Fibromuscular Dysplasia (of the brain) and MC

sarkin · Post by **sarkin** » Thu Sep 08, 2011 10:55 am

Re: my friend whose kids had such dramatic Enterolab results - both those kids (and not their asymptomatic brother) have hypermobility syndrome. I don't, and am not at all double-jointed. But I can bend just the first finger of my fingers. There must be a spectrum for this, too - I can't do any of the other magic you and your son can do, Z.

My friends' kids have had pain as well as extreme mobility - I hope your son never does.

Zizzle · Post by **Zizzle** » Thu Sep 08, 2011 12:53 pm

Thanks for mentioning hypermobility syndrome! I looked it up and am pleased to report my son and I probably have that, and not the more rare EDS. It seems rather common, phew. The Mayo Clinic has a good overview which put me at ease. It explains why my symptoms improved with age, and why my son seems to experience frequent "growing pains" even though he grows very, very slowly! I now have more reason to tell Dad to stop pressuring him into playing flag football (he fears it, with good reason. He's a very gentle boy). My daughter will surely excel in gymnastics and dance. I just need to explain to them why putting on a joint freakshow for their peers is not good for them.

When large groups of school children are tested, hypermobility is found in 7 to 40 percent of children. Between 8 and 11 percent of these children have hypermobility that can lead to pain after activities or at night. No one knows why some children develop discomfort, while others with equally hypermobile joints do not have pain or swelling.

There is often a family history of "loose-jointedness." There may occasionally be a patient and/or family history of congenital hip dislocations; scoliosis (curvature of the spine); elbow, kneecap or shoulder dislocations; or frequent ankle or wrist sprains.

I had all 5 markers as a kid, now I can only do the hand acrobatics:

Five specific mobility tests are used for diagnosing BHJS:

The wrist and thumb can be moved downward so the thumb touches the forearm.
The fingers (especially the 5th finger) can be extended back so they are parallel to the upper arm.
When standing, the knees are abnormally bowed backward when viewed from the side.
When fully extended, the arms bend further than normal (beyond straight).
When bending at the waist, with the knees straight, the child or young adult can put his or her palms flat on the floor.

And there is some good that may come from it:

Some studies have found that older people who were hypermobile in youth have better functional capabilities compared to those without hypermobility.

http://my.clevelandclinic.org/disorders ... drome.aspx

sarkin · Post by **sarkin** » Thu Sep 08, 2011 1:06 pm

Z, phew indeed!

I'm glad some good may come of this... I have a family history of congenital hip dislocation and scoliosis, and I can still put my palms flat on the floor. My mother could stand on steps and touch the stair below.

I hope your gentle boy finds his own favorite, less-jarring activity or sport,

S

Gloria · Post by **Gloria** » Thu Sep 08, 2011 2:06 pm

Zizzle,

What a relief to have figured out that you have a less serious, more common condition!

Sara wrote:My mother could stand on steps and touch the stair below.

Ouch! My back and legs hurt just thinking about the position. Not to mention my head, which surely would have landed on the steps further below.

Gloria

sarkin · Post by **sarkin** » Thu Sep 08, 2011 2:21 pm

LOL, Gloria - I got up and tested it, and I can do this, too - I sure can't put my palms down a stair below, but I can touch without straining.

If only there were something useful or productive I could do with this quirk...

Post by **tex** » Thu Sep 08, 2011 4:28 pm

Zizzle,

It's still EDS, it's just the most common form of it, which is benign. From the article you cited:

Another commonly used term for BHJS is the benign type of Ehlers-Danlos syndrome ("type 3").

Note the diagnostic criteria in the article at the following link:

Major diagnostic criteria should all be met to establish a diagnosis of EDS, hypermobility type:

Joint hypermobility, which is often confirmed by a score of five or more on the nine-point Beighton scale [Beighton et al 1973], although some individuals with objective joint laxity score fewer than five points (see below, The sensitivity and specificity of examination for joint hypermobility). One point is scored for each of the following:

Passive dorsiflexion of each fifth finger greater than 90°

Passive apposition of each thumb to the flexor surface of the forearm

Hyperextension of each elbow greater than 10°

Hyperextension of each knee greater than 10°

Ability to place the palms on the floor with the knees fully extended

Soft skin with normal or only slightly increased extensibility.

Soft skin is subjectively assessed, preferably in an area in which moisturizer has not been applied.

Skin hyperextensibility is assessed at a site lacking excess or loose skin and without evidence of prior trauma by gently pulling until resistance is met. An ideal location is the volar surface of the forearm, where the upper limit of normal extensibility is 1-1.5 cm. Extensor surfaces of joints have excess skin and should not be used.

Absence of fragility or other significant skin or soft tissue abnormalities, which are suggestive of other types of EDS. Such findings could include:

Spontaneous or easily induced skin cuts or tears

Spontaneous or easily induced tears or ruptures of tendons, ligaments, vessels, or other internal organs

Surgical complications, such as vessel rupture or sutures tearing through tissues and failing to hold

Spontaneous wound dehiscence

Recurrent or incisional hernias

Significant skin hyperextensibility (>1.5 cm on the volar surface of the forearm)

Thin, translucent skin

Atrophic ("cigarette paper") scars (although mildly atrophic scars are sometimes seen in EDS, hypermobility type, especially in areas subject to physical stress, such as extensor surfaces and the abdominal wall)

Molluscoid pseudotumors

Minor diagnostic criteria are supportive of but not sufficient to establish a diagnosis of EDS, hypermobility type:

Positive family history of EDS, hypermobility type (or family history of joint laxity), without significant skin or soft tissue fragility, in a pattern consistent with autosomal dominant inheritance

Recurrent joint dislocations or subluxations

Chronic joint, limb, and/or back pain

Easy bruising

Functional bowel disorders (functional gastritis, irritable bowel syndrome)

Neurally-mediated hypotension or postural orthostatic tachycardia

High, narrow palate

Dental crowding

The sensitivity and specificity of examination for joint hypermobility is dependent in part on the individual's age, gender, and medical history.

Young children (approximately age five years or younger) tend to be very flexible and are therefore difficult to assess.

Women are, on average, more flexible than men.

Older individuals tend to lose flexibility, and post-surgical or arthritic joints often have reduced range of motion. A history of former joint laxity or clinical demonstration of substantial laxity in multiple joints is sometimes accepted in lieu of a positive Beighton score in such cases, if the family history and other minor criteria are strongly suggestive.

The red emphasis is mine, of course.

http://www.ncbi.nlm.nih.gov/books/NBK1279/

Tex

Zizzle · Post by **Zizzle** » Thu Sep 08, 2011 8:50 pm

Wow, very interesting. I do beleive I probably had POTS as a teen. I attributed my near-fainting spells, hypotension and heart palpitations to MVP, but maybe it was POTS? Anyway, it seems POTS, EDS, MVP and bowel disorders all all related! Interesting indeed! I still have postural hypotension, exercise intolerance and some joint hypermobility, which is typical in a recovered/aging person.

Recovered individuals do complain of occasional, non-debilitating recurrence of symptoms associated with autonomic dysfunction including dizzy spells, lightheadedness, flushing, transient syncope, and symptoms of irritable bowel syndrome."

http://en.wikipedia.org/wiki/Postural_o ... a_syndrome

I remember looking up autonomic nervous system dysfunction as a teen, because all my weird symptoms stemmed from it. Again, I thought the MVP was to blame. Of course my parents thought I was a hypochondriac, and I didn't have any decent access to a doctor back then to talk to. So I took Vit C fpr the bruising, salt for the hypotension, frequent snacks for what seemed like low blood sugar, and I quit the track team.

Hmmm...Does autonomic dysfunction play a role in MC development or symptoms?

Post by **tex** » Thu Sep 08, 2011 9:26 pm

Probably:

We conclude that AN is common in patients with ulcerative colitis, regardless of disease activity and previous colectomy. In contrast to a predominantly sympathetic dysfunction in Crohn's disease, AN in ulcerative colitis was vagal.

"AN" stands for autonomic neuropathy

http://www.ncbi.nlm.nih.gov/pubmed/8362220

CONCLUSIONS: Clinically manifest AD is associated with lower quality of life and higher healthcare utilization in IBD patients. They may represent a cohort at risk for worse outcomes.

In this article, "AD" stands for autonomic dysfunction.

http://www.ncbi.nlm.nih.gov/pubmed/19727003

And with Crohn's disease:

Conclusion

Our data suggest that autonomic sympathetic neuropathy is an early systemic feature of CD independent of disease activity. The neuropathy was slightly more pronounced after seven years and this progressive course is consistent with the more pronounced autonomic neuropathy manifested by abnormal indexes previously demonstrated in patients with CD of longer duration [2].

http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1978494/

In spite of normal peripheral nerve function, almost half of the patients, 48% (16/33), showed signs of autonomic neuropathy (AN). The occurrence of AN was not related to duration or severity of Crohn's disease or to biochemical evidence of inflammation or malabsorption of vitamins and trace elements. We conclude that autonomic nerve dysfunction is a feature of Crohn's disease which may be relevant with regard to the frequent disturbance in bowel function in patients with this disorder.

http://www.ncbi.nlm.nih.gov/pubmed/2034989

It was certainly a problem for me, when I was reacting, and it still is - I lost the ability to feel hunger pangs, and the ability to determine when my appetite was satiated, early on, and that appears to be a permanent loss, in my case.

This also showed up in a Google search:

http://www.perskyfarms.com/phpBB2/viewtopic.php?t=3285

Tex

Sharaine · Post by **Sharaine** » Mon Sep 12, 2011 1:18 pm

Your prayers seem to have worked - thank you. I saw the neurologist on Thursday and he thinks I *probably* have a reversible condition. He's got me on medication to see if my headache resolves and stays gone. It's definitely growing less, but it comes back every day. We shall see.

Zizzle · Post by **Zizzle** » Mon Sep 12, 2011 1:21 pm

Wonderful news!! What medication does he have you on?

Wishing you continued improvement on the headaches.

:flowersmiley:

Gloria · Post by **Gloria** » Mon Sep 12, 2011 1:36 pm

Terrific news, Sharaine! I hope the medication continues to reduce the headache severity.

Gloria

Post by **tex** » Mon Sep 12, 2011 2:15 pm

Sharaine,

Finally - Some good news!

Good luck with the treatment.

Tex

sarkin · Post by **sarkin** » Mon Sep 12, 2011 3:28 pm

Sharaine,

Thanks so much for the terrific update - it sounds very hopeful indeed. I hope you continue to feel better and are soon headache-free!

Love,
Sara

Sharaine · Post by **Sharaine** » Tue Sep 13, 2011 3:22 pm

Zizzle,

I'm on 20 mg Verapamil TID. Not thrilled as it is making me constipated. I will see how it goes for another week.

Sharaine