From Migraine, to CSF Leak, to Brain Sagging – My journey

How did I get from having a severe migraine to a CSF leak which then caused brain sagging and intracranial hypotension?! I do my best to explain it all, along with all the technicalities.

If you prefer to watch a video version of this blog post then you can right here on my YouTube channel or just scroll through to read on:

Before I begin, in my previous post I explained in detail how my head pain started, what treatment I was given and how I was diagnosed with status migrainosus after my MRI brain didn’t show anything significant other than one line that kept bothering me (which I promised to speak about in this post and I will but that’s towards the end) – and that line is:

“mild pachymeningeal enhancement over the cerebral hemispheres is of unclear significance.”

If you’d like to read that post first (if you haven’t already) then you can here:

• The Never-Ending Head Pain: Status Migrainosus

Like I said, I’m going to go into details so it’s best I put a table of contents in case you wish to navigate through faster although you then may miss out on relevant information but I’ll leave that up to you.

Now continuing further, it was…

Day 39

I was admitted to the hospital for the second time.

My primary doctor was keen on me getting a lumbar puncture (also known as a spinal tap) right away and I was, by then mentally prepared for it too knowing that every other test had been done and this really was the only one left.

But I was scared because as someone with Ehlers-Danlos, local anaesthesia doesn’t work too well on me. So my preparation was not for what the result could be, but it was more to prepare me to endure more pain than is usually expected.

At some point I will write about this whole EDS and local anaesthesia issue but for now, I carry on with this story.

Relevant Read:

• My Journey: Being Diagnosed with Ehlers-Danlos Syndrome
• My Experience: Treatments to Manage Ehlers-Danlos Syndrome

What is a Lumbar Puncture / Spinal Tap

As the word suggests, a lumbar puncture is carried out in the lumbar region. A needle is inserted in the lumbar between two vertebrae to collect CSF – cerebrospinal fluid. This fluid is then tested for infections and possible medical issues. The pressure is also checked.

But to explain it better, here is how it’s done. As explained by Healthline.com:

“They’ll likely position you on your side. They’ll clean your back with an antiseptic solution to reduce your risk of infection and numb it with a local anesthetic. They’ll inject a hollow needle into your subarachnoid space to collect a sample of your CSF. You may feel some pressure at this point, but the procedure usually isn’t painful. After they remove the needle, they’ll clean and bandage the puncture site.”

Lumbar Puncture Number 1

It was late evening, the anaesthesiologist and a nurse – along with my primary doctor’s assistant and the neurologist came with their setup to carry out my lumbar puncture in my room.

The nurse struggled to find a vein for my IV line (another issue I should share some day soon), so the anaesthesiologist found one – an awkward one, but it was one I was happy with.

I warned him about my whole local anaesthesia issue but I got the feeling that he didn’t quite believe me… well he tried to. He gave me a double dose of local anaesthesia in the first shot and proceeded to push in the lumbar puncture needle.

I screamed – multiple times. Clearly it wasn’t enough, I mean the anaesthesia wasn’t enough.

Over the course of the procedure, I had six times the usual amount given to ‘usual’ patients.
Umm just to give some context, Medicinenet.com say:

“A lumbar puncture is usually not painful, as a patient is first given a local anesthetic. Most patients feel nothing except for the mild sting of the local anesthetic needle. It is possible to feel a pressure sensation as the needle goes in.”

I chuckle at such statements but it seemed like this pain was the least of my issues.

As I lay on my side, curled up, the lumbar puncture needle reached its destination but the cerebrospinal fluid wasn’t coming out and then it trickled but that too wasn’t enough.

I tried to relax, breathe slowly and ask my body to just get through this as my tears rolled down my face and into my ear (I was lying on my side, curled up, knees to chest, hugging my legs).

The anaesthesiologist suggested that we do this tomorrow with me sitting up in the hope that gravity will help the fluid come down better. But tomorrow?! I didn’t want to do this tomorrow – I wanted it over right away.

So I suggested that if we could get the hospital bed I was on to move up a bit, for it to incline then it should help me sit up with that lumbar puncture needle still in my back.

We did that and the flow improved, but still, a collection of fluid that usually takes 15 minutes, took us 90 minutes.

That in itself told us something was wrong. The anaesthesiologist and the neurologist discussed the need for a new brain MRI to be done.

By then, my back got patched up and I was given an ice pack for the soreness of my back which took two weeks to go.

My CSF was tested and it showed abnormal levels of protein. The range was: 15 – 45 mg/dL and I was at: 403.4 mg/dL.

This alarmed the doctors and confirmed that there was definitely something wrong in the central nervous system.

Day 40

MRI Brain No. 2

There was no way I could manage a MRI brain without being sedated, especially because of my claustrophobia issues, the severe head pain and now this back soreness. For that, I needed to be on a 6-hour fast after which I was sedated, the MRI was done and I was back in my room after being told that there were possible issues found but we had to wait for the final report.

O’ FYI I didn’t always have claustrophobia issues in MRI machines – if interested, you can read how that happened here:

• MRI & Claustrophobia. An Error That Changed My Approach

Day 41

The final report was now in and boy did it look very different to my MRI that was done on day 5!

Here’s what it said:

“Thin bilateral subdural collections and thick pachymeningeal enhancement over the cerebral and cerebellar hemispheres, flax, tentorium. Exaggerated enhancement is also present along the clivus with distention and convexity of the transverse sinuses.

In the spine, there is prominence of the posterior epidural space and veins with enhancement. Thin epidural fluid collection is noted in the cervical spine along the lateral margins of the thecal sac. Imaging findings are suggestive of intracranial hypotension.

Mild bilateral uncal herniation without mass-effect on the brain stem or effacement of basal cisterns.
Pachymeningeal enhancement has increased since the previous MRI study dated 19/3/2021 with the collections being new findings”

Phew!

Now once this was out, my primary doctor, neurologist and a neuro-surgeon came to meet me. All of them, in their own way explained that there is a possible cerebrospinal fluid leak which has caused the brain to drop – or to put it nicely – the brain has sagged causing swelling and pain which is known as intracranial hypotension.

They now wanted to identify where the leak was.

And guess what – I needed a new lumbar puncture for that!

But before I get onto the whys and hows of it…

What is intracranial hypotension?

I’m not qualified to explain this properly, but from what I was told by my doctors, this explanation from Columbiaspine.org fits the best:

“Spontaneous intracranial hypotension is a condition in which the fluid pressure inside the skull is lower than normal.The brain and spinal cord are covered by a tough, watertight membrane called the dura. Inside the dura is the cerebrospinal fluid (CSF), a liquid that bathes and cushions the brain and spinal cord. Normally, the CSF circulates inside the dura, gradually drains, and is constantly replenished with new fluid. But a leak in the dura can allow too much CSF to escape too quickly. This reduces the amount of CSF in circulation, reducing the fluid pressure and causing intracranial hypotension.”

Day 43

The previous day was a Sunday, so the second LP had to wait for this day plus it gave my previous lumbar puncture pain an extra day to heal – not that it made much of a difference.

But this was my worst nightmare coming true – another lumbar puncture in the same area with the local anaesthetic not working for me – but what added to my woes was for this lumbar puncture, I’d be fasting too.

Please remember, I am still having horrendous headaches – I can’t sit up properly without feeling like my neck is going to snap and now I had lumbar soreness too! Ooo and like I said, I’m fasting. Hunger = more head pain.

Lumbar Puncture No 2 with CT Scan

This lumbar puncture was different yet similar to the first one.

What was similar: I would be in the same position as the first one. The local anaesthetic wasn’t going to work so I would need more than usual. The anaesthesiologist would draw out some CSF to make sure she had got the right area and and… I was going to scream in pain.

What was different: I was fasting, because this time the anaesthesiologist needed to inject a contrast dye after she made sure she had reached the CSF. They were then going to put me in the Trendelenburg position (head down tilt) for 30 minutes to make sure the contrast dye gets to the brain, after which a CT scan would be done in the hope the dye would leak out somewhere and the imaging would catch it.

And that’s how it all went. I’ll spare you the details of my screaming and how ugly painful it was but, it really was bad.

I was happy to finally be back in my room, eating.

Day 44

The decision making day

My lumbar puncture CT Scan results were in and here’s what the report said:

“Anterior epidural extravasation of contrast in the cervical spine which is maximum at C6-C7 levels with extension laterally along the neural foramina paravertebral soft tissue. There are posterior osteophytes-disc complex at C6-C7 levels indenting the thecal sac. Circumferential epidural extravasation of contrast is present in the thoracic spine with calcification of the posterior annulus at T4-5 and T10-11. The exact site of CSF leak is unclear in view of long segment of extravasation, but appears to be at C6-C7-T1 levels in view of maximum concentration of anterior localisation of contrast with presence of posterior osteophytes.”

Bright and prompt, my neurosurgeon walked into my room and in his big booming voice explained these findings.

There appeared to be a bone spur in my cervical region (C6-C7) which was protruding and had possibly caused a tear in the dura (the dura is the “outermost of the three layers of membrane called the meninges that protect the central nervous system”).

This seemed to be the most likely site of the CSF leak, the reason for my intracranial hypotension.

The surgeon now gave me two options – one conservative but tried and tested, the other, invasive.

Option 1 – Conservative: the epidural blood patch is the standard surgical procedure done to plug a CSF leak. Under general anaesthetic, fresh blood would be drawn from my arm and using an epidural needle and with the guidance of an x-ray machine, the blood would be injected at the region where the leak is suspected. The blood then travels to the leak, begins to clot and patches the hole. This then helps the dura to heal.

Once the surgery is over, I would (again) be kept in the Trendelenburg position (head down tilt) for 30 minutes before I come out of anaesthesia and be taken to the recovery room.

The first month of recovery would be strict bedrest and then my symptoms would tell us how it’s all going.

Option 2 – Invasive: a cut would be made from the front of my throat. Going past the voice box, the surgeon would get to the cervical bone spur, shave it and patch the exact location of the leak. This patch would be accurate, rather than injecting blood in the hope the blood clots and patches at the right place as is the case with option 1. The risks are plenty with this type of surgery plus a much much much longer recovery time.

Although the decision was left to me, all three of my doctors believed that we must try the conservative approach first and if it doesn’t work then we can look at option 2.

Option 1 came with a 70% success rate which I was reassured was very high!

So I guess it was option number 1 then.

The doctors didn’t want to waste any time, they wanted the surgery done the very next day. I didn’t understand the seriousness of it, until much later.

Day 45

Surgery Day

At 4.45am I had breakfast and went back to sleep. I needed at least 7 hours fasting and with my surgery scheduled for noon, I had to have such an early breakfast which felt more like a midnight snack.

At 10am I was asked to apply some sanitising liquid at the end of my bath – these were peak Covid times in Mumbai that was dealing with its second wave so precautions were plenty.

By noon I was at the pre-surgery waiting area (which doubles up as a recovery room post-surgery). This is when my IV line came out and out came the anaesthesiologist who chose not to believe me.

He wanted to put a new IV line in my hand since that’s where my veins were most obviously visible to him – thin EDS skin helps that way but how it doesn’t help is that it reacts very fast. The skin on my hands are super sensitive, it cannot take an IV line. But he did it anyway.

I was taken into the operating room. I saw the huge TV screen – the screen that will guide them with where to inject the blood. I felt calm about it – we were getting closer to fixing my pain. As the general anaesthetic was injected – I screamed because that IV line still hurt but I passed out within seconds.

I woke up post surgery and post the 30 minutes of the head-down tilt screaming – just as I had done so while passing out.

Why was I screaming? Did the neck hurt so much?!

Frankly, the neck pain was the second most painful thing in that moment, the first was that IV line – trust me, it killed. I was also cold.

I shivered away as a warm air pipe was placed under my blanket. This is when I suddenly realised that I now face a new challenge. The challenge of getting better.

That first night I struggled more than I had in any of the three previous surgeries I’d had in my life. This is also when I realised that surgery related to the central nervous system, especially when your main issue was felt in the head was no joke.

The volatility of the area and the importance of following instructions was important.

I had to lie flat – no left, no right and no pillow. To stop myself from turning, I kept pillows on either side of my head.

But here’s where it went wrong. I needed to take my oral medication. For that the nurse inclined the bed a bit so that I could swallow and drink water. I had moved my head half a millimetre off the pillow to put the medicines in my mouth when the worst pain ever struck.

I screamed and cried and felt like my whole neck and head was being electrocuted. I thought I was done – I had no strength in me but I had to calm the pain, but it continued and so I continued to scream and cry. It took extra painkillers and 45 minutes for this episode to settle and 1 second for me to learn my lesson: NO MOVING THE HEAD AT ALL!

That night I also went through a panic attack. The fact that I couldn’t move, I could not turn left or right, it was dark – all made me feel suffocated and trapped.

This is where the nurse and a friend of mine helped me through – they got the lights on and got me a wet towel like I’d asked for so that I could breathe into something that made me feel like I was getting fresh air. I mean come to think of it, during these 45 days, I didn’t really have a lot of days when I could step out for fresh air and here I was on the 15th floor of the hospital where the windows don’t open (for obvious reasons).

Thankfully I settled and slept.

Day 46

My neurosurgeon came to check up on me the next morning. He gave me confidence that the surgery went well and that I now need to follow the instructions and be positive! 😀

Thankfully I was allowed to turn to the left and right – but not turn my head, instead turn the whole body left or right. It’s called a log-roll.

I was expected to have a high in-take of fluids as I had been doing my entire time but peeing had to be done in a bed-pan for the first few days post surgery.

Around early evening that day, the pain in my IV line kept getting worse. I had to keep an ice pack on it to save me from screaming, but the pain was unrelenting. My IV line was now blocked and eventually a new one was put – again, at my wrist where I knew I wouldn’t be comfortable. Within 12 hours this too got blocked and had to be removed. It was decided that I can do without it as long as I maintain a high in-take of fluids and take my medication orally.

Days 47 – 49

I spent the next three days recovering to the point that I could make it to the bathroom and back. It was tough, because four things weren’t allowed: bending, lifting, twisting and straining (BLTS) but I had to avoid them if I was to feel better.

I was happy to be going home – finally. Home to my mum, pup and very importantly, my bed.

Before I left, my primary doctor came to see me. Now that the situation was better and we were both more relaxed, I asked her for her honest opinion of whether I dodged a bullet and how bad did my situation actually get?!

She was upfront enough to tell me that I got very close to things getting ugly. If I had gotten admitted even two days later, my brain would’ve been close to putting me in a coma.

Was I surprised? I wasn’t, because I knew how bad I felt and by then I had read enough about my situation.

But yes, it did scare me, it did tear me up but I felt extremely grateful that my doctors were brilliant. I had all the support and the best facilities and minds available to me and like I said earlier, it was all during a time when Mumbai was struggling with it’s second wave of Covid-19.

She left giving me the one piece of crucial advice that I had heard every single day that I was in hospital – drink 2-3 litres of water every day. The brain needs fluids for it to float again and I desperately needed that replenishment.

I end now with an important question…

Could the doctors have prevented my situation from getting so bad?

Possibly yes. I go back to my first brain MRI done on day 5, back to that one sentence that stayed with me:

“mild pachymeningeal enhancement over the cerebral hemispheres is of unclear significance.”

If we look up “mild pachymenigeal enhancement”, you get information from radiopaedia.org, which says this :

“Pachymeningeal enhancement, also known as dura-arachnoid enhancement 4, refers to a dural and outer layer of arachnoid pattern of enhancement seen following contrast administration and may occur in the conditions listed below:

Infection, intracranial tumour – metastases (meningeal carcinomatosis-pachymeningeal carcinomatosis), intracranial hypotension, postoperative states, idiopathic pachymeningitis, cerebral venous thrombosis, neurosarcoidosis, extramedullary haematopoiesis, rheumatoid arthritis, granulomatosis with polyangiitis”

When I had read this 15 days into my migraine, the word ‘intracranial hypotension’ caught my eye simply because I have POTS – Postural Orthostatic Tachycardia Syndrome which can cause my blood pressure to drop which then linked with the word ‘hypotension’ – so this was me possibly overthinking but I was desperately looking for answers to my never-ending migraine.

I clicked on ‘Intracranial Hypotension’ believing it’ll lead to just another dead end. Here’s what showed up:

“Intracranial hypotension, also known as craniospinal hypotension is defined as cerebrospinal fluid (CSF) pressure <6 cm H2O in patients with clinical presentation compatible with intracranial hypotension, namely, postural headache, nausea, vomiting, neck pain, visual and hearing disturbances, and vertigo 17. It most commonly results from a CSF leak somewhere along the neuraxis.”

“It is also more commonly seen in connective tissue disorders, including Marfan syndrome, Ehlers-Danlos syndrome (type II), and autosomal dominant polycystic kidney disease (ADPKD).”

There it was Ehlers-Danlos syndrome finding me here too. And later when I got to know that a bone spur caused the tear for there to be a leak, I was told that a bone spur is common with those who have EDS.

So yes, a simple Google search told me so much, but there wasn’t enough evidence clinically for the doctors early on. To be fair, I was offered a lumbar puncture the first time I was admitted but fearing the pain and my MRI being pretty ‘normal’ it wasn’t insisted upon and I completely agree with how the doctors went about everything. In that sense, no complaints.

But yes, in theory, I could’ve suffered less. It didn’t have to get this close.

What worked for me was advocating for myself and having good doctors. We were all persistent and curious. We were working as a team to help me.

Again, I could not have gotten through this without my family, friends and knowing that my pup, Toby misses me and that I needed to get home for all of them.

As I type this post it’s been 6 weeks since my surgery – how am I doing now? Well for that, watch out for my next blog post which will come out once I complete my 8 weeks.

Until then, I hope my story helps someone out there.

Ooo just before I go, I thanked my family and friends in the first part of this blog post, but I cannot end this blog post without thanking the amazing Sheryl, Claire and Katie – the ladies who kept encouraging me while I hurt so much. Thank you sooo much – you’re wonderful human beings.

Take care 🙂

Relevant Read:

• My Acne Skincare Remedies – Endometriosis & Ehlers-Danlos Syndrome
• My Diet for Endometriosis & Ehlers-Danlos Syndrome

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Disclaimer: I am not a medical professional. I am a patient and have created this platform to share my experiences. This is all purely informative and in no way am I providing medical advice. Please consult a medical professional.