Secondary Logo

Journal Logo

Two Cases of Duchenne Muscular Dystrophy That Showed Different Reactions to Nerve Stimulation During Peripheral Nerve Block: A Case Report

So, MinHye MD; Sugiura, Takeshi MD, PhD; Yoshizawa, Saya MD; Sobue, Kazuya MD, PhD

doi: 10.1213/XAA.0000000000000524
Case Reports: Case Report
Free

In recent years, the technique of combined ultrasound and electrical stimulation-guided nerve block has been recommended. We present 2 patients with Duchenne muscular dystrophy who exhibited different muscle responses to nerve stimulation during the performance of peripheral nerve blocks for surgeries. Whereas a 2-year-old boy without severe disability showed the expected muscle contraction to electrical nerve stimulation, a 14-year-old boy with severe disability showed no muscle response. Our experience suggests that muscle responses to electrical nerve stimulation will vary with the stage of Duchenne muscular dystrophy.

From the Department of Anesthesiology and Intensive Care Medicine, Nagoya City University Graduate School of Medical Sciences, Mizuho-cho, Mizuho-ku, Nagoya, Japan.

Accepted for publication January 26, 2017.

Funding: None.

The authors declare no conflicts of interest.

Address correspondence to MinHye So, MD, Department of Anesthesiology and Intensive Care Medicine, Nagoya City University Graduate School of Medical Sciences, 1 Kawasumi, Mizuho-cho, Mizuho-ku, Nagoya 467–8601, Japan. Address e-mail to mine@snow.ocn.ne.jp.

Ultrasound-guided peripheral nerve block has been used widely in both adult and pediatric practice in recent years. For safety and success of the procedure, the dual guidance method of combined ultrasound and electrical nerve stimulation has been recommended.1,2

Regional anesthesia including peripheral nerve block is useful for obtaining perioperative analgesia in patients with muscular diseases. At the same time, a significant reduction in the muscle compound action potentials has been reported in patients with muscular disease.3 We present 2 cases with Duchenne muscular dystrophy (DMD) in which different responses to electrical nerve stimulation were obtained during performance of peripheral nerve block for elective surgery. The parents of each child provided written permission for publication of this case report.

Back to Top | Article Outline

CASE DESCRIPTIONS

Patient 1

A 2-year-old boy presenting with elevated serum concentration of creatine kinase (CK) was scheduled to undergo right biceps brachii muscle biopsy for suspected DMD. He weighed 12.7 kg and was 83 cm tall. Although his serum CK level was elevated to 15,467 U/L (normal range 62–287 U/L), he did not show any muscle weakness. Serum electrolyte concentrations were normal. Whereas computed tomography image showed slight muscle atrophy, the boy’s physical appearance was unremarkable. Because he was not a high-risk case for general anesthesia, general anesthesia combined with right brachial plexus block (interscalene approach) was scheduled. After induction of general anesthesia with propofol, a laryngeal mask airway was inserted carefully. Subsequently, an anesthesiologist with 13 years of experience performed right brachial plexus block using the dual-guidance in plane technique. The brachial plexus was identified easily between the anterior and middle scalene muscles that were not atrophied. Electrical nerve stimulation was performed with a nerve stimulator (Stimuplex HNS12, B/BRAUN, Melsungen, Germany) with 1 mA output, and contraction of the right forearm muscle was confirmed. The needle tip (Stimuplex A, 22G × 50 mm; B/BRAUN) was placed between the anterior and middle scalene muscles. After muscle contraction disappeared at 0.2 mA, 3 mL of 0.375% ropivacaine were injected. No adverse events were noted in the perioperative period. Muscle biopsy confirmed the diagnosis of DMD.

Back to Top | Article Outline

Patient 2

A 14-year-old boy was scheduled for left gracilis muscle biopsy because of high suspicion of DMD. His body weight was 35 kg, and he was 160 cm tall. He was severely disabled and wheelchair-bound because of cardiorespiratory muscle involvement that necessitated noninvasive positive pressure ventilator support at night. The serum CK level was 345 U/L, and serum electrolyte concentrations were normal. Preoperative computed tomography showed whole muscle atrophies. The only muscles suitable for biopsy were both gracilis muscles. Because general anesthesia in this patient was considered to be high risk, left femoral and obturator nerve blocks using the dual guidance method were planned. The same nerve stimulator and the same type of needle as in the first patient were used, and an anesthesiologist with 19 years of experience performed the nerve blocks. In-plane ultrasonography showed extremely thin muscle fibers that were almost invisible in every view. Fasciae and left femoral nerve barely could be identified. In contrast to the previous patient, no muscle contraction was observed at a stimulation energy of 1 mA, not even at an energy of 2 mA. Despite adjustment of position of the needle tip, no muscle contraction was observed. For lack of a better solution, we injected 15 mL of 1.5% lidocaine at both sites under ultrasound guidance alone. No adverse events were noted in the perioperative period. Muscle biopsy confirmed the diagnosis of DMD.

Back to Top | Article Outline

DISCUSSION

There are no published reports on the muscular response to nerve stimulation in patients with DMD. Patients with diabetic foot gangrene showed an increased threshold to electrical motor stimulation as compared with patients without diabetes.4 Unlike patients with diabetes who have neural impairment, the altered muscle response to nerve stimulation in patients with DMD is caused by skeletal muscle pathology. The serum CK levels in patients with DMD are at their greatest at the age of 3 years, decreasing thereafter at the rate of about 20% per year as the muscles become progressively atrophied.5 The serum CK concentration was much lower in patient 2 compared with patient 1, presumably because of severe skeletal muscle atrophy.

In patients with DMD scheduled for surgeries, anesthesia methods suitable for the disease stage have to be selected because the anesthetic complications often seemed to parallel the severity of the muscle disease.6 In recent years, the advantage of regional anesthesia in DMD patients with respiratory, cardiovascular involvement has been reported.7 Intercostal nerve blocks were performed successfully in a patient with DMD and associated severe respiratory impairment for surgery on the chest wall.8 Ultrasound and electrical stimulation-guided techniques improve safety and efficacy of nerve blocks. The stimulation-guided technique failed in patient 2, however, probably because of the advanced stage of the disease. Muscle responses to nerve stimulation seem to vary with the severity of the muscle disease.

Back to Top | Article Outline

CONCLUSIONS

In conclusion, this is the first report of muscle responses to nerve stimulation in patients with DMD at different stage. When performing electrical stimulation-guided nerve blocks in the presence of advanced muscular disease, we must be aware that muscle response to nerve stimulation will vary with the disease stage.

Back to Top | Article Outline

DISCLOSURES

Name: MinHye So, MD.

Contribution: This author helped with the anesthetic management of Case 2, and with development of the overall anesthetic plan for two cases.

Name: Takeshi Sugiura, MD, PhD.

Contribution: This author helped with the anesthetic management of Case 1, and with development of the overall anesthetic plan for two cases.

Name: Saya Yoshizawa, MD.

Contribution: This author helped with the anesthetic management of Case 1, and with development of the overall anesthetic plan for two cases.

Name: Kazuya Sobue, MD, PhD.

Contribution: This author helped with the development of the overall anesthetic plan for two cases.

This manuscript was handled by: Hans-Joachim Priebe, MD, FRCA, FCAI.

Back to Top | Article Outline

REFERENCES

1. Shah RD, Suresh SApplications of regional anaesthesia in paediatrics. Br J Anaesth. 2013;111suppl 1i114i124.
2. Brull RGebhard R, Hadzic A, Urmey WModalities of nerve block performance—is there a silver bullet? Dual Guidance, a Multimodal Approach to Nerve Location.2008: B.Braun Medical IncBethlehem, PA: 314.
3. Krishnan AV, Kieman MCAxonal function and activity-dependent excitability changes in myotonic dystrophy. Muscle Nerve. 2006;33:627636.
4. Keyl C, Held T, Albiez G, Schmack A, Wiesenack CIncreased electrical nerve stimulation threshold of the sciatic nerve in patients with diabetic foot gangrene: a prospective parallel cohort study.Eur J Anaesthesiol. 2013;30:435440.
5. Ramani RHines RL, Marschall KESkin and musculoskeletal diseases. Stoelting’s Anesthesia and Co-existing Disease. 2012:6th ed. Philadelphia, PA: Elsevier, Inc; 437465.
6. Urban MKFleisher LAMuscle diseases. Anesthesia and Uncommon Diseases. 2012:6th ed. Philadelphia, PA: Elsevier, Inc; 296318.
7. Bang SU, Kim YS, Kwon WJ, Lee SM, Kim SHPeripheral nerve blocks as a the sole anesthetic technique in a patient with severe Duchenne muscular dystrophy. J Anesth. 2016;30:320323.
8. Vandepitte C, Gautier P, Bellen P, Murata H, Salviz EA, Hadzic AUse of ultrasound- guided intercostal nerve block as a sole anaesthetic technique in a high- risk patient with Duchenne muscular dystrophy. Acta Anaesthesiol Belg. 2013;64:9194.
Copyright © 2017 International Anesthesia Research Society