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CASE REPORT
Anesthesia in a patient with multiple-system atrophy using
a combination of rocuronium and sugammadex
Akira Kajiura1, Hiroyuki Kinoshita1, 2, 3, and Takashi Yamasaki1
1
Department of Anesthesiology, IMS Fujimi General Hospital, Fujimi, Japan, 2Department of Anesthesiology, Tokushima University Graduate
School of Biomedical Sciences, Tokushima, Japan, 3Department of Anesthesiology, Seirei Mikatahara General Hospital, Hamamatsu, Japan

Abstract : Multiple-system atrophy is an adult-onset progressive neurodegenerative disease affecting thecentral nervous system, including the spinal cord. There has been no perioperative guideline of the muscle
relaxants used in multiple-system atrophy, although a recent article recommends anesthesiologists to use
shorter-acting drugs at the lowest possible doses in the patients. Here, we document the first case with multiple-system atrophy undergoing surgery managed with a combination of rocuronium and sugammadex. The
recovery time to the train-of-four count 2 after intravenous rocuronium 0.6 mg / kg, or the time from the start ofsugammadex 2 mg / kg intravenously to the train-of-four ratio over 0.9 was prolonged in our case more than 20and 2 minutes compared with those in healthy subjects, respectively. Neuromuscular monitoring, in addition
to the careful vigilance in the perioperative period, seems mandatory in the patients. J. Med. Invest. 68 : 381-382,
August, 2021
Keywords : Multiple-system atrophy, rocuronium, sugammadex

INTRODUCTION
Multiple-system atrophy is an adult-onset progressive neurodegenerative disease affecting the central nervous system, including the spinal cord (1). The estimated point prevalence is 7.8
per 100,000 among persons older than 40 years of age (1). Motorsymptoms are common in patients with multiple-system atrophy,
and sudden death occurs due to acute bilateral vocal-cord paralysis (1). There has been no perioperative guideline of the muscle
relaxants used in multiple-system atrophy. However, a recent article recommends anesthesiologists use shorter-acting drugs at
the lowest possible doses in the patients (2). Here, we document
the first case with multiple-system atrophy undergoing surgery
managed with a combination of rocuronium and sugammadex.

CASE REPORTInformed consent to publication was obtained from the patient. A 70-year-old male (159 cm, 49.8 kg, BMI 19.7) wasscheduled for anterior cervical fixation and the delayed posterior
one due to cervical spondylotic myelopathy. He was diagnosed
as probable multiple-system atrophy more than two years fromthe onset with the decreased perfusion of the cerebellar hemisphere and the symptoms, including urinary incontinence,orthostatic hypotension, and ataxia of the limbs (1). His preoperative pulmonary function testing revealed the obstructive
respiratory abnormality (vital capacity 2.31 L [percent vital
capacity 75% ; forced expiratory volume 0.7 L [forced expiratory
volume percent in one second 37%]]), whereas his respiratory
symptom was unremarkable. Upon the anterior fixation at thesupine position, anesthesia was induced with remifentanil 0.5
Received for publication March 4, 2021 ; accepted June 2, 2021.
Address correspondence and reprint requests to Hiroyuki Kinoshita,
Department of Anesthesiology, Tokushima University Graduate
School Biomedical Sciences, 3-18-15 Kuramoto, Tokushima, Tokushima
770-8503, Japan.

µg / kg / min in combination with a propofol target-controlled
infusion at 3 µg / ml. Neuromuscular monitoring (TOF Watch
SX, Nihon Kohden, Tokyo, Japan) at the left adductor pollicis
muscle by the ulnar nerve stimulation was initiated. The calibration of monitoring, including the 50 Hz-tetanus stimulationand train-of-four stimulation until the twitch height became
stable, was completed. The train-of-four count reached zero at
1.9 minutes after rocuronium 0.6 mg / kg intravenously. The
train-of-four count 2 reappearance time after rocuronium was
50.8 minutes, and sugammadex 2 mg / kg was administered forthe motor-evoked-potential monitoring, leading to the train-offour ratio over 0.9 at 4.8 minutes after the sugammadex. Upon
the posterior cervical fixation two weeks later, anesthesia wasinduced with remifentanil (0.5 µg / kg / min) and propofol (target-controlled infusion 3 µg / ml) simultaneously. TOF-Cuff (RGBMedical Devices, Madrid, Spain) was employed at the left arm
for neuromuscular monitoring after the Auto-Pilot mode’s calibration since TOF Watch SX is unreliable at the prone position.
The train-of-four count became 0 at 2 minutes after intravenousrocuronium 0.6 mg / kg ; train-of-four count 2 reappearance timeafter the rocuronium was 53 minutes ; the train-of-four ratio
reached over 0.9 at 4 minutes after the sugammadex 2 mg / kg
intravenously. Tracheal intubation in the patient was performed
using a video laryngoscope (McGRATHTM MAC, MEDTRONICJAPN CO., LTD., Tokyo, Japan). Body temperature measured
using the built-in thermister toward a bladder indwelling balloon catheter maintained from 36.9 to 37.4℃ during the above
neuromuscular monitoring. Neither respiratory complication nor
refractory hypotension was noted during perioperative periods
upon both surgical procedures.

DISCUSSION
In our patient, the recovery time to train-of-four count 2 after
intravenous rocuronium 0.6 mg / kg (mean 51.9 minutes), or thetime from the start of sugammadex 2 mg / kg intravenously to thetrain-of-four ratio over 0.9 (mean 4.4 minutes) was prolonged.
Indeed, these values are more than 20 and 2 minutes compared

The Journal of Medical Investigation Vol. 68 2021

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A. Kajiura, et al. Multiple-system atrophy and anesthesia

with those in healthy subjects, respectively (3, 4). However, thedose requirement of vecuronium in a patient with olivopontocerebellar atrophy, one of the old categories of multiple-system
atrophy, was reportedly normal (5). Therefore, further studies
will require to conclude both compounds’ pharmacological characteristics in patients with multiple-system atrophy.
We employed different neuromuscular monitoring systems,
including TOF Watch SX using the acceleromyography principle
and TOF-Cuff operating by another mechanism. The durations
from the intravenous sugammadex to the train-of-four ratio
reached over 0.9 were not significantly different between the patient’s two-time exposure to rocuronium. These results indicatethat both monitoring systems are clinically meaningful upon
the perioperative management of patients with multiple-system
atrophy, although a previous study indicated a possible underestimation of train-of-four ratios neuromuscular recovery period
by TOF-Cuff (6).
A recent systematic literature search regarding multiple-system atrophy documented the intraoperative problems in patientswith the disease state, including difficulties with airway management and hypotension (2). However, our patient did not show
any respiratory complication or refractory hypotension during
perioperative periods upon both surgical procedures. These
results may result from the fact that the patient was probably
in the late premotor phase of multiple-system atrophy only withurinary incontinence, orthostatic hypotension, and cerebellar
features (1). Recent literature recommends clinicians use shorteracting drugs at the lowest possible doses in patients with thedisease since residual effects of intravenous and inhalational
anesthetics, opioids, and muscle relaxants may influence their
postoperative respiratory outcomes (2). Also, rapid sequence induction and tracheal intubation are desirable because there is an
inherent risk of pulmonary aspiration from gastroparesis (2). It
is critical to prepare for a difficult airway because muscle rigidity may result in difficulty with tracheal intubation in patientswith multiple-system atrophy (2). Therefore, we administered
the rocuronium dose expecting its rapid action at the time oftracheal intubation in our case. However, we do not have a clear
recommendation about the optimal rocuronium initial dose upon
anesthetic induction.
In conclusion, approximately 50% of patients withmultiple-system atrophy require walking aids within threeyears after the onset of motor symptoms (1), and thus, neuromuscular monitoring and careful vigilance in the perioperative
period seem mandatory in the patients.

DECLARATION OF CONFLICTING INTERESTS
Hiroyuki Kinoshita is a consultant of IMI Co. Ltd, Koshigaya,
Saitama, Japan. Other authors declared no potential conflictsof interest for the research, authorship, and / or publication ofthis article. All authors received no financial support for the research, authorship, and / or publication of this article.

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