Amy's Journey with...

Hypermobile Ehlers-Danlos syndrome (hEDS) ~ Postural Orthostatic Tachycardia Syndrome (POTS) ~ Focal Impaired Awareness (Complex Partial) Seizures ~ Fibromyalgia ~ Chronic Myofascial Pain (CMP) ~ Polycystic Ovarian Syndrome (PCOS) ~ TMJ Dysfunction ~ Bipolar Disorder Type I Rapid Cycling ~ Migraines ~ Gastroesophageal Reflux Disease (GERD) ~ Obsessive Compulsive Disorder (OCD) ~ Keratosis Pilaris (KP) ~ Complex-Post-Traumatic Stress Disorder (C-PTSD) ~ Panic Disorder ~ Generalized Anxiety Disorder (GAD) ~ Social Anxiety Disorder (SAD) ~ Nonsuicidal Self-Injury (Self-Harm) ~ Piezogenic Pedal Papules ~ Hashimoto's Thyroiditis ~ Irritable Bowel Syndrome (IBS) ~ Seasonal Affective Disorder (SAD) ~ Specific Phobias ~ Chronic Headaches

Chiari & Tethered Cord

Ehlers-Danlos Syndrome & Chiari (pronounced kee-AR-ee)

Chiari often comes with EDS, so I decided to do a page on it.  I find it interesting that the most common misdiagnoses for "Chiarians" is fibromyaglia.  From what I've learned Chiari also often goes along with other problems, such as tethered cord.  Admittedly, I did not know much about Chiari until I was discussing my strange and worsening symptoms with a friend.  She gave me the name Chiari and Cervical Cranial Instability (CCI) to research.      The full title of the disease type that goes with EDS is Arnold Chiari malformation type I.  This is the adult form and the form most commonly found with EDS, though you don't have to have EDS to get Chiari.  Chiari symptoms can be completely disabling for many people, while some people do not experience any symptoms whatsoever.

So what is Chiari?  In really simple terms Chiari is a neurological condition in which the brain is literally squished out of the bottom of the skull because the skull is too small to hold all the brain. Chiari Institute physicians were the first to find the link between Chiari and Ehlers-Danlos Syndrome in a certain subset of patients.

See the brain being squished out?

This chart is from Wikipedia's Article on Chiari:

TypePresentationOther notes
IA congenital malformation. Is generally asymptomatic during childhood, but often manifests with headaches and cerebellar symptoms. Herniation of cerebellar tonsils.[5][6]The most common form.
Syndrome of occipitoatlantoaxial hypermobilityAn acquired Chiari I Malformation in patients with hereditary disorders of connective tissue.[7] Patients who exhibit extreme joint hypermobility and connective tissue weakness as a result of Ehlers-Danlos syndromeor Marfan Syndrome are susceptible to instabilities of the craniocervical junction and thus acquiring a Chiari Malformation. This type is difficult to diagnose and treat.[8]
IIUsually accompanied by a lumbar myelomeningocele[9] leading to partial or complete paralysis below the spinal defect. As opposed to the less pronounced tonsillar herniation seen with Chiari I, there is a larger cerebellar vermian displacement. Low lying torcular herophili, tectal beaking, and hydrocephalus with consequent clival hypoplasia are classic anatomic associations.[10] The position of the torcular herophili is important for distinction from Dandy-Walker syndrome in which it is classically upturned. This is important because the hypoplastic cerebellum of Dandy-Walker may be difficult to distinguish from a Chiari malformation that has herniated or is ectopic on imaging. Colpocephaly may be seen due to the associated neural tube defect.
IIICauses severe neurological defects. It is associated with an occipital encephalocele.[11]
IVCharacterized by a lack of cerebellar development.[12]

Kristen, in her blog Cure for Chiari explains Chiari like this:
"For the ones who don't understand completely, I will explain in simple terms.  Basically, Chiari Malformation is a malformation of the back of the scull (Posterior Fossa.)  It is formed too small and there isn't room for the brain to fit.  If there's no room, it's going to natural make room for itself.  So, it herniates out the back of the scull and goes down into the hole between the scull and Spinal Cord - Foramen Magnum.  This causes pressure to be put on the spinal cord, which causes symptoms that the brainstem or Cerebellum controls.
  • A daily occipital headache felt at the base of the skull that is made worse by coughing, sneezing, or straining
  • Painful tension in neck
  • Fatigue
  • Migraines
  • Dizziness
  • Visual disturbances / loss of vision / spots in vision / double vision / seeing spots or "halos" / nystagmus
  • Tingling / numbness in the extremeties
  • General imbalance / clumsiness
  • Memory loss
  • Restricted movement
  • Intolerance to bright light / difficulty adjusting to light change
  • Vertigo from position change or sudden standing
  • Difficulty walking on uneven ground / feeling ground under feet
  • Poor / degraded motor skills
  • Difficulty driving
  • Difficulty negotiating steps
  • Pressure / pain in the neck
  • Pressure / pain behind the eyes (soreness in the eyeballs)
  • Back pain
  • Neck spasms
  • Insomnia
  • Ringing in ears (like the tone heard in a hearing test)
  • Swaying
  • Pain when changing position
  • Tingling / crawling feeling on scalp
  • Intolerance to loud / confusing sounds
  • Decreased sensation to touch in extremeties
  • Decreased sensitivity to temperature
  • Pain & tension along ear / eye / jawline
  • Difficulty swallowing / lump in throat / sore throat / swollen lymph nodes
  • Drooling
  • Sleep apnea
  • Spontaneous vertigo
  • Hand tremors
  • Poor blood circulation / cold hands & feet
  • Sinus / mucous problems
  • Decreased muscle tone
  • Pressure in ears / ears feel stopped up
  • Nausea
  • Difficulty reading / focusing on text
  • Depth perception problems
  • Burning sensation in extremeties / shoulder blades
  • Menstrual problems / severe cramping during period
  • Fluid-like sound in ears (like water running)
  • Loss of sexual interest / lack of sensation in pelvic area
  • Pulling sensation while sitting / standing
  • Intense itchiness w/profuse sweating
  • Slurred speech
  • Gag reflex problems / lack of gag reflex
  • Pressure / tightness in chest
  • Loss of bladder control
  • Frequent urination
  • Dehydration / excessive thirst
  • Electric like burning sensations
  • Unequal pupil size
  • Loss of taste
  • Popping / cracking sounds in neck or upper back when stretching
  • Loss of smell / problems with sense of smell
  • Dry skin and lips
  • Sudden / abrupt changes in blood pressure due to awkward position of head
  • Hiccups associated with drinking carbonated beverages
  • Skin problems
  • Nystagmus (irregular eye movements)
  • Facial pain
  • Impaired coordination
  • Dysautonomia
  • tachycardia (rapid heart) 
  • syncope (fainting) 
  • polydipsia (extreme thirst) 
  • chronic fatigue 
  • Loss of pain and temperature sensation of the upper torso and arms (as a result of a syrinx)
  • Loss of muscle strength in the hands and arms (as a result of a syrinx)
  • Drop attacks – collapsing to the ground due to muscle weakness
  • Spasticity
  • Double or blurred vision
  • Hypersensitivity to bright lights
  • Numbness and tingling of the hands and feet


Ehlers-Danlos Syndrome and Tethered Spinal Cord Syndrome (or occult spinal dysraphism sequence)

Tethered cord often comes with Chiari, so I thought it an appropriate place to put it.  To quote Kristen once again, in her blog Cure for Chiari:

"There has been studies done that showed the link between: Chiari and Ehlers- Danlos Syndrome and a published paper was put out in 2007.  It is said that patients with Ehlers-Danlos Syndrome are about 7 times more likely to develop a Tethered Cord.  In the case of Ehlers-Danlos patients, the Filum Terminale is very thin and therefore can stretch.  This causes the spinal cord to tether, the pulling effect from the spinal cord causes the brain stem to heriate. There have even been some cases with patients with all three conditions that there is a 20% chance, their spinal cord re-tether after surgery.  Chiari can also be caused in EDS patients because the tissues in the neck move around a lot, causing the tonsils to herniate.  In some cases when the cord is still Tethered it can 'mask underlying Craniocervical Instability.'  This is due to the weak tissues in your neck, caused from the Ehlers Danlos Syndrome." 

Some of the symptoms of Tethered Cord listed on Chiari Connection International are:
  • Urinary urgency 
  • Frequency of urination 
  • Urinary incontinence 
  • Urinary retention 
  • Frequency of urination at night 
  • Difficulty starting urinary stream 
  • Constipation 
  • Diarrhea 
  • Occasional incontinence for stools 
  • Decrease interest in sexual relations 
  • Difficulty reaching an orgasm 
  • Decreased sensation in your pelvic area 
  • Low back pain 
  • Leg pain 
  • Numbness under the soles of your feet 
  • Keeping your knees bent at night 
  • Low back pain, leg pain, or urinary symptoms while walking up stairs 
  • History of severe growing pains during childhood and adolescence
Tethered Cord is neurological disorder caused by tissue attachments that limit the movement of the spinal cord within the spinal column.  These attachments cause abnormal stretching of the spinal column.  Tethered Cord is a progressive disease and may not be diagnosed until early adulthood.  Sometimes the symptom that causes someone to finally get diagnosed is loss of bladder and bowel control.  The degree of strain on the spinal column over time has a lot to do with the delayed presentation of symptoms.  In cases of Tethered Cord not related to EDS, a lot of Tethered Cord patients were born with Spinal Bifida.  By the way, the best way to prevent Spinal Bifida is by taking extra Folic Acid during your reproductive years and during pregnancy.  

Tethered Cord


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