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Beighton Scale

Published: 15 December 2018
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A scale used for measuring joint hypermobility.
Total score of 9 possible points.
Most geneticists look for a score of at least four (4) or five (5) for a diagnosis of the hypermobility commonly associated with Ehlers-Danlos Syndrome.

Can you or have you ever been able to:

  1. Hyper-extension of pinky (little finger at the metacarpophalangeal joint) to greater than or equal to 90°.
    [One (1) point for each pinky (little finger), for a maximum of two (2) points.]
  2. Hyper-flexion of wrist – ability to touch medial (ulnar) forearm with thumb.
    [One (1) point for each thumb, for a maximum of two (2) points.]
  3. Hyper-extension of elbow to greater than 10°.
    [One (1) point for each elbow, for a maximum of two (2) points.]
  4. Hyper-extension of knee to greater than 10°.
    [One (1) point for each knee, for a maximum of two (2) points.]
  5. Hyper-flexion of hips/spine – ability to touch toes and place palms flat on floor with unbent knees.
    [Maximum of one (1) point.]

Lhermitte’s Sign

Published: 15 December 2018
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An uncomfortable and often painful “electric shock” sensation that travels through your spine and out into your peripheral limbs, and is exacerbated by valsalva maneuvers (coughing, sneezing, etc). This phenomenon is commonly seen as a result of cervical spinal cord injury (and in this case should be taken very seriously), but also can be a result of certain autoimmune conditions (Multiple Sclerosis, Lupus, Bechet’s disease) or a vitamin B12 deficiency.

pseudomeningocele

Published: 15 December 2018
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An abnormal collection of cerebrospinal fluid (CSF) that forms a pocket inside the cranium, just outside of the dura. Generally occurs as a complication of duraplasty (a common part of a Chiari Decompression surgery). While a small amount of fluid getting through the dura patch is normal after surgery, the body should absorb it and it should resolve within the first month or two. If it fails to reduce in size and resolve, that is a good indication that there is a cranial dura leak exists. A pseudomeningocele are most common amongst patients with an underlying connective tissue disorder (such as Ehlers-Danlos Syndromes) as their dural tissue is more fragile than that of the general population, they are more likely to have problems with the adherence of the patch, and they are more likely to have other comorbid conditions that can further complicate the healing process (such as Intracranial Hypertension, where the untreated high pressure can actually cause a cranial leak). Since the pseudomeningocele is inside the cranium, it can cause a “mass effect” that can raise intracranial pressure.

Symptoms of Atlantoaxial Instability

Published: 29 September 2018
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HEADACHES:

  • Suboccipital Headaches

FACIAL PAIN/NERVE DEFICITS: 

NECK/BACK PROBLEMS:

  • Neck pain
  • Neck stiffness
  • Neck spasms
  • Limited neck mobility
  • Torticollis (head tilt)
  • Odontoid fractures
  • Cervical Vertigo

EQUILIBRIUM/BALANCE PROBLEMS:

  • Balance impairment
  • Unsteady gait
  • Incoordination
  • Clumsiness
  • Dizziness
  • Vertigo

CARDIO-RESPIRATORY PROBLEMS:

  • Autonomic Hyperreflexia
  • Dysphagia (trouble swallowing)
  • Hyperactive pharyngeal (gag) reflex
  • Drop attacks
  • Respiratory distress/failure

COGNITIVE PROBLEMS:

  • Fatigability

EYE/VISION PROBLEMS:

  • Blurred vision

EAR/HEARING PROBLEMS:

  • Tinnitis (ringing in the ears)
  • Fullness in the ears

SENSORY PROBLEMS (touch, taste, smell):

  • Sensory deficits

PROBLEMS WITH VOICE/SPEECH:

  • Hoarseness
  • Voice paralysis (full or partial)

PROBLEMS WITH EXTREMITIES:

  • Weakness and/or cramping in muscles of arms, shoulders, hands, or legs.
  • Tingling and/or numbness of arms, shoulders, or hands
  • Loss of sensation in feet
  • Burning pain that spreads to the arms, buttocks, or down into the legs (Sciatica)
  • Loss of fine motor skills (hand coordination)
  • Paraplegia (rare)
  • Hemiplegia (rare)
  • Quadriplegia (rare)

OTHER PHYSICAL PROBLEMS:

  • Bladder and bowel dysfunction
  • Other changes

Symptoms of Intracranial Hypotension

Published: 20 July 2018
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HEADACHES:

  • Orthostatic Headaches – Pressure headaches that are worse when upright (or after being upright for a period of time), and relieved from laying down. Headaches typically start within 15 minutes of sitting or standing up, and get a measure of relief after lying down for 30 minutes. However, when it comes to cerebrospinal fluid leaks (the most common cause of Intracranial Hypotension), the orthostatic component varies significantly on how long the leak has been present. When a leak first begins, the loss of intracranial pressure has not yet been fully established, and headaches tend to be non-positional. As the leak continues and the intracranial pressure continues to drop, the positional element usually becomes more noticeable. Leaks that have been leaking for a long period of time, tend to have a delayed orthostatic headaches (so the orthostatic feature is easy to miss) or the positional aspect may seem to disappear – headaches take hours to worsen or improve with change of position. They will often wake up in the morning with no headache and get one later in the day when they have spent hours being upright. Likewise, it takes longer to get relief from being supine.

NECK/BACK PROBLEMS:

  • Neck pain/stiffness
  • Interscapular (between the shoulder blades)

EQUILIBRIUM/BALANCE PROBLEMS:

  • Balance impairment
  • Unsteady gait
  • Incoordination
  • Clumsiness
  • Dizziness
  • Vertigo

COGNITIVE PROBLEMS:

EYE/VISION PROBLEMS:

  • Sensitivity to light
  • Double vision
  • Nystagmus

EAR/HEARING PROBLEMS:

  • Tinnitis (ringing in the ears)
  • Changes in hearing (muffled sound)
  • Sensitivity to sound(s)

SENSORY PROBLEMS (touch, taste, smell):

  • Metallic taste (leaks from nose)

PROBLEMS WITH EXTREMITIES:

  • Movement disorders, such as chorea or Parkinsonism
  • Tremors
  • Slow movement
  • Stiffness of extremities

Symptoms of Tethered Cord

Published: 16 July 2018
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BACK PAIN:

  • Lower back pain, especially when standing, walking or being active

 

PROBLEMS WITH EXTREMITIES:

  • Leg pain
  • Numbness in lower legs/feet (tingling/pins-and-needles sensation)
  • Gait Disturbance
  • History of growing pains
  • History of heel or toe walking as a child
  • High arches

 

INCONTINENCE ISSUES:

  • Urinary Dysfunction
    • Urgency
    • Frequency
    • Incontinence
    • Nocturia (waking up to urinate more than 1x per night)
    • Trouble emptying bladder
    • Hesitancy (trouble starting urinary stream)

 

  • Bowel Dysfunction
    • Frequent constipation
    • Frequent diarrhea
    • Occasional bowel incontinence

 

OTHER PHYSICAL PROBLEMS:

  • Frequent UTIs (Urinary Tract Infections) or having the sensation of a UTI
  • Decreased sensation in the pelvic area
  • Sexual Dysfunction
    • Decreased sexual interest
    • Difficulty reaching orgasm

 

Craniocervical Instability [CCI]

Published: 7 October 2017
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A structural defect and vertical hypermobility (back and forth sliding) of the craniocervical junction (interface between the occipital bone and the 1st and 2nd vertebrae) which can lead to a deformation of the brain stem, upper spinal cord and cerebellum (and associated neurological symptoms) due to compression.


Etiological Co-factors: Heritable Disorders of Connective Tissue (HDCT), such as Ehlers-Danlos Syndrome (EDS).

Orthostatic Headache

Published: 7 October 2017
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Positional headache that worsens when upright (sometimes after being upright for a prolonged amount of time) and gain relief when lying down.

Also see: Intracranial Hypotension, CSF Leaks

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