Chiari Malformation and PANDAS overlap questions.

[Stephanie2]

Hi Ladies,

My husband and I had a talk tonight that has scared me half to death. We actually discussed whether or not we should pursue Chiari decompression surgery in the near future for our 2 year old little angel who has pandas and chiari among some other health issues (also recovered from autism - praise God!).

 

I have posted these questions in the past and I got some response, but I thought I would try again in case there are some new people on here who have experience with Chiari and PANDAS.

 

So here are my questions/theories:

 

1. The sensory sensitivities I am witnessing could be from both chiari and pandas, right? (sound, tactile, light)

2. How about feeding issues, can't tolerate any texture. He is at the point where he demands to eat only baby food! At first I thought it was part of his OCD, but now that he has done a steroid taper and his OCD seems to be mostly remitted, I am wondering if it is more of a sensory issue or even an inability to swallow whole food (some chiarians end up on feeding tubes until surgery can correct it - I am so scared that is where we are headed). Any thoughts?

3. Speech delay that comes and goes. I guess with chiari and pandas waxing and waning the way they do, it is hard to tell if both or either of these could be causing the speech issues. Sometimes my husband and I feel like he is making a ton of progress but then the words go away and it's like we are back to square one.

4. And here's the big question: Could the inflammation that is caused by PANDAS actually create a "swelling" of the brain that could be causing the cerebellar(sp?) tonsils to further herniate into his neck, thus the worsening of chiari? I really just wonder if I should do IVIG first and see if the "swelling" goes down and if not then pursue the chiari surgery (not the other way around).

 

Unfortunately, there are no MD's or scientists that would have these answers b/c there are no studies that look at the overlap of chiari/pandas.

 

Any input would be great!

 

Stephanie

Facilitator - PANDAS Support Group of Florida, affiliated with www.pandasresourcenetwork.org

Edited July 9, 2010 by Stephanie2

[Stephanie2]

Oh and if this is not enough, he is scheduled for inguinal hernia surgery in the morning and is off his pandas meds for a couple days !

 

Prayers please! Thank God DS5's pandas is in remission at the moment!!

Edited July 9, 2010 by Stephanie2

[Stephanie2]

Oh and if this is not enough, he is scheduled for inguinal hernia surgery in the morning and is off his pandas meds for a couple days !

 

Prayers please! Thank God DS5's pandas is in remission at the moment!!

[Stephanie2]

Oh and if this is not enough, he is scheduled for inguinal hernia surgery in the morning and is off his pandas meds for a couple days !

 

Prayers please! Thank God DS5's pandas is in remission at the moment!!

 

Did I just reply twice to my own post? I think I did...

 

What a day...

[thereishope]

I can approach these from the PANDAS perspective only...

 

Sensory: My son's sensory was affected during exacerbations. Even when he is fully recovered, he will still have some sensory things here and there but nothing to do be concerned about. realistically, a lot of people have as sensory issue and are not aware that is what it is.

 

Feeding issues: My son had this. Like yours, he was to the point of eating very mushy food. I actually thought about feeding him baby food for nutrition at some point but just couldn't do it. I don't know why he was like that. I even explained what a feeding tube was to him and it didn't phase him. Once he began recovery, the eating issues took a long time to fully correct themselves but they did eventually. He no longer has any food issues.

 

Speech delay: My son has this. And, like yours, the severity will change. When he is in an exacerbation, it will worsen and he will stop improvement. Once recovered, he's back on track.

 

I know this doesn't really answer your question about how chairi and PANDAS overlap and how to determine what is caused by what. But maybe it helped a little? I tried!

 

Prayers being sent your way!

Edited July 9, 2010 by Vickie

[sptcmom]

Stephanie- does he have Chiari type 1 or type 2? In my experience, some children when diagnosed early around toddlerhood, I've seen milder presentation and some neuros classify it as type 1 with minor protrusion of the cerebellar tonsils. Mom decided to have surgery due to numerous flareups daily and the decompression surgery was successful. I've seen another little boy with craniosynestosis since infancy, had corrective surgery at 6 months of age with good results- he now is 4 and has always had massive feeding issues, sensory tactile and oral motor both, overall massive tactile issues and very high anxiety level, just potty trained. The child with Chiari had all the symptoms you mention like speech delay etc plus started getting cerebellar involvement issues with balance, timing and grading force of movement, very high pain tolerance, poor body awareness, poor bilateral coordination and praxis type activities that need the cerebellar feedforward feedback mechanism.

The swelling that can exacerbate Chiari downward glide of the cerebellar tonsils has to be an event like a fluid type swelling like a cyst, or meninigomyleocoele filled with cerebrospinal fluid that can actuallycreate a push with gravity assisting ofcourse. It can also be due to the membrane swelling/ subarachnoid hemorrhage caused by a traumatic brain injury. A localized inflammation in the basal ganglia area isn't really a literal swelling swelling I don't think. Its more like an irritation of the neurons being attacked and annoyed by the antineuronal antibodies.

Did you do a spinal tap to check for organisms ? Sometimes neuros will recommend.

Edited July 9, 2010 by sptcmom

[Stephanie2]

Stephanie- does he have Chiari type 1 or type 2?

 

Vickie,

Thanks, that did help. It gave me some hope that this is not all chiari related...maybe IVIG will clear up the lingering pandas issues and then I will see what's left and decide that there's not enough to do chiari surgery.

 

Chiari type 1

[norcalmom]

Yes - we've have sensory issues too. I think you are right to go one step at a time. Least invasive first and see if it warrants the next step. Please keep us posted. I'm sending you good thoughts!

k

[peglem]

Yes - we've have sensory issues too. I think you are right to go one step at a time. Least invasive first and see if it warrants the next step. Please keep us posted. I'm sending you good thoughts!

k

I was going to post exactly that- guess you beat me to it. I'm thinking if the chiari and the symptoms your child has improve with the less invasive procedure- then you'll know and it will make your decision less complicated. And you do have my prayers!

[sptcmom]

Stephanie- does he have Chiari type 1 or type 2?

 

Vickie,

Thanks, that did help. It gave me some hope that this is not all chiari related...maybe IVIG will clear up the lingering pandas issues and then I will see what's left and decide that there's not enough to do chiari surgery.

 

Chiari type 1

Chiari speech delay is more like waxing and waning, loudness issues, lisping, word finidng difficulty and can change a few times in a day depending on the stress level a child is experiencing - physical and mental stress. No real expressive or receptive language delays, only perceived as expressive language delay and strong possibility of low tone oral motor apparatus, muscular low tone overall in the body and most issues due to motor planning of the speech. Possible auditory processing issues with cerebellar connections like doesn't seem to listen or hear instructions given when moving/running, playing etc. Takes a highly skilled therapist to understand this. Careful use of Tomatis method /Listening program helps along with neuro sensory RX.

PANDAS speech issues are more of a semantic pragmatic language issues, concrete thought processes, difficulty with abstract thoughts and concepts, nuances of language and interpreting others' body language etc. More of the higher level speech issues, like sequencing and organizng language is affected. Tomatis method Bone conduction listening program works better but will need a couple cycles along with higher level multisensory processing therapy-

I'm talking now only about the speech/ language issues. Other issues will need to be addressed based on presentation.

 

With true PANDAS, after treatment, the semantic pragmatic language issues clear up almost like a fog lifts. Pandas kids retain all the language and all skills learned but may not have been able to consistently reproduce at will till being traeted. Some will respond right after ABX and some will need IVIG but the issues will clear up including gross motor, fine motor, visual motor ( visual perceptual esp visual spatial issues seem to persist longer).

Chiari will need the decompression surgery to see major consistent change.

Children recover well with any surgery in the hands of a good surgeon. Neuroplasticity plays a huge role in all children till myelination and perceptual development is completed which is approximately 7 yrs of age for girls and 8 for boys from what I remember.

Tons of luck and prayers coming your way

- Jodie

[kimballot]

Stephanie - First off - my heart goes out to you having to sort through all of this with your little two year old.

 

Regarding Chiari Malformation - my experience is with kids with spina bifida as many children with spina bifida also have chiari malformation. I understand that your child does not have spina bifida, but you may want to look at some of the information in the spina bifida parent websites to try to determine when parents went for decompression vs. waiting it out.

 

Here is a website that talks about it a bit - and it might help lead you to more- it talks about looking at how far down the tonsils have herniated and such. http://www.spinabifidaassociation.org/site...alformation.htm

 

I have also seen some increases in sensory sensitivity in some kids. That is such a general thing to see that it is difficult to say if it is from PANDAS or Chiari. Some of the things I have seen with kids with chiari malformation that are due to compression are muscle related, such as increased muscle tone (tightness in the muscles when you try to passively move them), having an eye that turns in or turns out, difficulty with speech, and difficulty eating. I have also seen kids become fatigued just prior to decompression surgery.

 

Hope that is helpful -- keep us posted on how things go!

Edited July 9, 2010 by kimballot

[tantrums]

I'm still trying to figure this out myself, AND trying to get that MRI to find out if DS7 has ACM. But I have done some research as I'd originally thought he was presenting with ACM when this started and I'd not yet heard of pandas. I also have ACM and have to have the decompression, so I can somewhat venture a guess with some of it.

 

The language/speech - I agree with PP that said it was also waxing and waning. I have days I can barely form a complete sentence. Mainly focused around word finding, but then it can affect a whole conversation. I would think a 2 year old learning language, this could cause more of a problem. Also - don't forget how ACM can affect hearing. I know I have such ringing and buzzing in my ears sometimes that it's hard to hear correctly. This would certainly affect the way a toddler learns language.

 

I DO think the swelling from pandas would affect the cerebullar tonsils. Very close in area and if your son was lucky enough (I know - eye roll here) to be diagnosed early then he must have a significant herniation already even if it is only ACM1. I had a neurosurgeon explain to me how the fluid builds up but then disipates so I sometimes will have actual months where I feel pretty good with little problem. But the pressure when it is too high - causes all sorts of weirdness. So add swelling to the BG from pandas and I would imagine it's just a whole other issue to deal with in that little brain

 

Personally, I tend to think the food texture issue is OCD. Usually ACM would only cause gagging/choking, not a preference. Although, it's possible he did do that and is scared now.

 

Light sensitivity I would think is definitely related! I have major light sensitivity and also sound. This was one of the first things that made me cringe with my DS - he started with headaches that are aggravated by light (and also movement of his head ) Also, if he has any vertigo - that could affect a TON of other sensory issues and he wouldn't be able to verbally express the problem to you yet

 

I'm so sorry you're dealing with both of these and with more than one child no less! Like I said, I'm still left wondering with my DS if he is affected with ACM. Problems with the insurance for sedation for that MRI and a neuro that says ACM is NEVER hereditary have held us up. But I do know at least my DS isn't presenting as surgical. I just want to know for future monitoring, sports, etc... at this point. I also don't want him to suffer as many years as I did.

 

Also - honestly from what I have read on the surgery, especially for children, it really isn't that bad. I personally don't fear it at all myself. But of course I've had one heck of a headache for 14 years. I would fear it more for my child of course. But I hear more stories of kids just sailing through it more than any with ANY issues from it.

[Stephanie2]

All I can say is HOLY COW, you are a well-informed group of ladies!! I am going to come back to this later - gotta go get my son. My head is spinning just from skimming those posts!!

 

 

 

I'm still trying to figure this out myself, AND trying to get that MRI to find out if DS7 has ACM. But I have done some research as I'd originally thought he was presenting with ACM when this started and I'd not yet heard of pandas. I also have ACM and have to have the decompression, so I can somewhat venture a guess with some of it.

 

The language/speech - I agree with PP that said it was also waxing and waning. I have days I can barely form a complete sentence. Mainly focused around word finding, but then it can affect a whole conversation. I would think a 2 year old learning language, this could cause more of a problem. Also - don't forget how ACM can affect hearing. I know I have such ringing and buzzing in my ears sometimes that it's hard to hear correctly. This would certainly affect the way a toddler learns language.

 

I DO think the swelling from pandas would affect the cerebullar tonsils. Very close in area and if your son was lucky enough (I know - eye roll here) to be diagnosed early then he must have a significant herniation already even if it is only ACM1. I had a neurosurgeon explain to me how the fluid builds up but then disipates so I sometimes will have actual months where I feel pretty good with little problem. But the pressure when it is too high - causes all sorts of weirdness. So add swelling to the BG from pandas and I would imagine it's just a whole other issue to deal with in that little brain

 

Personally, I tend to think the food texture issue is OCD. Usually ACM would only cause gagging/choking, not a preference. Although, it's possible he did do that and is scared now.

 

Light sensitivity I would think is definitely related! I have major light sensitivity and also sound. This was one of the first things that made me cringe with my DS - he started with headaches that are aggravated by light (and also movement of his head ) Also, if he has any vertigo - that could affect a TON of other sensory issues and he wouldn't be able to verbally express the problem to you yet

 

I'm so sorry you're dealing with both of these and with more than one child no less! Like I said, I'm still left wondering with my DS if he is affected with ACM. Problems with the insurance for sedation for that MRI and a neuro that says ACM is NEVER hereditary have held us up. But I do know at least my DS isn't presenting as surgical. I just want to know for future monitoring, sports, etc... at this point. I also don't want him to suffer as many years as I did.

 

Also - honestly from what I have read on the surgery, especially for children, it really isn't that bad. I personally don't fear it at all myself. But of course I've had one heck of a headache for 14 years. I would fear it more for my child of course. But I hear more stories of kids just sailing through it more than any with ANY issues from it.

[tantrums]

I'm sure all of us would MUCH prefer to be a bit less informed I thought I had my head full (no pun intended) with the ACM stuff. Didn't know I had room for pandas stuff too!

[Chemar]

not sure if this can be of help to you Stephanie, as it doesnt involve the PANDAS aspect, but there is a lot of archived Chiari info at NeuroTalk