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Conditions related to Chiari malformation

Read on to learn about the various conditions associated with Chiari malformation and how they can affect you or your loved ones.

Basilar migraine

Also known as Bickerstaff syndrome, basilar migraine can best be described as a headache with symptoms such as:

  • Dizziness
  • Ataxia
  • Tinnitus
  • Decreased hearing
  • Nausea and vomiting
  • Dysarthria
  • Diplopia
  • Loss of balance
  • Altered consciousness
  • Syncope
  • Bilateral paresthesias or paresis
  • Loss of consciousness

Basilar migraine is most frequent in adolescent girls and young women. It 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.

Cervicogenic headache

Headaches in the back of the head — called occipital headaches — may occur from problems in the neck, particularly the upper area. Since the cause of the pain originates in the neck, it is called cervicogenic headache.

The headache usually radiates from the neck to the occipital area, and also 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 percent of the U.S. population have cervicogenic headache.

Treatments for cervicogenic headache

Depending on the cause, cervicogenic headache can be treated with the following:

  • Intra-articular (facet-joint) corticosteroid injections
  • Local anesthetic block (injection)
  • Nonsteroidal anti-inflammatory drugs (NSAIDs)
  • Physical therapy
  • Radiofrequency neurotomy
  • Surgery, including anterior and posterior techniques

Chronic fatigue syndrome (CFS)

CFS is a debilitating and complex disorder that produces intense fatigue that is both not improved by bed-rest and worsened by physical or mental activity. Persons with CFS function at a substantially lower level of overall activity.

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

Empty sella syndrome ESS

ESS originates from a spinal fluid pouch pressing down on the pituitary gland. ESS can also affect the nearby optic nerves, and is seven times more common in women than men. Seventy-five percent of those affected have increased intracranial pressure.

If you have ESS, you may also experience:

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

Treatments for ESS

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

Surgical treatment may include:

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


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.

Treatments for fibromyalgia

The goals of treatment are to reduce pain and improve daily living activities. Treatments can include:

  • Acupuncture
  • Exercises
  • Medications
  • Physical therapy
  • Relaxation techniques
  • Stress reduction techniques, such as biofeedback

Idiopathic intracranial hypertension (IIH)

IIH, also known as pseudotumor cerebri, describes increased pressure inside the brain, without obvious cause. IIH causes a variety of neurological symptoms, particularly headache and vision problems.

Treatments for IIH

For those who are obese and experiencing IIH, weight loss is the most important treatment. Just six to ten percent loss of total bodyweight can be effective in resolving the condition. If you are taking a medication that increases the chances of developing IIH, you should consult with your doctor to determine if you can come off of the suspected medication.

There are certain medications that can be used to treat IIH, including:

  • Acetazolamide
  • Furosemide
  • Steroids

If medical treatment proves ineffective, the most common surgical treatment for IIH is a cerebrospinal fluid shunt.

IIH occurs when there is a blockage of the large venous sinuses draining the brain, and we are now able to use microcatheters to treat the blockage.

Additionally, our ophthalmologists can perform optic nerve sheath fenestration to 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.

Multiple sclerosis (MS)

MS is a chronic, inflammatory disease of the nervous system that affects the lining around the nerves in the brain and spinal cord. It is believed that MS is an autoimmune disease, and a neurologist will need to evaluate you, determine the diagnosis, then recommend a course of treatment. While difficult to treat, it has become easier over time since the development of new medications.

Occipital neuralgia

Occipital neuralgia is one of the most common pains, similar to the headache seen with the Chiari malformation. With 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.

Treatments for occipital neuralgia

Initial treatment of occipital neuralgia 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 can be performed. If nerve blocks are effective, but are not lasting, other procedures can be used, including:

  • C2 ganglionectomy
  • Cryotherapy
  • Occipital nerve stimulation
  • Rhizotomy

Rebound headache

Rebound headaches — also known as analgesia rebound headache — are headaches due to chronic uses of narcotic pain medication. Long-term use of narcotics can cause headaches that 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.

Spontaneous intracranial hypotension SIH

SIH is a condition of low spinal fluid pressure that often causes headache and other neurological symptoms. Most cases are due to a hidden spinal fluid leak, although some are due to other causes. In some cases, the cause of SIH may not be found.

SIH is twice as common in women than men, with the average age of development being 40 years old. If you have connective tissue disease or Chiari malformation, you are more likely to develop SIH.

Treatments for SIH

In many people, the condition resolves on its own over time. However, sometimes treatment is necessary. Some types of noninvasive treatment include:

  • Bed rest and increased fluids
  • Carbon dioxide inhalation
  • Increased salt intake
  • Intravenous or oral caffeine and theophylline
  • Steroid therapy

When a spinal fluid leak is present, it mostly occurs in the spine, especially in the thoracic (chest) spine. Radiology tests may be needed to identify the site of the spinal fluid leak, including:

  • CT myelography
  • Radioisotope cisternography
  • Spinal MRI

Invasive treatment options include:

  • Injection of blood or fibrin sealant at the site of the leak
  • Epidural blood patch, if a spinal leak is not found
  • Surgery, but only if other therapies prove ineffective