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Chiari I signs and symptoms

Chiari symptoms are caused by disruption of CSF flow and compression of the brainstem
and spinal cord.

Chiari I symptoms in
infants / children:

Trouble feeding and swallowing

Excessive drooling

Noisy breathing (strider),
especially with crying

Apnea (stop breathing spells)

Irritability, head banging and nighttime awakening (signs of headache)

Stiff neck

Poor arm strength (trouble crawling)

Chiari I symptoms vary from person to person and are not necessarily related to the size of tonsillar herniation. Some people with large herniations have no symptoms (asymptomatic). Yet others with small herniations have severe symptoms. When symptoms are present, they are often vague or nonspecific. As a result, the diagnosis of Chiari is often delayed until more severe symptoms occur or after current symptoms persist for some time. Symptoms are caused by disruption of the CSF flow and compression of nervous tissues.

Because the brainstem is responsible for most body functions, Chiari causes all kinds of strange symptoms. People may experience symptoms that range from headache to irritable bowel. The five most common symptoms are:

1. posterior headache on exertion with neck pain (70%)
2. hoarseness or swallowing problems
3. sleep apnea
4. weakness or numbness in an extremity
5. balance problems

People with Chiari I often develop symptoms during their teen or early adult years. The disorder is also seen in young children and older adults. In some cases, a head or neck injury from a car accident or sports injury triggers the onset of symptoms. Some patients have had Chiari triggered by a spinal tap or epidural anesthesia.

Listed below are Chiari symptoms grouped by the body area affected:

Headache

• Pressure-like headaches that start at the back of the skull and may radiate behind the eyes.
• Headaches that worsen with physical strain, coughing, sneezing, or bending forward (Chiari headaches are often mistaken for migraines)

Pain / spine problems

• Neck pain, pain across shoulder blades
• Chest pain
• General body pain
• Curvature of the spine (scoliosis) may be present with syringomyelia
• Joint hypermobility (Ehler-Danlos syndrome)
• Tethered cord

Balance / ear problems

• Ringing or buzzing in the ear (tinnitus)
• Dizziness, spinning (vertigo)
• Imbalance, clumsiness
• Trouble walking (gait)
• Hearing loss

Eye problems

• Blurred or double vision
• Sensitivity to bright light
• Spots or floaters in your vision
• Jerking eye movements (nystagmus)
• Difficulty tracking or following objects with your eyes

Sleep problems

• Snoring
• Sleep apnea
• Fatigue
• Inability to fall or stay asleep (insomnia)

Face and throat problems

• Difficulty swallowing, choking and gagging
• Facial pain, numbness or tingling
• Hoarseness, change in voice
• Chronic cough

Problems in arms and legs (spinal cord signs)

• Numbness or tingling in arms / hands / legs
• Weakness in arms / hands / legs
• Poor hand coordination
• Loss of feeling in arms / hands
• General body weakness

Thinking (cognitive) problems

• Trouble speaking, word finding
• Trouble thinking
• Problems with memory and concentration
• Depression or mood changes
• Nervousness or anxiety

Other problems

• Nausea and vomiting
• Abdominal pain
• Frequent urination
• Irregular heart beat, palpitations
• Passing out episodes, syncope

Chiari is often misdiagnosed because of the variety of bony and soft tissue abnormalities that compress the spinal cord, brainstem, cranial nerves, or blood vessels, resulting in a wide array of possible symptoms. In fact, the diagnosis is often delayed until symptoms become severe or persistent. However, accurate diagnosis and an effective treatment plan are important to prevent further injury to the person’s nervous system.

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