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Dr. Andrew Rynne

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Suggest remedy for congenital hydrocephalus

Answered by
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Dr. Vinay Bhardwaj

Neurologist, Surgical

Practicing since :2006

Answered : 544 Questions

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Posted on Mon, 26 Oct 2015 in Brain and Spine
Question: 55yo family member with congenital hydrocephalus and a VP shunt since 1yo. Deteriorating slowly in cognitive and motor functioning over lifespan but in the past 12 months the decline has become quite rapid. He is now barley able to walk, sporadically incontinent, takes a long time to verbally respond and has had to resign from supermarket work of 17 years. Neurologist says it is stress, possible dementia. I have recently discovered by looking at CT reports that mild long standing ventriculomegaly was observed twice in 2009 by the radiology doctor - but no mention of it in following CT reports by other doctors. Could this be a possible cause of decline?
Have uploaded CT scans from 2009 to the most recent 2015. Any opinions would be greatly appreciated.

https://yyyy.yyy.com/yyyyy?yyyyyy
doctor
Answered by Dr. Vinay Bhardwaj 19 hours later
Brief Answer:
Managment of Hydrocephalus, need some information

Detailed Answer:
Hi, Thanks for coming to HealthCareMagic.

I'll try and parse out the information you've provided so I'm sure I understand it. While doing that, I'd like to ask you a few questions so I can come to a plan.

So, I'm sure you know what Hydrocephalus is, but I don't want to make any assumptions, so please bear with me on the remedial lecture.

Buried deep in the core of your brain is a small cluster of cells which constantly filter blood into a fluid called "Cerebro-Spinal Fluid". This stuff is very important to keep the neurons in your brain well fed. Now, alot of this CSF is made on a daily basis. Liters of it in fact. Some of it gets stored right in the center of the brain in reservoirs called Ventricles. But they can't store all that much. So your brain has a well developed system for reabsorbing the excess and used up CSF.

99% of the time Hydrocephalus occurs due to blockage of this re-absorption pathway.

When that happens the CSF builds up in those ventricles.. making them stretch. The stretching of these ventricles is the Ventriculomegaly that you mentioned. The symptoms of hydrocephalus are the very same as you have described. So I'm sure you guys are all on the same page as far as the diagnosis.

One quick question though.. You said the patient is 55. And that the Hydrocephalus was 'congenital' (ie: He was born with it). Also you said that a shunt (VP shunt) was placed a year ago. What kind of treatment has this patient been on since birth? Was an Endoscopic Third Ventriculostomy done? The Neurosurgeon may have called it the ETV procedure.

Reason I'm asking is it's important for me to understand the current anatomy of your patients brain to understand the sudden neurological deterioration they have suffered.

Adult Hydrocephalus is also seen at this age.. That's when the blockage in the drainage pathways happens slowly over years.

Now, you've said that the patient has a shunt. I'd really like to know what kind of shunt they have? A static shunt? A programmable one (one where the pressure n the ventricle can be monitored and controlled)? When was the last time the shunt was "tapped" (ie checking if the shunt is working or not by suctioning some CSF out of it with a small syringe.). Tapping is a way of confirming that the shunt is actually working.

Since there has been some sudden deterioration.. there is a strong possibility that the shunt is malfunctioning... either OVER draining the ventricle or UNDER draining it. Both can cause the symptoms you've mentioned. The best way to determine that is to get a CT scan done of the patients brain right away and compare them against earlier scans.

I know you've uploaded some scans.. to a google drive.. but I can't seem to open that drive. I'm not sure why.. If you could please upload screenshots of the scans to this query (there should be a link on the right hand side of the screen). If you have trouble with that, you can email the screenshots to YYYY@YYYY and ask the CRM team to upload the screenshots to queryID 224507 (that's this query's number).

Now, if there is a problem with the shunt.. it may require replacement. It's a surgery that basically mirrors the placement of the shunt. It's unfortunately a fairly common occurrence, so I wouldn't be surprised.

On the other hand. It may be that the long standing hydrocephalus is finally entering its final stages. Even with the best shunting and constant monitoring, hydrocephalus takes a toll on the brain.. tiny blood vessels keep on breaking down on the inside of the ventricles and the neurons which are exposed to the unfiltered blood slowly get damaged.. leading to Dementia. It is an unfortunate part of the natural evolution of this disease. If the patient has had this since birth.. then he has been quite well controlled to have come this far.. but the dementia is, to a great extent, inevitable.

Whatever be the case, I'll be happy to help you and your patient form a plan to combat the problem. I just need more information. So if you could clarify and answer the questions I've asked that would be great. If the patient has a discharge summary from a recent admission.. that could be scanned and uploaded as well, it would be invaluable. Uploading screen shots of the CT-scans would be a great help as well.

I look forward to your response.

Vinay Bhardwaj, MD




Above answer was peer-reviewed by : Dr. Vinay Bhardwaj
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Follow up: Dr. Vinay Bhardwaj 1 hour later
Thank you very much for the reply.
It is very appreciated.

To answer your questions (sorry to confuse) he does have congenital hydrocephalus and was treated with a shunt when he was 1 year of age (not one year ago). I have read the his whole medical history and it says a ventriculostomy was done which was of no help then VP shunt was installed - during this he suffered a stoke to which resulted in little weakness on his left side.
I think it was a programable shunt. (Was installed in 1962 and the report writing is nearly incomprehensible)
The shunt has been barley monitored since it has been fitted.

I have uploaded screenshots of the CT scans from 2009 and the most recent 2015. Your opinion on them would be very much appreciated.
doctor
Answered by Dr. Vinay Bhardwaj 21 hours later
Brief Answer:
Very doubtful that its a programmable shunt

Detailed Answer:
Hey there, thanks for following up.

I'm a bit skeptical that this is a programmable shunt. The first programmable ones came out in the late 90's. There may be a valve in there, but it's got to me manually controlled. Anyways. It looks like the shunt is definitely malfunctioning.

I can't see the CT scans you've uploaded on gmail. Could you email them? you can send them to YYYY@YYYY , send it with a message requesting the tech team to upload them to queryID 224507.

It looks like the shunt is going to have to be tapped. It can be done by a neurosurgeon at a hospital. The shunt HAS to be tested.. even CT scans are only INDIRECT evidence of function.. the best thing is the tapping i mentioned before. Sometimes, the patient would have to be admitted to the hospital and the shunt would have to be 'externalized'.. That means the part of the shunt going into the brain (its also called the Omaya Reservoir) would be connected to an external pressure gauge. The pressure would then be measured while the patient is in bed for a day or 2. The pressures should be above 10 and below 25 (CM of H20), depending on how the gauge is calibrated.

All this kind of stuff needs to be done by a good neurosurgeon. I can help you find one if you like. I look forward to your reply.

V
Above answer was peer-reviewed by : Dr. Chakravarthy Mazumdar
doctor
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Follow up: Dr. Vinay Bhardwaj 10 minutes later
Thanks for the reply.
I may have made a mistake, the shunt very well may not be a programable one.

He was in hospital recently for 1 month. He was taken to emergency due to being unable to wake and very disorientated. They where reluctant to admit him but finally did. He was in the medical ward for 2 days but then transferred to the rehab ward where the rehab doctor initially said rehab is a waste of time and the underlying issue needs to be investigated. There where no neurosurgeons at the hospital. I suggested all the tests you have mentioned and they basically laughed at me.

I have uploaded screenshots of the scans to the google drive maybe you could view them if the other files are unable to be read. I will alternatively email the screenshots as advise

Your opinion would be of great appreciation
doctor
Answered by Dr. Vinay Bhardwaj 38 minutes later
Brief Answer:
Got'em! This doesn't seem to be a shunt issue

Detailed Answer:
Hi there! Thanks for the quick reply! The Google drive link worked this time. I was able to download the DICOM files and look at them on my viewer. Now. I'm trained in Neurosurgery and critical care, am not a Radiologist. But I'm pretty sure I've got a good handle on these scans.

From 2009 to 2015, there have been some pretty serious changes. The ventricles have come to a fairly normal size even though they look enlarged.. thats because the entire mass of the brain has shrunk over time. The ventricles don't look to be under pressure at all in the scan in 2015. I'm reasonable sure that if there was a shunt malfunction.. that wouldn't be the case.

Now lets dive into the brain matter itself and look away from the ventricles. I really wish i could have you in a room with me and take you over the scans myself.. because i want to point at a few cuts of the brain and i don't know how to make that happen on this platform.. but i'm going to try..

Deep inside the brain, in the center.. there is this strip of bridging white matter (tissue) called the Corpus Callosum.. It's very very important for cognitive function.. it helps one side of the brain communicate with the other. That tissue looks very weak.. or poorly developed. It looks as though your patient NEVER had a fully developed callosum in fact (if i look at the 2009 scan, the callosum is also pretty rarified and thin). If you look at the white matter of the brain in general.. there are plaques and tangles that make me think that the brain tissue itself has deteriorated. This is very important. To me this means that different parts of the brain are having alot of trouble talking to one another. This is the core basis of Dementia.

I hope i'm making sense here.. but the pressures don't seem to be the problem.. at least anymore. It looks to me that the substance of the brain itself has degenerated over time. This could be due to a disease process other than hydrocephalus or it could be due to uncontrolled hydrocephalus over the decades.. but the outcome is the same.. the brain matter / substance itself is degenerating.

Unfortunately, hydrocephalus can lead to this.. in fact. it ALWAYS leads to this. I'm an egotistical guy for sure.. but even my ego won't let me claim that we neuroscientists can control hydrocephalus forever. The disease is inevitably degenerative and our science hasn't gotten us to the point where we can do anything more than delay those changes for increasing periods of time. We can't stop the damage entirely and also very importantly.. we can't reverse the damage that's been done. We're working on it though. I work with stem cell trials here in XXXXXXX and I know of alot of good work happening all over the world.. but it's still a work in progress.

What does this ultimately mean for your patient? I still think that it's a good idea for that shunt to be examined.. hand-on by a qualified Neurosurgeon.. my ego will allow me to say that unless you get an opinion on that shunt by a qualified neurosurgeon.. then all the other opinions are second rate. This is kind of our thing.

But, the damage has been done. Your patient will most likely not be able to recover from the constellation of symptoms you've reported EVEN IF the shunt is replaced entirely.

It looks like your patient may need to take medications that address the chemical basis of dementia (those plaques and tangles on the CT lead to deficiencies in the presence of key neurotransmitters in the brain).

Medications like Donepezil and Modafinil have been known to help people in these situations and I would encourage you to guide your patient tword these therapies along with alot of congnitive therapy and physical therapy. A good Neurologist can help you start these therapies.

I'm so sorry. I wish I could have told you that fixing the shunt would fix everything. But the CT scans don't look that way to me. I strongly suggest you explore the medications i mentioned, they can hopefully give your patient a better quality of life for the rest of his life.

Take heart, your patient obviously has a wonderful person for a caregiver (you) and I'm sure you will do your best for them. If there is any way I can help.. I certainly will.

V
Above answer was peer-reviewed by : Dr. Chakravarthy Mazumdar
doctor
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Follow up: Dr. Vinay Bhardwaj 51 minutes later
Thank you very much for the response.
We had a neurosurgeon here look at the scans and he come to a conclusion that it is unlikely the shunt is even functional and that he has become independent without it.
Does this mean he possibly had no hydrocephalus to begin with as a child (maybe misdiagnosed due to unobserved agenesis of the corpus callosum) ?? Could the ventriculomegaly be a newly acquired type of untreated hydrocephalus? NPH??
Or if the shunt is functioning, is there any evidence on CT of over drainage that could be causing issues?

Further to this, you mentioned that the entire mass of brain has shrunk overtime. Could this be due to calvarial thickening from his dilantin medication? Is so, could his skull be compressing his brain to make it smaller - possibly causing his symptoms?


Dear Dr Bhardwaj,

Thank you again for your response, it was the best we have ever heard and explained everything we have been looking for in the past 2 years.
I know you are very busy but we our appointment with the new neurologist is coming up finally this week and if you could respond to my last query it would be greatly appreciated (i promise it will be the last one haha). We are just trying to plan what to ask at the appointment.
doctor
Answered by Dr. Vinay Bhardwaj 3 days later
Brief Answer:
Sorry for the delay

Detailed Answer:
Hey. I'm terribly sorry for the delay. Was out of town in a part of the countryside where there wasn't any cellular connectivity and as soon as I got back on Sunday I had to head to the hospital where I was slammed with patients.

Let me see if I can answer all of your questions with a single story (the story of your patient since birth). This is of course only my theory, but I'm reasonably confident in it and it fits.

At birth, for reasons that we can't really ever know. A special series of cells in your patients developing brain failed to migrate from the spinal cord into the brain. This series of cells is instrumental in creating connections between different parts of the brain and also helps create the drainage pathways in the brain. It may not have been a failure of migration, it may have been bleeding in the brain at or before birth as well.

This set off a series of a series of reactions. The brain started having trouble dealing with the excess CSF and that pressure prevented several structures in the brain from developing fully (the brain is actually constantly growing and maturing well into your teens and some parts of the brain don't fully develop till your early 20's).

Once the brain reached whatever level of development it could reach.. the pressure problem never really went away. This does odd things to the tiny tiny blood vessels that snake through the center of the brain. They underwent a constant cycle of breakdown, bleeding and redevelopment... every tiny bleed tended to damage the sensitive neurons that had already developed. Over time.. the overall mass of the brain started reshaping itself to deal with the high pressures. By the time the shunt was placed.. the brain had probably already remolded itself. The shunt may have delayed degradation of the brain over time. But the degradation still happened. Now there are some very specific parts of the brain that get eaten away at first due to this ventriculomegally (pressure problem),,, these parts of the brain correspond to the control centers for things like bladder control, cognitive function, emotional lability etc. Thats why the constellation of symptoms you see in NPH (normal pressure hydrocephalus) is so specific.. the parts of the brain affected are right next to the ventricles.

So in a nutshell.. this has got to be something that started at birth and over time the brain compensated as best as it could while sacrificing mass and neuronal tissue in a limited fashion to remold itself to handle a high pressure ventricle. The downstream effect of this is now a situation that looks a heck of alot like NPH.

So I'd say this is NPH. But I wouldn't treat it the same way. I'm sort of surprised with your Neurosurgeon. If He/She thought that the shunt wasn't working.. then they should have made every effort to confirm that theory and take the shunt out. No sense in having a foreign object in your patient brain and skin that doesn't work. It's a nightmare infection-wise.

I'm glad your going to a neurologist. I don't really thing there is anything surgical or even structural that we can do to reverse this series of events. I DO think certain medications and physical therapy can give your patient a better quality of life though.

Again, I'm so sorry for the delay. You can always ask me as many questions as you want and I'll do my best to answer them in a timely manner.

Take care,

V

Above answer was peer-reviewed by : Dr. Chakravarthy Mazumdar
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