대한마취과학회지 2008; 55: 124～7

□ 증례보고 □
Korean J Anesthesiol Vol. 55, No. 1, July, 2008

General anesthesia for laparoscopic cholecystectomy

Department of Anesthesiology and Pain Medicine, Seoul National University Bundang Hospital, Seongnam, Korea

Jang-hyun Kim, M.D., Jung-won Hwang, M.D., Yun Mi So, M.D.,

Kennedy’s disease is a rare lower motor neuron disease affecting the limbs and bulbar musculature. Regional anesthesia is
generally the recommended anesthetic technique in patients with Kennedy’s disease because of bulbar involvement and airway
clearing disturbance. We administered general anesthesia in a patient with Kennedy’s disease who was undergoing a laparoscopic
cholecystectomy. We closely monitored the degree of neuromuscular blockade throughout the operation and injected atracurium
on demand. There was no exacerbation of neurologic signs or symptoms postoperatively. Therefore, we report the successful
administration of general anesthesia for laparoscopic cholecystectomy in a patient with Kennedy’s disease. (Korean J Anesthesiol
2008; 55: 124∼7)

Key Words: general anesthesia, Kennedy’s disease, laparoscopic cholecystectomy, lower motor neuron disease.

Kennedy’s disease is an X-linked spinobulbar motor neuron his grandfather, father and elder brother. He presented hand
disorder characterized by degeneration of lower motor neurons tremor 20 years ago as an initial symptom, facial fasciculation
1)
supplying the limbs and bulbar musculature. Regional around mouth 10 years ago, and he had difficulties in stepping
anesthesia is preferred in patient with Kennedy’s disease one stair step since 5 years ago. His symptoms had got
because bulbar muscle weakness makes the patient vulnerable progressed and he could not sit down by himself lately. But
2)
to aspiration in general anesthesia. There was one case report he had never visited medical center for any evaluation or
about regional anesthesia of Kennedy’s disease, but no case diagnosis by that time.
report about general anesthesia of Kennedy’s disease. We On physical examination, he had facial and tongue
report a case of general anesthesia for laparoscopic fasciculation, resting hand tremor, muscle atrophy in all
cholecystectomy of a patient with Kennedy’s disease. extremities, both proximal and distal part of each and slight
right side gynecomastia. He had no defect in sensory but
CASE REPORT decreased muscle power in all extremities, especially in both
proximal lower extremities. Both pathologic reflexes (i.e. toe
A 57-yr-old man (62.3 kg, 162.3 cm) was planned to sign, Hoffmann sign) and deep tendon reflexes were not
receive a laparoscopic cholecystectomy for gallbladder polyp. detected. He had slurred speech and difficulties in
Before the operation, he received the medical evaluation of expectorating sputum. He did not complain the aspiration but
peri-operative risks because he had symptoms and familial sometimes he woke from sleep due to choking up by his own
history of muscular abnormalities. The diseases had affected sputum.
Needle electromyogram (EMG) and nerve conduction study
Received：March 14, 2008
Corresponding to：Jung-won Hwang, Department of Anesthesiology and (NCS) showed wide spread denervations over 4 segments (C6,
Pain Medicine, Seoul National University Bundang Hospital, T8, T9, L2). Somatosensory evoked potential study (SSEP)
300, Gumi-dong, Bundang-gu, Seongnam 463-500, Korea. showed both peripheral and central conduction defect. Plasma
Tel: 82-31-787-7492, Fax: 82-31-787-4863, E-mail: jungwon
@snubh.org creatine kinase concentration was 353 units/L. All of the clues

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 Jang-hyun Kim, et al：Kennedy’s disease and general anesthesia

suggested Kennedy’s disease, and gene study confirmed the pull off the endotracheal tube but we decided to restrain the
diagnosis. Spinal and bulbar muscular atrophy (SBMA) gene patient and extubate later for safety. Postoperative one day, the
test was positive with abnormal CAG repeats and survival of trachea was extubated, and the patient looked tolerable and
motor neuron 1 (SMN1) gene test was negative. was moved to the ward. The patient discharged without any
He also had hypertension for 5 years and moderate to operation-related problem at third day after surgery.
severe fatty liver, but systolic blood pressure was ranged from
100 mmHg to 120 mmHg with aspirin, losartan (CozaarTM, DISCUSSION
MSD-Korea, Korea) and atenolol, and liver enzyme was within
normal limit. Kennedy’s disease is an inherited disorder characterized by
It was decided to proceed with the operation after discussion degeneration of both motor and sensory neurons.3) The
with neurologists. The patient had no premedication and was mutation is an expanded repeat of the CAG trinucleotide at
anesthetized with propofol and remifentanil performing total the Xq11-q12 band of the long arm of the X chromosome.
intravenous anesthesia (TIVA) by target site infusion device The gene product is an androgen-receptor protein which is
(OrchestraTM, Fresenius Vial, France). Nerve stimulator (Inner- expressed in nuclear inclusions in the spinal motor neurons.
4)

vator252, Fisher & Paykel Healthcare Ltd., New Zealand) was The exact mechanism by which the neuronal degeneration
used for monitoring neuromuscular blockade. The T4/T1 ratio occurs remains unknown. Only males express the full
was 100% before the induction of the anesthesia, but the phenotype, but female carriers often show subclinical phenotype
amplitude of each twitch was relatively low. One hundred expressions such as muscle cramps and finger tremors.
percent oxygen was applied to the patient for 5 minutes for Kennedy’s disease is frequently misdiagnosed as Amyotro-
denitrogenation via fitting mask. Under the Sellick maneuver, phic Lateral Sclerosis (ALS). The presence of a subtle sensory
target controlled infusion of propofol for 5μg/ml of the effect neuropathy and no signs of pyramidal tract involvement could
site concentration and remifentanil (5 ng/ml) were started. be distinguishing factors of Kennedy’s disease from ALS. If
After loss of consciousness, gentle mask ventilation was genetic study is performed, Kennedy’s disease should not be
applied keeping airway pressure under 10 cmH2O. After misdiagnosed, but genetic study is too expensive to be
atracurium (10 mg) was administered and TOF disappeared, performed as routine diagnostic tool.
the endotracheal tube (ID 7.5 mm) was inserted into the Typical onset of disease is between the age 40 and 60, and
trachea. After intubation, effect site concentrations of propofol the disease may appear as early as mid-20s.5) Symptoms
and remifentanil were reduced to 3.5μg/ml and 3 ng/ml appear as easy fatigability, muscle cramps, and weakness in
respectively. Forth twitch in TOF re-appeared 15 minute later, the limbs at first, and disability increases until the patient is
but the amplitude was low. There were no signs of wheelchair bound as the disease progresses. Bulbar muscle
spontaneous respiration or abdominal wall tightness. Eighty weakness is expressed as difficulties in chewing, swallowing,
minutes later, the patient presented the sign of spontaneous and speaking. Life expectancy does not appear to be
respiration on capnography and we administered 2.5 mg of compromised if the complications are prevented. Fall down and
atracurium, one fourth of induction dose, and TOF disappeared. aspiration are two major complications of Kennedy’s disease in
Total anesthetic time was 105 minutes, and surgery time was ordinary time.
60 minutes. There was no specific event during the surgery In the aspect of anesthetic management, aspiration is the
except administration of ephedrine 5 mg once due to low most important complication in Kennedy’s disease. Sensitivity
blood pressure. to neuromuscular blocking agents (NMBAs) is also a major
After surgery, the patient was transferred to intensive care concern because there is the possibility of delayed recovery of
unit (ICU) with the endotracheal tube kept. As we planned bulbar muscle power, which may result in regurgitation,
ventilatory care after surgery, we didn’t reverse muscle aspiration, airway clearance disturbance, and disability to keep
relaxation, but the TOF was 4 on 4 at discharge and the airway.
patient showed adequate spontaneous respiration in the ICU, so Epidural anesthesia has been successfully administered to
we applied T-piece with 5L of oxygen and the SpO2 was patients with lower motor neuron disease without either
maintained between 95 and 100 percent. The patient tried to neurologic exacerbation or impairment of pulmonary function

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but the operations were urethrotomy, hernia repair and the duration of action of the relaxant and recovery from
2,6,7)
hysterectomy. We planned general anesthesia, however, neuromuscular blockade. Of all anesthetics currently used,
because the pneumoperitoneum produced by carbon dioxide TIVA has the least potentiating ability.14) In this regard, we
(CO2) during the laparoscopic cholecystectomy causes chose TIVA as an anesthetic technique.
abdominal distension and hypercapnia. Pneumoperitoneum Third, which NMBA should be used? There is no
makes the patient difficult to breathe and feel uncomfortable prohibition except succinylcholine. Patients with lower motor
during the surgery, even though several studies suggest that neuron disease are at risk for hyperkalemia after the
laparoscopic cholecystectomy could be performed under administration of succinylcholine because of the up regulation
regional anesthesia.8-10) General anesthesia with intubating the of postsynaptic nicotinic acetylcholine receptors.15) We used
trachea would be better way to protect the airway from atracurium which is intermediate acting NMBA and
aspiration possibly caused by abdominal distension, bowel metabolized by Hofmann elimination and nonspecific ester
manipulation, and position during the surgery. hydrolysis. Because the patient had moderate to severe fatty
There are several considerations related to general anesthesia. liver, rocuronium and vecuronium which are metabolized in
First, as general anesthesia depresses the swallowing reflexes,11) both liver and kidney, were avoided. Cisatracurium was not
the patient with Kennedy’s disease may be predisposed to available in our center. We reduced the intubation dose of
regurgitation and aspiration. So, drugs such as antacids, H2 atracurium to one third of the usual dose 10 mg (intubation
blockers, gastrokinetic agents, and anticholinergics should be dose of atracurium is 0.5 to 0.6 mg/kg, which is from 31 mg
considered to reduce both the incidence and the severity of to 38 mg in this patient) while monitoring the degree of
regurgitation and aspiration. However we did not consider any muscle relaxation with a nerve stimulator because there were
premedication in this case, since the patient showed the bulbar cases of ALS patients who had hypersensitivity to
16)
involvement but not the signs or symptoms of the aspiration. nondepolarizing neuromuscular blockers.
A nasogastric tube can be inserted before surgery in order to Fourth, hypothermia may prolong the duration of action of
decompress the stomach and avoid vomiting and aspiration. No nondepolarizing NMBAs. The Hofmann elimination process of
premedication such as sedatives or hypnotics was given and atracurium is slowed by a decrease in pH and especially by a
the trachea was intubated with Sellick maneuver. decrease in temperature.17) We used blanket-roll and air-warmer
After the surgery, we transferred the patient to ICU with the during the surgery to prevent hypothermia.
endotracheal tube and kept the tube cuffed to reduce the risk We managed a patient with Kennedy’s disease by general
of the aspiration by early extubation until the patient had anesthesia without any problems or exacerbating neurologic
enough power to expectorate the sputum by himself. In the symptoms. We suggest that general anesthesia can be a proper
ICU, the patient applied to 100% oxygen via T-piece and the technique of anesthesia in patients with Kennedy’s disease if
saturation was maintained. The patient tried to pull off the we pay enough attentions.
tube half an hour later but the limb power was weak and we
thought that the bulbar muscle power was not properly REFERENCES
recovered yet. As the patient showed proper tongue movement
1. Sperfeld AD, Karitzky J, Brummer D, Schreiber H, Haussler J,
and no needs to oxygen supply, endotracheal tube was
Ludolph AC, et al: X-linked bulbospinal neuronopathy: Kennedy
removed at 16 hr after the surgery. Sperfeld et al.12) reported
disease. Arch Neurol 2002; 59: 1921-6.
that about 47% of Kennedy disease patients had shown 2. Okamoto E, Nitahara K, Yasumoto M, Higa K: Use of epidural
recurrent laryngospasm in ordinary times, and early extubation anaesthesia for surgery in a patient with Kennedy's disease. Br
could be an exaggerating factor. So we decided to extubate J Anaesth 2004; 92: 432-3.
carefully, being ready for re-intubation. 3. Amato AA, Prior TW, Barohn RJ, Snyder P, Papp A, Mendell JR:
Kennedy's disease: a clinicopathologic correlation with mutations
Second, there is no evidence that specific anesthetic drugs or
in the androgen receptor gene. Neurology 1993; 43: 791-4.
combinations of drugs are best for patients with lower motor
4. Shimada N, Sobue G, Doyu M, Yamamoto K, Yasuda T, Mukai
neuron disease.13) But inhaled anesthetics can potentiate the E, et al: X-linked recessive bulbospinal neuronopathy: clinical
neuromuscular blocking effect. This results in the decreased phenotypes and CAG repeat size in androgen receptor gene.
dosage requirement of the NMBA and the prolongation of both Muscle Nerve 1995; 18: 1378-84.

126
 Jang-hyun Kim, et al：Kennedy’s disease and general anesthesia

5. Harding AE, Thomas PK, Baraitser M, Bradbury PG, hypoglossal nerve activity during the swallowing reflex in cats. Br
Morgan-Hughes JA, Ponsford JR: X-linked recessive bulbospinal J Anaesth 1985; 57: 208-13.
neuronopathy: a report of ten cases. J Neurol Neurosurg Psychiatry 12. Sperfeld AD, Hanemann CO, Ludolph AC, Kassubek J: Laryn-
1982; 45: 1012-9. gospasm: an underdiagnosed symptom of X-linked spinobulbar
6. Kochi T, Oka T, Mizuguchi T: Epidural anesthesia for patients muscular atrophy. Neurology 2005; 64: 753-4.
with amyotrophic lateral sclerosis. Anesth Analg 1989; 68: 410-2. 13. Stoelting RK, Dierdorf SF: Anesthesia and co-existing disease/
7. Hara K, Sakura S, Saito Y, Maeda M, Kosaka Y: Epidural handbook for anesthesia and co-existing disease. 4th ed.
anesthesia and pulmonary function in a patient with amyotrophic Philadelphia, Churchill Livingstone. 2002, p 257.
lateral sclerosis. Anesth Analg 1996; 83: 878-9. 14. Bock M, Klippel K, Nitsche B, Bach A, Martin E, Motsch J:
8. van Zundert AA, Stultiens G, Jakimowicz JJ, Peek D, van der Rocuronium potency and recovery characteristics during steady-
Ham WG, Korsten HH, et al: Laparoscopic cholecystectomy under state desflurane, sevoflurane, isoflurane or propofol anaesthesia. Br
segmental thoracic spinal anaesthesia: a feasibility study. Br J J Anaesth 2000; 84: 43-7.
Anaesth 2007; 98: 682-6. 15. Beach TP, Stone WA, Hamelberg W: Circulatory collapse
9. van Zundert AA, Stultiens G, Jakimowicz JJ, van den Borne BE, following succinylcholine: report of a patient with diffuse lower
van der Ham WG, Wildsmith JA: Segmental spinal anaesthesia for motor neuron disease. Anesth Analg 1971; 50: 431-7.
cholecystectomy in a patient with severe lung disease. Br J 16. Rosenbaum KJ, Neigh JL, Strobel GE: Sensitivity to nond-
Anaesth 2006; 96: 464-6. epolarizing muscle relaxants in amyotrophic lateral sclerosis: report
10. Tzovaras G, Fafoulakis F, Pratsas K, Georgopoulou S, Stamatiou of two cases. Anesthesiology 1971; 35: 638-41.
G, Hatzitheofilou C: Laparoscopic cholecystectomy under spinal 17. Leslie K, Sessler DI, Bjorksten AR, Moayeri A: Mild hypothermia
anesthesia: a pilot study. Surg Endosc 2006; 20: 580-2. alters propofol pharmacokinetics and increases the duration of
11. Nishino T, Honda Y, Kohchi T, Shirahata M, Yonezawa T: Effects action of atracurium. Anesth Analg 1995; 80: 1007-14.
of increasing depth of anaesthesia on phrenic nerve and

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