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Interesting thoughts Julie. I have had anaphylaxis before and in general have elevated mast cells ... or at least that is what a bladder biopsy showed. When Ava has these issues, her bp is usually fine, though once at pedis it was quite elevated. This was during an ANS event, half her body was purple and cold. Though she does have quirky rashes every now and again and a somewhat exaggerated reaction to insect bites, se never has had an anaphylactic reaction. Her o2 falling and hr elevating is almost always during sleep. I am very interesting in learning more about te metallic smell & histamine levels. Thanks

Hi there. My daughter is on mod keto diet for her epilepsy called LGIT. Typically it is not required to avoid art sweeteners, but we do as I think they can be a sz trigger and in general think they are unhealthy. Anyway, we use Toms of Maine Tooth Past and I NEED to chew gum. The gum I chew is sweetened w/ xyletol. A low GI sweetner. I forget the name of the gum. But you can find many products sweetened w/ this at Whole Foods, online etc. Here is a link on xylitol: Xylitol

Hi Pat, an autonomic seizure is typically a partial seizure and most of the time NON-convulsive. Refer to Panayiotopoulos Syndrome. Your seizure sounds like it is a generailzed convulsive tonic clonic which most often cause autnomic symptoms. It sounds to me though your sz is related to your dys and why you may be getting such extreme symptoms (the brady is not typical BUT STILL is my worse fear) The main reason for me seeing the dys neuro is for this very reason. If Ava does have the dys I have AND she has true autnomic seizures ... I worry she is at greater risk for a dangerous arrythmia or bradycardia to asytole. My daughter is hooked up to a pulse oximeter every night and anytime she has seizures ... so I can measure her o2 & heart rate. It will alarm if HR gets too low or too high or if her o2 drops too much. So far her heart rate has never dropped. Thankfully. I did not get to talk to Dr Novak in detail, but he did say dys can trigger seizures, both true neuro & the cardiogenic. I am still trying to figure out the cardiogenic sz. and trying to figure out how and why dys triggers true seizures and how to control the dys to avoid these sz. Also trying to figure out what is true neuro seizure and what is not. This is very important as the treatment for clusters or prolonged seizures are benzodiazepines. Benzos increase the risk of resp distress. I would think w/ dys there may also be a risk of resp distress. Anyone know if it is??? Thanks for your input.

Have you visited the chiari institute site? They have info re both chiari & EDS. They also have the article that was published in 07 by the institute, Dr Francamono & Dr Nazli (both leading authorities on EDS). It described in the article that chiari in EDSers does not always show up on a regular MRI. Here is a link to the article: Chiari & Connective Tissue Disorders and here is an article to help understand the original article Understanding the abstract I think MRI should be done while EDS pt is upright.

I don't know if e normally does, but said he'd treat Ava. He has said he has treated children in the past. In fact he talked about treating children w/ dysautonomia, epilepsy & disability. Can you tell me more about the sweat test?

risk I wouldn't have the surgery either, I am just curious. In general EDSers are at greater risks w/ any surgery, but I have also been told the surgery does not always help EDS folks. It makes sense to me that it wouldn't. When I do hear of other folks having it ... I typically tell them I'd only go to see the folks w/ the Chiari Institute. I am so sorry to hear Sara has so many issues.

Have you done any other testing w/ Novak yet? I think he wants to put an EEG on Ava and try and induce an episode ... but I don't see how he'd be able to. He also wants to try and induce sweating w/ her as one of her issues is extreme heat intolerance and am pretty sure she does not sweat in her face. Unfortunately he said it is difficult to induce sweating in a small child but is looking for some of the older methods ... meds I have never heard of.

oops, I see Sara has many other health issues. For the chiari, did treatment help w/ any of her symptoms esp seizures? I also question if it may be an issue w/ Ava. My brother has chiari & epilepsy. NIH believes I have it ... I also have small seizures every now and again (though I consider them to not have all that much significance).

I was first Dxed w/ IC when I was 21. The urologist said it'd get worse as I got older. I have learned to control it w/o meds by avoiding known triggers ... and I have many. The worse is sugar, but sugar tends to cause inflammation in other areas too. The test I had for te IC was a cyctoscopy w/ biopsy & hydrodistension. My results also showed obvious IC (the bloodyness petechia & chronic inflammation) The hydrodistension itself was the most effective treatment I had ever had for it. Has Sara tried this as a form of treatment? Anyway, I found it interesting that this new aut neuro says there may not be such a thing as IC and that it may just be part of something else. Does Sara have any other health issues?

Ok, guess I should have gone back to their site and read their info a bit closer. Here is the reason they do the skin biopsy:

Skin of the abdomen. They look for inflammation I think. I think it is standard in their lab (uMass). I am wondering if it has to do w/ the Mast cell issues some here seem to have.

No and I had to look that one up. It states it is a sz in response to fear, pain or other external stimuli. It also states it causes the hr to drop to the point of bradycardia. Ava is opposite. She is always quite tachycardic during these episodes. Her seizures are best described as the same sz that occur w/ Panayiptopoulos syndrome (autonomic seizures) Here is a link: Panayiotopoulos Syndrome If you read the article, you can see how it'd be easy to confuse or be able to differentiate between these true neuro seizures & autonomic dysfunction symptoms. She has had all of the symptoms w/ PS, but they have changed somewhat to more vague but intense symptoms, becoming completely paralyzed w/ speech arrest, along w/ the usual aut symptoms (tachy, o2 drop, flushing and/or pallor and temp changes) She did have a prolonged episode last year where she went into the same paralytic state, but it was very gradual and began w/ generalize hyertonia (or tonic episode). Even though it was generalized or all of her, it did not look like and was not a typical generalized tonic seizure as she remained somewhat alert and responsive. Also, all of her curled inward, whereas the tonic sz I ave seen in the past, the person stiffens up and arches back. In Ava's odd tonic episode, she looked more like a person w/ quadraplegia and hypertonia, but it was only temporary of course.

Have you been tested for chiari?

I have had some testing done in the past, but never a full work up. NIH thought both me & my daughter has dys. My daughter & I aut function testing w/ Dr Peter Novak in March. I am familiar w/ the ttt and some of the other tests, but was surprised to here biopsy was part of it. Is the biopsy to look for the mast cell issue I keep reading about here? Years ago I had a bladder biopsy. It showed chronic inflammation & increased mast cells. Is this relevant to the dysautonomia? I do have a dx of interstitial cystitis, but when I mentioned it to Dr Novak, he seemed to indicate there may not be such a condition. The inflammation & so forth is very real of course ... but he seemed to imply it was something else. I have always had all sorts of inflammation, many in my family do. I have always thought all of my health issues are related ... it'd be great to actually find out this is so ... and why it is so.

I primarily get the morning anxiety if I have taken a dopamine antagonist drug for two consecutive days or more. These drugs include many of the OTC sleep aids (such as tylenol pm), antiemetics (compazine, reglan, phenergan, dramaine) and antihistamines (benedryl and the older ones). These drugs will trigger and exacerbate any of my dys symptoms.

My O2 also appears to drop when I'm standing. I have not had it tested, but during my last tilt table test I needed supplemental oxygen. Wondering if there is a link there. Sara Sara, did they ever keep an EEG on you while they did the ttt? I do believe Ava's are at least part of these episodes are true neuro seizures, but wonder if part is a cardiogenic type seizure. The reason I believe it is true neuro sz, is that they are triggered by sleep aka sleep induced. Does anyone have any dys symptoms that are sleep induced and NOT seizures? I know neuro seizures are commonly sleep induced. PS: Sara, I think I came across one of your posts asking about a good EDS doc. Have you considered seeing Dr Nazli w/ the NIH? She is awesome. She also understands seizures and dys. Dr Francamono is also very good I understand.

I have thought about some of the GABA supps. The only med my daughter takes now are benzodiazipines as needed. Either Ativan or Diastat. Benzos help to stop seizures (and prevent) as they help w/ GABA. Would you happen to have any articles on the GABA supp you are using? No one can say for sure what the cause of the stroke was, but everyone assumes it had to do w/ my difficult preg. I ad hyperemesis te entire time, gestational hypertension, a partial abruption @ 28 weeks w/ pre-term labor. I was induced at 35.5 wks w/ pitocin. I was on the "pit" for two and a half days with my daughter going into fetal distress when contractions reached a certain point. We ended up needing a c/s. The cord and placenta were in pretty rough shape. She has had some pretty extensive metabolic testing ... but I still suspect something metabolic w/ her. At a week old she was acidotic (low bicarbs), she also had very salty feet as a baby (neg CF test), she has a metallic & burnt smell to her ... sometimes this occurs w/ her seizures (these are REAL smells, not auras). Her saliva would also burn my skin as well as trigger a raynauds response in me while breastfeeding (observed by my PCP) Because my daughter is on a mod keto diet, she has metabolic blood work done every three months. The labs are reviewed by the head ped metabolic specialist at MGH. I have met w/ this doc once before during a hospital admit. She said nothing jumped out at her BUT said there still could be something. One interesting abnormal result was carnitine. Free & total at one point were. Sometimes this happens w/ the diets, but what is interesting is they went from low at the beginning of the diet to elevated a after she had been on it for a while. This is NOT typical from what I understand. I have also considered mito issues as she has had a reaction to the flu vax. Seizure along w/ some regression. Anyway, I am forever looking for more answers. The stroke does not explain everything. She does have some autistic like "quirks" but she is very high functioning. Those quirks could be because her damage is on the rt side of her brain. She also likely has ESES (electrical status). Her EEG pattern shows almost continuous spiking (non-seizure) on the rt hemi and severe slowing on the left. Even w/ all of these issues, my daughter has made tremendous progress in all areas of function ... I just need to figure out and correct these unusual seizure episodes. Thanks everyone for your help & insight.

Her sugar has been tested during status, it is typically higher than usual (the sz likely causes it to elevate) I have not checked her BP here at home, but it has been checked a number of times at the ER and by the EMTs. EMTs used to pick her up a couple of times a month for prolonged seizures. Thankfully she has not needed them in more than two years ... so she has not had her bp checked during an episode since, but if it occurs again, I will try and elevate her feet to see if it helps. What type of sz did you have? A question I had for Dr Novak was whether or not autonomic dysfunction can trigger seizures. He said yes, so my next question was can improving autonomic function help to prevent seizures ... he said sometimes. I will do nearly anything to stop these seizures.

Hi there. I don't smell smoke, but I smell a burnt smell every now and again. I have small sz occasionally and so does my daughter. As above posters mentioned, sometimes these types of smells are auras ... either migraines or seizures. But the smell I smell is "real" and specific to certain areas. It is in my sweat sometimes. My daughter gets the burnt smell to her hair. I am assuming it is some sort of metabolic thing. We also get a metallic like smell to us ... again, it is an actual smell, not an aura.

Hi all. I am new here. I have a hx is Ehlers Danlos Syndrome & Dysautonomia. I have a 4 y/o that I am much more concerned about. She has EDS (likely) but she also suffered a stroke during birth that later resulted in seizures. Her first obvious seizure was a three hour non-convulsive status episode. She went on to have several more episodes like this until she eventually got some control w/ a modified keto diet, LGIT. She is not sz free however. Her seizures are very difficult to detect. They are primarily autonomic simple partials. A seizure for her consists of sudden nausea, dilated pupils, tachycardia (her hr will jump from 80 up to as high as 180), her o2 drops down into the 80s and occasionally 70s, sometimes she will vomit. These episodes typically only lasts about 15 to 20 seconds, but she tends to cluster. They occur over 4 to 6 weeks. She rarely has status (prolonged seizure) anymore, but when she does ... it is terrifying. There is a ton of epilepsy in my family, but these sz my daughter has are bizarre and extremely frightening. Her last status was in September. I found her laying on her side unable to move or speak. She was able to respond w/ her eyes though and was quite alert & responsive. During this event, her HR jumped to about 170, her o2 dropped slightly and her temp jumped to 102 (an autonomic response NOT a febrile seizure). SO basically she was quite alert and could respond w/ her eyes but completely paralyzed and unable to speak. I have sleep paralysis every now and again, this is what some of her status episodes remind me of. I managed to get some video of that episode. My daughter sees one of the best epileptologists in the country. I showed her the video, she thought it was seizure but was not sure if it was all seizure and wants a movement disorder specialist to see the vid. I have contacted one of the leading authorities on Autonomic seizures/status and he says it sounds like aut status, but I think he assumed she was unresponsive. I have also spoken to aut specialist Dr Peter Novak. When I see him again, he will watch the video, but he questions whether it is even a seizure at all. I am certain at least some of it is, but I am wondering if a true seizure may be triggering something else such as status cataplexicus (prolonged cataplexy) or if it may be triggering some other strange issue. I am thrilled my daughters seizures have improved, but the fact that her seizures are mostly purely autonomic scares the heck out of me! I know she likely has dysautonomia ... I worry these autonomic seizures & dysautonomia put her at greater risk for a dangerous arrhythmia. Anyone out there have any experience w/ autonomic seizures along w/ dysautonomia? Thanks

Hi all. My name is Jen & I am new to this forum. I was excited to see this topic here. I have quite a bit of info re keto. Med professionals are now experimenting w/ keto and many other neuro issues beside epilepsy ... brain cancer, autism etc. I have a hx of EDS & Dys. I have a 4 y/o daughter w/ EDS, hemiplegia from stroke & refractory epilepsy. She has been on a couple of meds for her seizures but they failed. She is currently on a modified ketogenic diet called the Low Glycemic Index Treatment (LGIT). It was dev by one of the leading keto experts (our epileptologist). LGIT is not as rigid as keto or unhealthy, but can be as effective for some. One of my daughters biggest dys symptoms is heat intolerance. I believe that her face does not sweat. Her intolerance is quite significant and can cause seizures. Some of her other dys symptoms are GI, pupil, & skin color issues. After being on LGIT, I noticed many of her autonomic symptoms improved quite a bit, that is until this past summer. We had a huge mold issue (inside & out). My dysautonomia issues are the usual. POTS, ortho hypotension, the usual GI & bladder issues and temp regulation problems. After seeing many specialists and trying many meds, the one thing that actually works for most of my issues is diet and avoiding certain med types (especially dopamine antagonists). The diet that works for me is also fairly low glycemic, but I could never tolerate the low carb part of the diet that my daughter is on. It will actually trigger more pots & low bp. Anyway, I look forward to getting to know you all. I have zillions of articles on the different diets, Ketogenic, LGIT, MAD (another mod keto diet) and so on. If anyone is looking for any specific info, I'd be happy to provide links.