Surgical Anesthesia in HSP and PLS
By Malin Dollinger, M.D., SPG4
Recent chatroom discussions have uncovered a frequent lack of awareness of special anesthesia requirements in patients with HSP and PLS. This discussion is to set down medical information and comments about anesthesia and to offer references in the anesthesia literature that you can download and give to your anesthesiologist. Anesthesiology is a complex science, not just “putting someone to sleep for a few hours,” and a lot of research has been done and is continuing.
First, a brief classification of types of anesthesia: General anesthesia means you are “sound asleep” and unaware of anything, including what would otherwise be painful such as surgical incisions and cutting. In the “old days” this was a lengthy process, for example using open drop ether (used when I was six years old and underwent surgery). Later, things like sodium pentothal and lately, very effective agents such as Propofol are being used where you both “go to sleep” and “wake up again” in a very short time span. General anesthesia is the form of anesthesia which especially concerns us, not because of the anesthetic agent, but with the muscle relaxants commonly also used. Regional anesthesia will anesthetize only a part of the body, for example a spinal anesthetic for surgery on the legs, e.g. a hernia, local anesthesia for smaller areas, using the same type of anesthetic as the dentist, and other types of regional anesthesia, where larger nerves, e.g. in the chest, are numbed by local injection of anesthetic agents. Then there is “conscious sedation,” also called “twilight sleep,” where you are given intravenous drugs that make you unaware of your surroundings, but you can follow simple directions. It was often used for GI procedures such as colonoscopy, but nowadays Propofol is often used, since it works so fast, so well, and wears off so quickly without major side effects such as nausea and vomiting, both of which are problems with older anesthetics.
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. Other topics regarding surgery in people with HSP or PLS were offshoots of the above discussion. For example, how to improve leg muscle rigidity and tightness which might interfere with the planned surgery. A Synapse reader told me about receiving Botox injections in her leg muscles, a splendid solution to enable proper surgical positioning without the "tight legs” getting in the way. I once had prostate surgery and they couldn’t hold my legs apart. Finally, a nurse on each side held each leg outwards during the entire operation.
Now here are some details for you, and especially for your anesthesiologist, with some references you can download, print and give to her or him. These “pearls of wisdom” are from scientific/medical articles mostly written by anesthesiologists who deal with such matters every day. That’s the purpose of publishing medical research, so other physicians and their patients have the benefit of new and important advances in medical care. Suppose a doctor discovered a new antibiotic that worked against antibiotic-resistant bacteria. Wouldn’t it be vital, and sometimes life-saving for her to publish that information so we could all benefit? So here we are, about to discover and review what the anesthesiology researchers have to say about giving anesthesia to persons with PLS and HSP.
The following section is intended to be printed and given to your anesthesiologist at least several days before surgery and not the night before or the morning of surgery. I’ve quoted exact excerpts from some of the scientific articles and have not tried to edit out or translate the “doctorese.” Your anesthesiologist needs to hear the important message from the anesthesiologist who wrote the article using the correct medical language. These are for your direct benefit, thus better if exactly quoted. Print the following, including the references, for your anesthesiologist.
From the paper authored by J.A. Franco-Hernández, et al. (see reference 3), the use of neuromuscular blockers is complicated in patients with familial spastic paraplegia. Succinylcholine is contraindicated since it may induce hyperkalemia, and there should be caution in the use of non-depolarizing muscle relaxants due to the risk of exaggerated muscle relaxation. The literature sources have not shown whether regional anesthesia exacerbates the neurological symptoms. However, regional anesthesia is not always possible. Therefore, general anesthesia with non-depolarizing neuromuscular blockers would represent a safe alternative - particularly considering that there are drugs offering rapid and safe reversal of muscle block induced by rocuronium and vecuronium. In our two patients we chose general anesthesia because of the duration and complexity of both operations.
Great care is required at extubation in patients with FSP, particularly if neuromuscular blockers were administered during the operation. If possible, long-acting neuromuscular blockers should be avoided, with routine monitoring of neuromuscular relaxation throughout the operation, using a standard peripheral nerve stimulator. A TOF ratio of over 0.9 must be confirmed before awakening, accelerating patient recovery with neostigmine or drugs that selectively bind aminosteroid neuromuscular blockers, such as sugammadex.
Although the anesthetic management differed between the two operations (Total Intravenous anesthesia versus inhalatory anesthesia), agreement existed regarding the choice of muscle relaxant, i.e., rocuronium, due to the possibility of antagonizing its effects with sugammadex. Functional deficiencies being similar to those prior to general anesthesia, subsequent follow-up of both patients revealed no significant worsening of neurological signs.
Conclusion: The main interest of our study is that both patients were subjected to general anesthesia, with the use of a specific reversal agent for non-depolarizing neuromuscular block, followed by complete recovery and no worsening of the existing neurological disease.
Thomas, et al reported use of spinal anesthesia, in connection with an obstetric procedure, and spinal anesthesia worked well and normally.
Dizdarevic and Fernandes used a paravertebral nerve block and multimodal [several types] anesthesia for breast cancer surgery in a PLS patient. The anesthesia was reliable and effective, she had Little or no pain postoperatively, and received no opioid pain medication.
Olmez and Topaloglu reviewed various reported types of anesthesia in HSP patients.
Kunisawa et al used nitrous oxide, oxygen and sevoflurane anesthesia in an HSP patient, and there were no anesthesia problems (article in Japanese)
References discussed:
1. Thomas, I., et al. Spinal Anaesthesia in a Patient with Hereditary Spastic Paraplegia: case report and literature review. International Journal of Obstetric Anesthesia. Vol 15: Issue 3, 254-256, 2006
2. Dizdarevic, A and Fernandes, A. Thoracic Paravertebral Block, Multimodal Analgesia, and Monitored Anesthesia Care for Breast Cancer Surgery in Primary Lateral Sclerosis. Case Report in Anesthesiology. Vol 2016, Article ID 6301358, 4 pages. http://dx.doi. org/10.1155/2016/6301358
3. 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
4. Olmez, A. M.D. and Topaloglu, H. M.D., Hereditary Spastic Paraplegia / Anesthesia Post 11.01.2012
http://www.medmerits.com/index.php/article/ hereditary_spastic_paraplegia/P13
5. Kunisawa, T., et al. Anesthetic Management of a patient with Hereditary Spastic Paraplegia. Masui 2002;51:64-66.
Your anesthesia may be local, regional, or general, but in any event, a knowledgeable and expert anesthesiologist can create the same low risk of anesthesia as in people without HSP or PLS. Examples have been given, and especially important is the avoidance of the use of long-acting neuromuscular blockers, such as succinylcholine. There are other special measures for HSP and PLS patients, such as ways to “line up” crooked broken bones so they will heal properly using traction and Botox injections for a leg or arm fracture or using local Botox injections where a part of the body needs the spasticity reduced for the surgery.
I suggest you copy this entire article to give to your anesthesiologist [or surgeon for transfer to your anesthesiologist] at least several days prior to your surgery. Of course, you will undergo the usual preoperative screening for everyone undertaking surgery, including checking for heart or lung disease, blood clotting abnormalities or any other medical issues that need to be known and dealt with to make the contemplated surgery as safe as possible.
