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Conditions Associated with Chiari I Malformation

What conditions may be associated with the Chiari I malformation or cause similar symptoms?


Basilar Migraine

What is 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 also known Bickerstaff syndrome.

Basilar migraine 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

What is Cervicogenic Headache?

Headaches in the back of the head, called occipital headaches, may occur from problems in the neck, especially from the upper part of the neck. Since the cause of the pain originates in the neck, is 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% 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 of the neck, the atlantooccipital and atlantoaxial joints, or from below C3-4. In some people, even problems the C4-5 and C5-6 disc can cause cervicogenic headache.  

What causes Cervicogenic Headache?

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 we get older, our discs dry out and lose some of their height, and our neck curve tends to become straighter. In some people, the neck begins to curve forward. This forward-curvature causes strain on the muscles at the back of the neck and can result in neck pain and occipital headache.

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

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

What radiology studies are helpful in finding the cause of Cervicogenic Headache?

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.

What are the treatments of Cervicogenic Headache?

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

  • 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

What is 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.

Symptoms

  • 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

What is 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.

What are the signs and symptoms of Empty Sella Syndrome?

Headache in is common and occurs in 60-80% of persons with the empty sella syndrome. 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.

Patients with an empty sella may also have:

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

How is the diagnosis of Empty Sella Syndrome made?

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.

What is the Treatment?

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

What is Fibromyalgia?

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.

What are the symptoms of Fibromyalgia?

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.”

How is the diagnosis of Fibromyalgia made?

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 (Pseudotumor Cerebri)

What is Idiopathic Intracranial Hypertension?

Increased Intracranial Hypertension (IIH), also known as pseudotumor cerebri, is increased pressure inside the brain without obvious cause on initial evaluation. IIH causes in a variety of neurological symptoms, especially headache and visual problems.

What are the symptoms of Idiopathic Intracranial Hypertension (IIH)?

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).

What are the causes of Idiopathic Intracranial Hypertension (IIH)?

The cause of IIH (pseudotumor) is often initially unknown or uncertain. Among the possibilities are decreased absorption of cerebrospinal fluid (CSF) which results in extra spinal fluid inside the brain. Another possibility is abnormal circulation of venous blood, the blood in the veins of the brain. The increased fluid, whether from fluid between the brain cells or in the veins, causes increased pressure within the cranium (intracranial pressure).

The increased pressure affects structures within cranium. The optic nerves are especially sensitive to the increased pressure and thus many people with IIH complain of visual problems such as a greying of vision.  

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.

What are the neurological findings in Idiopathic Intracranial Hypertension (IIH)?

On neurological examination, the clinician 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

What are risk factors for Idiopathic Intracranial Hypertension (IIH)?

Idiopathic intracranial hypertension 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

What is the relation to Idiopathic Intracranial Hypertension (IIH) to the Chiari I Malformation?

MRI scans in 68 patients with idiopathic intracranial hypertension (IIH) showed that 10% had Chiari I malformation, defined in the study as a herniation of the cerebellar tonsils of 5 mm or more. Of the 68 patients with IIH, 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 surgery for the Chiari malformation. Of these, 42% had were found to have both Chiari and IIH. It is important to note that IIH is a cause of failure following posterior fossa decompression for Chiari.

What does the evaluation for Idiopathic Intracranial Hypertension (IIH) consist of?

The evaluation of a patient suspected of having IIH may include:

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

What is the treatment of Idiopathic Intracranial Hypertension (IIH)?

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 IIH 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 IIH include:

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

Surgical Treatment: When medical treatment for IIH fails, the most common surgical treatment for IIH 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. Lumbar-peritoneal shunts can have many problems such as disconnection, blockage, and migration of the catheter from the abdominal cavity to the fatty tissue of the belly wall.

In persons with signs of blockage of the large venous sinuses draining the brain on the MRV, new treatments using microcatheters to treat the blockage are now available in some centers.

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

What is Multiple Sclerosis (MS)?

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

What is 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.

What are the symptoms Occipital Neuralgia?

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.

What are the causes Occipital Neuralgia?

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.

What are the treatments for Occipital Neuralgia?

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

What is 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 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

What is Spontaneous Intracranial Hypotension (SIH)?

Spontaneous intracranial hypotension (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 (noted below). In some cases, the cause of SIH may not be found.

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.

What are the symptoms of Spontaneous Intracranial Hypotension (SIH)?

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.

In addition to headache, 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

What are the causes of causes of Spontaneous Intracranial Hypotension (SIH)?

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

Evaluation

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

What are the medical treatments of Spontaneous Intracranial Hypotension (SIH)?

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

What are the invasive treatments of Spontaneous Intracranial Hypotension (SIH)?

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 

Invasive 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.

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