or weakness
  • hearing changes (echoing, being underwater),
  • tinnitus
  • taste changes
  • malaise, mental confusion
  • hyperprolactinemia, galactorrhea
  • radicular symptoms involving the upper limb
  • stupor, coma

SIH is twice as common in women than men. The average age of development is 40 yrs of age. Persons with connective tissue diseases or Chiari malformation are more likely to develop SIH.


  • Spinal fluid leak due to minor trauma
  • Preexisting weakness of spinal dura
  • Unknown
  • Medical causes: dehydration, diabetic coma, and uremia.

It is important to let your provider know if there is a history of trauma, connective tissue disease, Chiari malformation, a spinal tap, spinal anesthesia, or an epidural block (spinal injection).

When SIH is suspected, a lumbar puncture (spinal tap) can be performed.  In SIH, the spinal fluid pressure will be low, often less than 60 mm water.

An MRI of the brain with and without contrast is important. Findings include:

  • Dura membrane is thicker than normal
  • Engorgement of venous sinuses
  • Subdural fluid collections
  • Hyperemia of pituitary gland
  • Sagging of the brain

Medical Treatment
In many people, the condition resolves on its own over time. However, others need treatment which may include:

  • Bed rest and increased fluids
  • Intravenous or oral caffeine and theophylline are dramatically effective in 75% - 85% with spinal tap headache
  • Increased salt intake
  • Carbon dioxide inhalation
  • Steroid therapy has shown variable results

Invasive Treatment
When a spinal fluid leak is present, an “overwhelming majority” are in the spine, especially in the thoracic (chest) spine. Radiology tests may be needed to identify the site of the spinal fluid leak including:

  • Spinal MRI
  • CT myelography
  • Radioisotope cisternography 

Treatment options include:

  • Injection of blood or fibrin sealant at the site of the leak if one can be identified. If the site of the leak is not found, an epidural blood patch in the lumbar spine may be effective.
  • Surgery is used only if medical therapy fails and the site of leak has been identified.

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