Suspect SM

[Teri]

Hi everyone, thank you so much for all your information and insight. Sorry about not giving an update sooner, we've had some other things going on and I haven't been online very much.

We made an appointment with the closer neuro for a consultation. He won't do an MRI without it, and so we're scheduled for Monday the 14th. He was booked solid until then. In the meantime, our vet put Sadie on Furosemide and I am actually seeing an improvement! She seems happier and more alert and energetic. It's amazing the difference it has made. She's always been a happy girl, spinning in the air and dancing when she knows its dinnertime for example, but now she seems more joyful and for more than just a few moments - now for most of the day. I am very surprised it has made a difference. I am now wondering if we should take her off it just before her appointment so he can see her 'natural' state? I hate to make her worse again, but does he really need to see the symptoms himself or can he just take my word for it what her symptoms have been? I may give his office a call tomorrow when they open to see what they recommend. During the consult I will be checking out his credentials for SM surgery in case she needs that. Dr Bagley trained him so maybe he'll be okay. If not, we'll go to Dr Bagley across the state.

Anyway, I'll keep you posted on the consultation on the 14th! Thank you so much for all your suggestions and support!

[Karlin]

Thanks for the update -- I am so glad the furosemide is helping. I've never heard of a neurologist wanting to remove a dog from something that is relieving pain in order to see how the dog is doing so I wouldn't worry about it but check with his office if you want to make sure. A neurologist will be able to test all the things in a consult that indicate the seriousness of SM -- things that will not be much affected by frusemide. But s/he will also rely on your description of symptoms. I would write down all you have seen, frequency of scratching, etc so you have that to hand.

The neurologist is doing exactly the right thing -- they will always do a consult and physical exam before the refer for an MRI (just like human doctors). They wouldn't schedule an MRI without being sure that you need one.

I'd consider asking the neurologist for a recommendation for your vet for the point at which a dog should be referred to a specialist. I am concerned that your vet thought it would be better to try and treat with something like furosemide and only if that *didn't* work, to refer to a neurologist. The reality is that this condition should *always* be immediately referred to a neurologist unless the owner is firm in only wanting to give some short term pain relief before putting the dog to sleep. Finding a correct and effective medical palliative approach absolutely *requires* a neurologist; likewise only a neurologist can give a prognosis and possible recommendation for surgery. The earlier the condition is treated in any form, the better a chance at a decent life for the dog, and the less suffering the dog will endure. With many dogs, even weeks or days can make the difference between permanently crippling damage and avoiding it by surgery, for example -- or between life and death if the damage has gone too far. Vets need to clearly understand how very serious this condition is, and how urgent it is for owners to get their dogs to a specialist when the vet can find no other reason for SM-like symptoms. Certain symptoms -- scoliosis and air-scratching in particular -- are unlikely to be due to any other cause except SM in cavaliers.

Let us know how the consult goes.

[Teri]

Thank you for the support. It means a lot to me. And you were right of course. I called the neuro's office and they confirmed that I should just keep her on her meds for the consult, so I'm happy about that. And yes, I plan to share with my vet what I've learned from the neuro (and you guys!) since she admitted to me from the start that while she knows clinically what SM is, she's never seen it herself before nor has she ever treated it. She is interested in learning more about it and will be talking with the neuro after the consult.

Another thing I am concerned about is that when Sadie is in public, she won't show any signs. I tried to get her to scratch for the vet by walking and also by touching her neck, nothing. Her back was straight, she didn't scratch, she wouldn't present any symptoms. The vet checked her strength in each leg and she passed with flying colors. So I hope she shows a few more signs for the neuro!

I'll keep you guys posted after the consultation on Monday! Thanks again for all your support!

[Karlin]

That's all good news.

It is a specialist condition so vets should not be expected to even recognise it -- it is smart of your vet to have even suspected . But at the same time, a vet should immediately talk to a specialist on a condition like this, not try to treat minimally and see what happens. With a neurological condition like this, the best chance for the dog is spotting this early when there are the most options and least amount of permanent damage. Clare Rusbridge provides some good background info (that I have available as downloads, with Clare's permission, on www.smcavalier.com).

A neurologist can do the same tests as a vet and spot problems right away. It's like the difference between a cardiologist and a vet listening to a heart. A cardio has specific focused training to spot heart problems and know exactly what the problem is and can easily hear grade 1 early murmurs. About half of vets will not pick up a murmur at all until it is in the middle grades.

[Teri]

Yesterday we took Sadie in to the neurologist and it well. She, of course, didn't show any symptoms that we regularly see at home no matter what we did that normally gets a reaction, so to him she looked perfectly normal. She's also been having much better days since being on the furosemide. He did all kinds of tests on her and every one of them was 'normal'. So he was happy to see that. But based on her symptoms that we reported to him, he strongly suspects "COMS (Caudal Occipital Malformation Syndrome)". I hadn't heard of it referred to as that but he wrote it down for me. He is also in contact with Drs Dewey and Marino and they are all on a listserv together with other neurologists where they talk about these things, what works, what doesn't, etc.

Anyway, he didn't push us into getting an MRI. Said that on a scale of 1-10 he thought she was about a 2 so it wasn't imperitive that she have an MRI immediately unless we wanted to know. Well, we do want to know so she's scheduled for an MRI this Thursday. Once she has the MRI then he will prescribe the best treatment for her based on the findings. We will talk to him that same day to get the results and the treatment plan so we should be well informed by then.

I'll keep you guys updated after Thursday! Wish us luck!

[brotymo]

I will be pulling for the best of outcomes for your Sadie. She is lucky to have such a consciencious owner.

[Karlin]

I'm glad you had a good meeting and I am sure you will feel more comfortable having had an MRI so you know what is going on.

The neurologists attending the London SM conference actually voted to discontinue use of the term COMS because it is inaccurate in a number of ways -- this included Dr Marino -- but many (including Dr Marino) do seem to continue to use this term. US neuros use it more than others probably because the term was used by Drs Marino and Dewey in several papers on the subject and not that much has been written overall.

Minutes from the meeting:

Occipital hypoplasia and Caudal occipital Malformation Syndrome (COMS) -
These terms were rejected because there is no proof yet that the condition is related to
either a malformed or hypoplastic occipital bone(s). Current evidence suggests that
there may be other significant factors in the pathogenesis. In addition these terms can
be confusing as for example the term COMS may imply the malformation only or the
malformation and syringomyelia.

You can read more on the meeting here:

http://sm.cavaliertalk.com/research/...n/minutes.html

[Teri]

Sadie's MRI appointment was first thing in the morning today, no food after 10pm last night, water was okay. She was a little grumbly when we left the house because we "forgot" to feed her breakfast. We got her there at 8am, made sure she was first on their schedule (they give priority to people who are out of town), signed the papers, made the payment and then headed out. We were told to expect her to be ready to go home around 1pm.

Around 11am the doctor called and said they finished the MRI and he's reviewed the scans. He said it is an extremely severe case of COMS and she also has hydrocephalus. She has a really large syrinx which has left only 5-10% of the normal spinal tissue present. He was surprised that she was so bad when she didn't present any symptoms to him and mild symptoms to us. He's seen and heard of it before of course but he says it never ceases to surprise him whenever it happens and he finds it very frustrating. He said that if he had seen the scan before he met her he would have assumed she wasn't mobile.

Basically we have two options, medications and/or surgery. We will be starting her on the recommended meds in the next day or two and see how she does. We need to get the meds set up at our local pharmacy, one needs to be compounded which is why the delay. In the meantime she will continue on her current meds. He recommends Omeprazole 0.5 mg/kg once a day to reduce CSF production, and gabapentin 15-20mg/kg every 12-8 hrs to help control abnormal sensations. She may also need to be on prednisone but we'll try this first. We arrived back at the clinic to pick her up and they gave us a three page printout of the results, including her scan where they inserted labels of what we were looking at. They also recommended that she not go for long walks, that she doesn't climb a lot of stairs, and absolutely no jumping on or off things like furniture. They said going up a couple stairs to the couch or off the deck was fine, but no long flights of stairs. They said it was better to carry her. We don't have more than three steps from our house into the garden and have a couple steps so she can get on the sofa, so we're good there.

She has slept a lot after coming home and had a glazed look on the drive home. She's been pretty groggy from the anesthesia but is starting to perk up tonight. She had a light dinner, and is checking for crumbs in the kitchen so she's getting back to normal.

So... that's our news. We're still in a bit of shock and feeling overwhelmed here. We expected the diagnosis, but not the severity. She seems so happy, and is even better since being on the furosemide. We will do everything we can to keep her comfortable, happy and pain-free. Haven't decided on surgery yet. The neuro seemed to think we should do the meds first and then do surgery if/when the meds stop working. He said if we do surgery, that given the extent of the syrinx, the goal of the surgery would primarily be to prevent the worsening of any clinical signs. He said to contact him if her clinical signs worsen or if we'd like to schedule surgery.

Are there any stats of dogs who are this severe who have been successfully managed with meds alone?

[Karlin]

Wow, that must have been such a lot to take in. I am sorry you got a more worrying report back as you do always hope for the mildest scan. Also yes that is definitely the case that sometimes really mild scans have very severe symptoms and vice versa but this is always pretty unusual.

If you want an honest opinion from me, if this were my dog, I'd want to see if Dr Marino or Dewey or Rusbridge would give you a second opinion on the scan; you can probably contact LIVS in Long Island and arrange for a second opinion reading or same from Clare Rusbridge for a fee of some sort. I'd just be concerned that if so little space is left in the spinal column that you may end up with a severe onset where the only real option might be to pts which means you had no options. Medication is always an option but 'option' in the case of SM is a limited term and has usually, a pretty narrow meaning in severe cases. Medication generally has to be viewed as something which may help limit pain for a certain amount of time but for symptomatic dogs things generally worsen and medications do not halt or prevent or reverse SM. If you are seeing such a massive syrinx, that shows gradual severe progression over time, slow enough that Sadie has to some degree learned to cope. I have heard DR Marino speak of a similar MRI that he showed at a UK meeting where he noted that if a dog had such a syrinx come about suddenly say due to an impact, he'd expect the dog to be in crippling pain. Only because it comes on gradually does the dog learn to cope.

With a really severe scan I'd be wondering how long the dog could continue to progress and cope and how much permanent damage will be done once that syrinx enlarges any further. I'd be opting for surgery myself or viewing the situation as having chosen the option of palliative care. But that is me. However this is based on having a pretty good idea of what Drs Dewey, Marino or Rusbridge would say with a scan like that -- I think they'd be saying do the surgery, if you want any chance of significant time left with Sadie. But again that's something you'd want to speak to someone directly about which is why I am suggesting a second opinion IF surgery is an option in your mind.

The other reason I'd suggest this is that, based on your original post, I would not call the symptoms you have seen as mild or insignificant. This is what you noted about Sadie:

We have suspected that my girl Sadie (almost 5 yrs old now) has SM. She does the bunny hop and air scratching when she walks, she scratches her neck/ear area, she's slowed down and sleeps more, she needs steps to get up to the couch because she won't jump up anymore, she won't jump off anything, and we have to pick her up in just the right place so she doesn't yelp, and she sometimes looks like she has a headache. These have all been mild and variable symptoms where they only show up once in awhile since she was two. But lately they have increased in frequency and she also started getting a head tilt (we noticed it just a couple weeks ago after we watched a video of her we had just taken) and she is getting some curvature of her spine at times. We are worried she might be in pain and want to do more to help her.

Many neurologists -- including all those doing research in this area, whom I have heard speak a couple of times -- would definitely consider these moderate to severe symptoms -- head tilt, scoliosis and inability to get up stairs or onto furniture count as severe symptoms for Dr Rusbridge and Dr Marino.

That won't be what you wish to hear after going through so much already but in your position, and considering the two options, I'd very definitely want a second opinion and I'd want it right away.

With a syrinx that severe, if I were considering surgery I'd probably be looking at going to one of the neurosurgeons who do the modified decompression with titanium mesh or a shunt to drain the syrinx. Regular decompression doesn't seem to reduce syrinx size and with a large syrinx I'd probably want to get some reduction of syrinx size.

Again, I am speaking about the choices I would make, and I know this is a very personal decision and is never easy. We all have different approaches and paths with this condition.

[Ruth M]

Hi Teri
Thinking of you and the dilemma you are facing. My nearly 2 year old b&t 'Tilly' has been diagnosed with severe SM through MRI - and I have been on your journey - what a rollercoaster of emotions However access to this site has been a revelation - Tilly has the correct dose of gabapentin (now taking it really well in cream cheese - thanks Karlin )!
You face a tough decision - the neurologist I saw at the Royal Vetinary College (UK) didnt even mention surgery as an option - just gabapentin and wait and see. They said many dogs lead a full and relatively pain free life with the meds. I was thinking of a referral to Clare Rusbridge.
Tilly has the gabapentin 3 times daily - and is doing really well on it, she is slighly sleepier, but has a good appetite, jumps for her lead and loves her walks!
Heres my decision - and I dont want to sway you but there is sooo much to consider. We will not opt for surgery. Tilly has already had major surgery (luxating patella) and the impact and worry on our family was huge. Even recovery from the MRI was tricky.
I was heartbroken when I found out Tilly had SM - you get a cavi and soon realise they become a pivotal part of your family life - shes my little buddy! But then I took a step back and reassessed the situation - why did we get a doggie in the first place - to enrich our family life. I was fretting about Tilly so much it was slowing enveloping me - and my family. Now that I have decided - keep my girl as pain free as possible, give her an awesome life - live it to the full - and one day at a time - we ALL (including Tilly) seem so much more relaxed and are just loving our time together - however long that may be.
Anyway thats me - I really feel that now we have made a decision a great weight has been lifted.
Just wanted to share this with you - its not the decision for all I know, but I think its good to look at all the angles - as Im sure you are.
Thinking of you and yours.
Ruth