Stiff Person Syndrome and Anesthesia Case Report

By DR. M. Farhan Ashraf

Background
Case report describes the successful management of a rare and disabling neurologic disorder, the stiff person syndrome. The patient had a delayed emergence despite apparent full reversal of neuromuscular blockade.

Stiff Person Syndrome(SPS)

Stiff person syndrome (SPS) is a rare and disabling neurologic disorder characterized by muscle rigidity and episodic spasms that involve axial and limb musculature. SPS was recognized as a distinct entity in 1956 by Moersch and Woltman

Pathophysiology
An autoimmune disorder resulting in a malfunction of -amino butyric acid (GABA)mediated inhibitory networks in the central nervous system Anesthetic implications are less well described. Association of the disease with other autoimmune conditions such as diabetes and thyroiditis.

Case Report
A 62-yr-old woman (height, 1.70 m; weight, 61 kg) was scheduled for resection of a colon carcinoma. Her medical history revealed hypothyroidism, vitamin B12 deficiency, and SPS. Her medication at admission was prednisone 20 mg once a day, baclophen 12.5 mg twice a day (daily dose = 25 mg), diazepam 7.5 mg twice a day (daily dose = 15 mg), levothyroxine 25 g once a day, and vitamin B12 injections

Induction
Anesthesia was induced with propofol (2.5 mg/kg) and sufentanil (0.25 g/kg). After the administration of atracurium (0.6 mg/kg), the trachea was intubated. Anesthesia was continued with isoflurane (0.61.0 vol%) and oxygen/air for the duration of the procedure

Maintenance
Cefuroxime 1500 mg, clindamycin 600 mg, and dexamethasone 10 mg were administered IV. In the following 2 h, additional atracurium (35 mg), sufentanil (10 g), and morphine (8 mg) were administered

Reversal
At the end of the procedure, which was uneventful, neuromuscular monitoring showed four strong twitches Although the patient was responsive, she could not open her eyes, grasp with either hand, or generate tidal volumes beyond 200 mL

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Neostigmine 2 mg (0.03 mg/kg) and glycopyrrolate 0.2 mg did not alter the clinical signs of muscle weakness

Perioperative Management
The patient was sedated with propofol 5 mg kg1 h1 and further mechanically ventilated in the recovery room.

After 1 h, the sedation was stopped and mechanical ventilation was terminated. At that time, baclofen 12.5 mg was administered into the gastric tube. Two hours later she was in a good clinical condition, and her trachea was extubated.

Discussion
In our case, several drugs could have caused muscle weakness . Initially atracurium could be suspected. Pharmacokinetic analyses suggested that plasma concentrations were far less than therapeutic levels.

The same can be said for opioids, fentanyl, and morphine

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Because IV drugs can be excluded as causing muscular weakness, perhaps volatile anesthetics were the cause. In the case report by Johnson and Miller muscle weakness was observed only when baclofen was combined with inhaled desflurane or isoflurane.

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In addition, recent animal studies show that baclofen enhances volatile anesthetic-induced anesthesia

Conclusion
The complicated course of recovery was most due to the interaction between isoflurane and baclofen causing muscle weakness.
This case demonstrates a potential danger in combining baclofen with volatile anesthetics in patients with SPS

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