Uncomfortably Numb – the possible complications of anaesthesia and HSP/PLS

Is anaesthesia a problem for someone with HSP [hereditary Spastic Paraplegia]? This was a question I needed an answer to when I was told that I may need surgery for the spinal cyst I have. Blank looks, eye-rolls or shrugs were about the sum of it. So, I turned to Dr Google and found a few clinical papers but they were on medical websites I could not access or gave only the abstract. There were others available discussing the use of anaesthesia for other neurological patients, which although in the same bracket, didn’t give me the definitive answer.

I did however, garner facts that those with neuromuscular disorders, especially with degenerative or progressive conditions, can experience adverse reactions/complications to anaesthesia; some of which at the least could exacerbate neurological symptoms, but at worst be life-threatening.

Then, a few days ago, I received an email with the latest copy of Synapse (Volume 22, Issue 2 – Spring 2019) , the e-newsletter from the US-based Spastic Paraplegia Foundation. Most of it is US-centric, so I idly flipped through – but then something caught my eye, lo and behold, the article I had been searching for – ‘Surgical Anesthesia in HSP and PLS’ (Malin Dollinger, MD.) Dollinger is a medical doctor, who also has Hereditary Spastic Paraplegia (SPG4).

Dollinger’s first sentence summed up my own findings that there is a ‘frequent lack of awareness of special anesthesia requirements in patients with HSP and PLS’ [Primary Lateral Sclerosis] So what can we do to prevent any complications? Dollinger provides an extract from a paper authored by Franco Hernández, J.A., et al. ‘Use of Sugammadex in Strumpell-Lorrain disease: a report of two cases.’ He advises us to print it out and give a copy to our primary care providers, to our specialists and crucially to those who will care for us in the event of an operation – not just the day before but when we are assessed for our pre-op. Heck, I say give it to them all to keep on file – you know we have to be our own advocates! But what do we do in an emergency, when there is no time, or perhaps the ability, to talk, to explain – should we wear a wrist band? But what would it say that would be universally understood? That’s another question…or two.

So, after a description of the different types of anaesthesia (local/general/twilight sedation etc), Dollinger explains what occurred in cases where certain types of neuromuscular blocking drugs, including depolarizing and non-depolarizing muscle relaxants, were used in patients with HSP/PLS:

This topic became vitally important when people with HSP or PLS were given general anesthesia along with a long-acting muscle relaxant, such as succinylcholine, and unexpectedly became weak and unable to work their muscles for several days or even a few weeks! Persons with PLS were especially vulnerable in this situation because their condition often includes involvement of the nerves at the base of the brain which control swallowing and breathing, both of which could be affected by the long-acting muscle relaxants. “Life support” might be needed in that situation until the muscle relaxant wears off.

This is exactly what I was concerned about should I need elected – or god forbid – emergency surgery. It also made me wonder if this could be why I had reacted badly to anaesthesia in the past. Even though I have only recently been diagnosed with HSP, I imagine it has been quietly causing havoc in me since childhood. In retrospect, so many odd symptoms seem remarkably joined-up. After a general anaesthetic at 19, I took ages to fully come round and felt dreadful for weeks; then 8 years later my dentist attempted to extract my painfully impacted wisdom teeth using a local. I was terrified but joked to the lady in the waiting room, that if she saw me being wheeled out on a trolley, she’d know things had gone belly up. Little did I know that 20 minutes later, I would indeed be rolled out past her (her face rictus with fear and local anaesthetic) unable to draw breath, with an oxygen mask clamped firmly over my numb face and my legs shaking uncontrollably in an undignified impression of a vitus dance! Four hours later, after an EMG and several cups of hot tea, I was released from A&E with the diagnosis of a ‘panic attack’. Thank god, I have never had a ‘panic attack’ like it again! Round two of teeth extraction occurred under ‘twilight’ sedation; which felt more like ‘dark night of the soul’ sedation, as once again I took some coaxing to come round and felt like s**t for the next two weeks with dizziness and an inability to walk very far’. Makes you wonder…

Anyway, I digress. I have, thanks to this timely article, got my definitive answer – there is indeed a problem with anaesthesia for those of us with neuromuscular disorders BUT armed with this knowledge – and/or a knowledgeable anaesthetist – the problematic risks can be lowered to that of a person without the disease. It is reassuring that there is some advice and guidelines out there for us but sometimes finding it takes a bit of sheer luck – or serendipity!

Some people may not think twice about having an anaesthetic, but for me – and all those with neuromuscular conditions – Dr Dollinger’s article could literally be a lifesaver.


Read the article via the link – Dollinger, Malin, MD. ‘Surgical Anesthesia in HSP and PLS’, Synapse, Spring 2019, Vol 22, Issue 2, pgs 14-15 https://sp-foundation.org/news-resources/newsletter.html accessed 18/05/2019

Franco Hernández, J.A., et al. Use of Sugammadex in Strumpell-Lorrain disease: a report of two cases. Revista Brasileira de Anestesiologia. Vol.63 No.1 Campinas Jan/Feb 2013 https://www.ncbi.nlm.nih.gov/pubmed/24565095

If you want to know more about HSP and PLS and/or would like to donate to help research, or you are a sufferer and would like to know more about research and living with HSP/PLS, the SPF has a massive resource section – you can find them here https://sp-foundation.org/

Drowning in my diagnoses…part one

An ill-fitting box

‘…I put the word “diagnosis” in quotes because I have not yet seen that case in which a “diagnosis” led to a “cure”, or in fact to any outcome other than a confirmed, and therefore an enforced, debility.’ – Joan Didion, Blue Nights.

I spent decades wanting a diagnosis, a meaning, a reason for the constellation of symptoms that haunted my life. I traipsed back and forth to the doctors with increasing anxiety, so much so that they diagnosed me with increasing anxiety. I couldn’t make them see what I knew, what I had known for years – there was SOMETHING WRONG WITH ME. But I didn’t fit nicely into any of their boxes except one marked ‘neurotic’. A battered and worn box that had previously held many other women, with many other random symptoms that would one day manifest as something bigger, and we’d need a bigger box. So, I took up residence in the box marked ‘neurotic’ and was passed around grudgingly, each new consultant, specialist or worn out registrar would lift the lid, take one look and mark me unremarkable…just neurotic. I hate to bang the feminist drum, but women have been dealt this hand for centuries – it would be unremarkable, if it weren’t for the reason that they (doctors) were, and are often wrong.

My symptoms were seemingly random but now that I have my (two) rare diagnoses, I can see the pattern. I say I can see the pattern – I could always see the pattern, it just didn’t make sense to me – it was a Rorschach test inked into my DNA. So, I had sleep studies and brain scans and blood tests. I had electrodes glued to my scalp and seizures induced. But still no more than an eyebrow was (reluctantly) raised; my seizure was not a good enough seizure – ‘we don’t treat those ones now’ the bored neurologist said. I was put back in the box.

I wasn’t comfortable in the box; my lower back and cervical spine had been causing me pain for far too long, as had the ‘electric currents’ that shot through my thighs. My hearing was going, the tinnitus whooshed through my head; I imagined neurons lighting up like a pinball machine. Then one day, half way across the world in a tropical paradise, I noticed my left calf was disappearing – my highly toned legs looked decidedly wonky. I was duly sent to another specialist.

He sat imperiously behind the desk; a medical student banished to the back of the room, deliberately diminished by his mentor’s imperiousness. He asked for my medical history…back pain (immaterial), neck pain (irrelevant), my mum’s MS (slightly arched eyebrow), moderate deafness – he leans forward like a predatory hawk – ‘you don’t seem very deaf’, he says dismissively. I blink and carry on…depression… ‘Ah!’ he says, ‘Ah!’ says the medical student – they lock eyes knowingly and I crawl back in the box; the one marked ‘neurotic’. He says has no explanation for the disappearing calf muscle except that I must have had a crush injury at some point. I tell him I have no recollection of a crush injury of that leg at any point. He says I must have had a crush injury. The MRI shows some form of focal irritation of my gastrocnemius. No doubt from the crush injury I must have had.

Fast forward a year and I am sick of the pain and stiffness that permeates my body. Having recently won the divorce lottery, I treat myself to a full body MRI scan. The results are fairly boring except for an ‘incidental finding of a large Tarlov cyst’ on my sacrum and a ‘lumbosacral transitional vertebrae’. I do what any self-respecting researcher does and Google them both. They seem perfect candidates for my constellation of symptoms…apart from the deafness.

I now feel as if I am at last beginning to see something in that Rorschach test, and it looks remarkably like a sacrum… to be continued