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Due to the rare nature of the disease, no specific technique has been recommended. The decision to employ either general anesthesia or regional block has to be made based on the type of surgery, involved anatomy, disease severity, patient preference, and comfort level of the participating anesthesiologist.1
Total intravenous anesthesia has been reported to be affective. Regional anesthesia could be advantageous by providing effective and deep analgesia without necessitating the use of muscle relaxants and inhalation agents.
Challenges to spinal block include difficulty locating anatomical landmarks, limitations to proper positioning, and a chance of painful spasms and rigidity in response to the needle insertion. Presence of an intrathecal pump may necessitate a fluoroscopic guided neuraxial procedure.
It is crucial that the anesthesiologist be informed of the medications and dosages you are taking. The various forms of anesthesia drugs often fall in the GABAergic category. When combined with your daily medications, they can cause a fatal overdose and severe hypotonia.
There are no contraindications to any anesthetic agents or procedures. But due to the often high levels of muscle relaxers, patients should be monitored for hypotonia and the need for supportive ventilation.
Many anesthestic agents involve blockade of GABA receptors at various sites. Both inhalation agents and intravenous agents have the potential to cause hypotonia by causing GABA antagonism. They can increase the proportion of densitized neuromuscular receptors and can result in a non-competitive blockade and prolonged duration of action. The depth of neuromuscular blockade should be closely monitored and the muscle paralysis must be adequately reversed, keeping in mind there may be no correlation between depth of muscle paralysis and the hypotonia observed in several case reports.
There is no direct effect of SPS or its therapy on the neuromuscular junction, therefor it should not affect the actions of nondepolarizing agents.
Patients may be on any combination of benzodiazepines, Baclofen, Gabapentin, Vigabatrin, Venlafaxin, plasmapheresis, IVIG, or high-dose corticosteroids.
It is important to cover with steroid prophylaxis to supplement for the possible cortisone suppression. Diazepam and Baclofen therapy should be continued during the perioperative period. Discontinuation could cause severe withdrawal reaction. The anesthetist should be aware of and prepared to respond to such a reaction. The patient may need supplemental steroid therapy if they are on long-term steroid medication.
Skeletal muscle relaxants should be avoided as they can potentiate hypotonia. Use of muscle relaxants needs to be monitored and small doses of short acting relaxant should be titrated.
Though there is little research, case reports document resistance to succinyl choline.
If the patient has kyphosis, hyperlordosis, or contracture, care must be taken with positioning. The patient should be positioned with the appropriate aids or pillows.
The patients have a startle reaction to voluntary movement, emotional upset, or unexpected auditory, tactile, or somatic stimuli. Especially when prone, the should be well supported for rigidity and sudden spasms.
In addition to routine monitoring of blood pressure, oxygen saturation, ECG and endtidal CO2, the anesthetist should monitor for neuromuscular paralysis. There is potential for respiratory insufficiency due to muscular rigidity.
Bispectral index should be kept between 40 and 60, indicating the depth of anesthesia.
Although no association with malignant hyperthermia is suspected, it allows you to differentiate the possible complications including Baclofen withdrawal and Nerolept malignant syndrome.
1. Anesthesia recommendations for patients suffering from stiff-man syndrome. (Full file) Orphan anesthesia.net.
2. Sidransky MA, Tran NV, Kaye AD. Anesthesia considerations in stiff-person syndrome. Middle East.J. Of Anesthesiol;2013 Jun; 22(2):217-21. (Full File)
3 Bouw J, Leendertse K, Tijssen MA, Dzoljic M. Stiff person syndrome and anesthesia: case report. Anesth Analg. 2003 Aug;97(2):486-7 Link to article.
This case report describes the successful perioperative management of a patient with a rare and disabling neurologic disorder, the stiff person syndrome. The patient had a delayed emergence despite apparent full reversal of neuromuscular blockade. We suggest an interaction between the GABAergic effects of baclofen and volatile anesthetics as a possible cause.
4 Johnson JO, Miller, KA. Anesthetic Implications in Stiff-Person Syndrome. Anesthesia & Analgesia. March 1995 - Volume 80 - Issue 3 - pp 612-613. (Full File)
5. Neubert L, Green P, Green MS. Anesthetic care of stiff person syndrome in the outpatient setting. (Full File)
6. Qin X, Wang Dx, Wu XM. Anesthetic management of a patient with stiff-person syndrome and thymoma: a case report. Chin Med J (Engl). 2006 Jun 5;119(11):963-5.
7. Yagana O, Özyilmaza K, Özmadena A, Sayinb O, Hanci V. Anesthesia in a patient with stiff person syndrome.
DOI: 10.1016/j.bjane.2013.02.004 Link to article.
8. Shanthanna H. Stiff Man Syndrome and Anaesthetic Considerations: Successful Management Using Combined Spinal Epidural Anaesthesia. J Anaesthesiol Clin Pharmacol. 2010 Oct-Dec; 26(4): 547–548. (Full File)
9. Toscano FV, Vick AK, Shay HH, Delphin ES. Total intravenous anesthesia (TIVA) for stiff-person syndrome. Open Journal of Anesthesiology, 2012. Published Online September 2012 (http://www.SciRP.org/journal/ojanes)
11. 8. Gust R, Böhrer H. Stiff-man syndrome associated with continuous sufentanil administration. Anaesthesia. 1995 Jun;50(6):575. Link to article.
12. Gros AJ Jr, Thomas LC, McKinley KL, Van Gerpen JA. Prevention of an acute severe exacerbation of Stiff-person syndrome during surgery. Anesthesiology. 2006 Apr;104(4):885-6. Link to article.
13. Ledowski T, Russell P. Anaesthesia for stiff person syndrome: successful use of total intravenous anaesthesia. Anaesthesia. 2006 Jul;61(7):725. Link to article.
ANESTHESIA & SURGERY