Orthostatic Hypotension Vs Pots

[kkrylee]

My 17 yr old daughter has been diagnosed by the Tilt Table Test with Orthostatic Hypotension. During the entire test her blood pressure fluctuated dramatically reaching as low as 62/28. Her pulse barely varied between 86 to 104. This would not be POTS if understand correctly but is Orthostatic Hypotension. But when a physical BP is taken from sitting to standing, her blood pressure does not change immediately but her pulse does shoot up. This leaves me confused.

Her symptoms are numerous: dizzy, total transient vision loss, temperature changes, constant nausea 24/7, possible absence seizures, shooting burning pains in head, facial numbness/tingling, facial flushing, etc. Her main issue started, along with these symptoms, on Oct 4th at lunch. She began with a head pressure pain at the base of her skull that has never, not even for a minute, ceased. The other symptoms are every day throughout the day but not constant, except the nausea which is constant.

We tried salt tablets, high intake of fluids, regulated diet but did not see any improvement in symptoms. Does anyone have any thoughts on this constant headache pressure? From info I can find, teens have POTS but not OH. Do any of you know a teen with OH and not POTS? And, on her tilt test, there are a few times where "dop" is listed after her Heart Rate #, and some where "D" is written in instead of one of the BP #'s. What does that mean?

I appreciate any help you guys can give. We are new to this.

[pat57]

kkrylee,

I'm very tired and wish I could take more time with this reply, BUT..

I suspect the D means doppler would pick up the vitals. My TTT says "P" which means palpable.

I put in your daughters symptoms on Pub Med and got this

Spontaneous intracranial hypotension.

Mokri B.

Department of Neurology, Mayo Clinic, 200 First Street SW, Rochester, MN 55905, USA.

Spontaneous intracranial hypotension (SIH) is typically manifested by orthostatic headaches that may be associated with one or more of several other symptoms, including pain or stiffness of the neck, nausea, emesis, horizontal diplopia, dizziness, change in hearing, visual blurring or visual field cuts, photophobia, interscapular pain, and occasionally face numbness or weakness or radicular upper-limb symptoms. Cerebrospinal fluid (CSF) pressures, by definition, are quite low. SIH almost invariably results from a spontaneous CSF leak. Only very infrequently is this leak at the skull base (cribriform plate). In the overwhelming majority of patients, the leak is at the level of the spine, particularly the thoracic spine and cervicothoracic junction. Sometimes, documented leaks and typical clinical and imaging findings of SIH are associated with CSF pressures that are consistently within limits of normal. Magnetic resonance imaging of the head typically shows diffuse pachymeningeal gadolinium enhancement, often with imaging evidence of sinking of the brain, and less frequently with subdural fluid collections, engorged cerebral venous sinuses, enlarged pituitary gland, or decreased size of the ventricles. Radioisotope cisternography typically shows absence of activity over the cerebral convexities, even at 24 or 48 hours, and early appearance of activity in the kidneys and urinary bladder, and may sometimes reveal the level of the leak. Although various treatment modalities have been implemented, epidural blood patch is probably the treatment of choice in patients who have failed an initial trial of conservative management. When adequate trials of epidural blood patches fail, surgery can offer encouraging results in selected cases in which the site of the leak has been identified. Some of the spontaneous CSF leaks are related to weakness of the meningeal sac, likely in connection with a connective tissue abnormality.

Good luck!

[MightyMouse]

OH doesn't have the 30 or more beats increase in HR while upright that's seen inn POTS.

Nina

[masumeh]

both POTS and OH are probably incomplete diagnosis for your daughter's case. You really need to take her to a neurologist, and probably get an MRI to see what is going on in her neck and head to cause the pain at the base of the skull. Pressure on the nerves in the neck can cause all the symptoms you described. Maybe she is having a correctable problem. Let's hope.

Many of us POTSers have damage in the nerves of that area (autonomic nerves stemming off the cerebral spinal column), resulting from a variety of causes such as viral infection, whip lash accidents, congenital malformations, etc. It cannot be erased, can actually become worse, and only partially might heal over a four year period. But pressure on those nerves, as might be your daughter's case, can be relieved. Pressure is not permenant.

I hope you find answers and help soon!

[ajw4790]

Hi,

Welcome! I would definitly make sure that she sees a neurologist and gets a full workup as far as the pressure at the base of the skull. I suppose it could be some kind of unretractable migraine, but it is best to check something like that out. Also, don't be afraid to seek a second opinion, or travel etc. to go to a children's hospital that has more at their disposal.

As far as POTS vs. OH, it sounds more like OH, but also at that point of time it is possible to not show it and then if retested be positive for POTS, especially if noted that when go sit to stand HR shoots up.

Has she tried any meds?

Have they taken a MRI of the skull/neck?

Did she have any of the symptoms prior to this date at all?

Do they figure there was a triggering even to this (ie illness, surgery, sports injury, concussion, head trauma, growth spurt)?

I hope you find answers soon!

[anna]

Hi,

I would also take a look at the chiari institute web site, sorry I do not have this address to hand but sould be able to find via search engine.

Anna

[kkrylee]

I appreciate all of the responses: First let me state that she has had MRI's & MRA's of the head and neck, spinal tap, echocardiogram, ekg, eeg, and more blood tests than I can count (CBC, hormone, Iron testing, heavy metals, Lyme, etc.) All test have been normal except the OH.

She does have a very large globular pituitary, but 2 pediatric endos insist that it is fine for a teenager. I had a pituitary specialists at MD Anderson look at the MRI's and he said could be pituitary tumor but not definitive. Would have to wait a year or so to watch if changes. We have seen 2 neurologists, 2 ped. endos, 1 cardiologist, 1 ped. neurosurgeon, opthamologist, and internal med. No one has an answer for her symptoms. She is scheduled to see another ped neurologist this month.

I did consider Chiari but it would be Chiari 0 if she did have it. Her tonsils do not extend below foreman but her posterior fossa does look very crowded.

I looked into SIH Spontaneous intracranial hypotension after mentioned here. Her symptoms fit fairly well, except that headache does not change with positions, her MRI did not show any problem, and her spinal tap did not note low pressure. She did have a severe spinal headache after spinal tap and had to have a blood patch.

All symptoms started on Oct 4th except the transitent vision loss which has been happening 10-20 x's day for 4 years.

That's why I came back to just OH. It seems that her symptoms could all be OH but I can't find info on teenager with OH. Everything goes back to POTS. And tilt test said OH not POTS.

We are so confused. She came home last night, had to leave in middle of movie because had a severe head pain on left top and side of head along with facial burning, spinning sensation, and spots in vision. She laid on floor and just cried. Her BP was 94-57 laying down, pulse was 90 and her sugar level was 83. This headache is not her constant headache pressure. The constant pressure in rear base of head was still there as it always is.

[pat57]

The poor thing.

http://www.syncope.co.uk/orthostatic_hypotension.htm

maybe have a look at that. One site said the pain is suboccipital which I looked up and found it was at the base of the neck.

Not sure if the "coat hanger pain" is after a neck injury or not.

[flop]

The coat hanger pain is due to lack of blood to the muscles of the neck and shoulders (blood supply "stolen" by the brain as it is more important than the muscles).

[pat57]

I had heard that flop, but in that search "neck pain and Orthostatic Hypotension " the Orthostatic Hypotension seemed to be coming up as caused by neck injury.

So I'm not sure if Idiopathic Orthostatic Hypotension also gets coat hanger pain. One could assume so, but you know how that goes.

[Megan]

I am so sorry she (and you!) are going through this. As awful as it is, my best advice is to continue to see doctors until you get an answer (you seem to already be doing this.) While I don't really know the differences between OH and POTS, my experience with having POTS has shown me that it can be entirely unpredictable sometimes. Also, if all of her MRI's etc look alright, would chiropractic care be useful? (I seem to be advocating this a lot lately...but it really helps me!) I don't fully understand your daughter's condition so I don't know if it would be dangerous, but it might be something to consider.

Meg

[Sunfish]

kkrylee et al -

i just wrote more in your other post, but did want to briefly chime in re: the "coat hanger pain" subject. this type of pain can occur with any type of orthostatic intolerance, doesn't have to be related to chiari or to an injury, has no relation to age, etc. it is usually relieved, at least to some degree, by positional changes (aka worse when upright & better when reclined/ lying down). i've had this myself for years, though it's varied in severity over time. it's been something that i've discussed over the years with numerous autonomic specialists (grubb in ohio, khurana & rowe in maryland, vanderbilt, cleveland clinic, & several others by way of hearing them speak at a conference) and all of the docs have always agreed that it's an issue of insufficient blood flow &/or oxygenation when i'm pushing my body beyond it's capability to remain upright (standing &/or sitting, depending on my health situation at the time).

hope this helps,

melissa

[anna]

Hi KKrylee,

I have just read on an other site (the uk EDS site) about a girl who was having many of the problems your wrote about, the girls mum mentioned that after much investigations by all sorts of Dr's it seems she has a pheochomocytoma on a sympathetic nerve, it is this that was causing her daughters sever symptoms now she is on some meds that have worked very well for her.

I am not by any means saying this is your daughters problem but may be you could ask the mum a few more Q.'s I am sure she would be more than happy to pass on her experiances.

Anna

[kkrylee]

Thank you Anna for that information. I tried to find the post that you were referring to but I could not find it. I did research Pheochomocytoma and everything that I found states that patient will definitely have hypertension. My daughter has hypotension. She never gets hypertension. But I definitely appreciate your input. I know that her diagnosis will come from someone who can lead us in the right direction. So I do want every lead that I can follow up on.

[jbrian00]

kkrylee et al -

i just wrote more in your other post, but did want to briefly chime in re: the "coat hanger pain" subject. this type of pain can occur with any type of orthostatic intolerance, doesn't have to be related to chiari or to an injury, has no relation to age, etc. it is usually relieved, at least to some degree, by positional changes (aka worse when upright & better when reclined/ lying down). i've had this myself for years, though it's varied in severity over time. it's been something that i've discussed over the years with numerous autonomic specialists (grubb in ohio, khurana & rowe in maryland, vanderbilt, cleveland clinic, & several others by way of hearing them speak at a conference) and all of the docs have always agreed that it's an issue of insufficient blood flow &/or oxygenation when i'm pushing my body beyond it's capability to remain upright (standing &/or sitting, depending on my health situation at the time).

hope this helps,

melissa

I'm interesting in this "coat hanger" pain. Can anyone tell me what it feels like?