EXPERIMENTAL AND THERAPEUTIC MEDICINE 19: 1091-1094, 2020

Successful treatment of a 12‑year‑old boy withGuillain‑Barré syndrome requiring tracheostomy dueto respiratory muscle paralysis: A case report
MANABU MIYAMOTO1, GEORGE IMATAKA1, GO ICHIKAWA1, YUTAKA SAITO2, TAKASHI KASHIWAGI3,YOSHIYUKI KAJI2, KOJI WAKE2, KEI FUNAKOSHI4, TAKAHIDE NAGASHIMA4,NORITO KOKUBUN4 and SHIGEMI YOSHIHARA1Departments of 1Pediatrics, 2Emergency and Critical Care Medicine, 3Otorhinolaryngology ‑ Head and Neck Surgery,and 4Neurology, Dokkyo Medical University, Mibu, Tochigi 321‑0293, Japan
Received February 8, 2019; Accepted October 16, 2019
DOI: 10.3892/etm.2019.8311
Abstract. Childhood���������������������������������������������Guillain‑Barré syndrome (GBS) occasionally leads to respiratory failure early after onset, requiringlong‑term ventilation management after tracheal intubation.
However, patients requiring tracheostomy management arerare. In the present study, a case of a 12‑year‑old boy withGBS who required artificial respiration management due torapid progression of respiratory muscle paralysis is reported.
Intravenous immunoglobulin (IVIg) and pulse steroid therapy
were provided; however, both were ineffective and tracheostomy was necessary 26 days after onset. A second course ofIVIg and pulse steroid therapy was administered on day 34.
With continued rehabilitation, the patient was able to walklong distances on day 74 and was subsequently discharged on
day 89. In cases of severe GBS, when IVIg and pulse steroid
therapy do not improve the respiratory muscle strength of thepatient, early tracheostomy may improve the patient's qualityof life during artificial respiration management.
IntroductionGuillain‑Barré syndrome (GBS) is an acute motor‑neuron,
peripheral neuropathy characterized by symmetrical ascen­ding motor paralysis and a generally monophasic clinicalcourse. The clinical manifestations of GBS include attenuation or disappearance of tendon reflexes, sensory disorders,such as numbness and pain, autonomic nervous systemdisorders, such as bradycardia and tachycardia, arrhythmias,abnormal blood pressure, and intestinal motility abnormalities. Moreover, 20‑30% of patients require mechanicalventilation because of respiratory failure, and approximately
half of the patients have cranial nerve deficits, primarily facialpalsy (1). Generally, paralysis symptoms appear 1‑2 weeks
after infection, the symptoms peak within 4 weeks, and then,the patients recover (2,3). The most common treatments for
GBS are a combination of methylprednisolone steroid pulse
therapy and intravenous immunoglobulin (IVIg), and a current
double‑blind, randomized clinical trial is on‑going to assess
the effect of a second IVIg course in severely affected GBS
patients (4). Occasionally, however, patients may requirelong‑term artificial respiratory management secondary torespiratory muscle paralysis (5,6). In children, the incidenceof mechanical ventilation is quite high, with reported rates of
19.2% (7), 20% (8) and 14% (9). Although tracheostomy may
improve the patient's quality of life during long‑term ventilation management, this approach has been infrequent amongchildren with GBS.
In the present study, the clinical course of a patient with
GBS who required artificial respiration management andtracheostomy due to rapid progression of respiratory muscle
paralysis is reported.
Case report

Correspondence to: Dr George Imataka, Department of Pediatrics,

Dokkyo Medical University, 880 Kitakobayashi, Mibu, Shimotsuga,Tochigi 321‑0293, JapanE‑mail: geo@dokkyomed.ac.jp

Abbreviations: GBS, Guillain‑Barré syndrome; IVIg, intravenous
immunoglobulin; MMT, manual muscle testing

Key words: Guillain‑Barré syndrome, children, tracheostomy,immunoglobulin, steroid pulse

The study was approved by the Ethics Committee of theDokkyo Medical University (Mibu, Japan). Formal written
informed consent was obtained from the parents of the patient.
Our patient, recruited on July 2015, was a healthy 12‑year‑oldboy with a history of febrile convulsions at 4 months of age.
He reported allergies to mackerel and buckwheat, and had
an unremarkable family medical history. Prior to visiting thehospital, he developed common cold symptoms accompanied
by fever and headache, which improved in a few days. However,during that 1st week, he developed numbness in his fingers andlimbs prompting a visit to the Neurosurgery Department on
day 8. Head computed tomography revealed normal findings.

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MIYAMOTO et al: TREATMENT OF GUILLAIN-BARRÉ SYNDROME

Figure 1. Clinical course of patient with GBS. GBS, Guillain‑Barré syndrome; MMT, manual muscle testing; IVIg, intravenous immunoglobulin; mPSL,methylpredonisolone pulse.

On day 9, he visited a private clinic because of vomiting,received intravenous fluid therapy without improvement, andwas subsequently admitted to a general hospital. Although hewas able to sit up at the time of hospital admission, he foundit difficult to walk unassisted for long periods. Magnetic
resonance imaging of his head and spinal cord showed noabnormal findings. On day 12, he was urgently transferred toour University Hospital because of dysphagia and dyspnea.
On admission, he weighed 51 kg, had a height of 155 cm,
body temperature of 36.6˚C, heart rate >100/min, and bloodpressure of 168/116 mmHg. His oxygen saturation was 92%
breathing room air and his consciousness level remained
high. He produced weak respiratory sounds without a heartmurmur, and attenuated intestinal peristalsis on chest andabdominal auscultation. The patient had difficulty keeping
his eyes closed which were noticed to be dry. Neurologicalexamination revealed a decreased muscle tone in his limbs,
with manual muscle testing (MMT) showing bilateralsymmetry, upper limb grades of 2, lower limb grades of 0‑1,and lower‑limb‑dominant muscle weakness. Numbness and a
decline in the superficial sensation of his hands and fingers
with absent deep tendon reflexes in both upper and lower limbs
were also observed.
Blood examination showed elevated white blood cellcount (15,700/µl) and platelet count (60x104/µl). Additionally,
the patient tested negative for Epstein‑Barr virus, cytomegalovirus, herpes simplex virus, varicella‑zoster virus, andMycoplasma spp. infections. Stool cultures detected resident
bacteria, and were negative for Campylobacter spp. Levelsof anti‑ganglioside antibodies, including GM1, GM1b, GD1a,
Ga1NAc‑GD1a, GQ1b, GD1b and GT1a, were not markedly increased. Peripheral nerve conduction velocity testing

revealed decreased median nerve action potential, prolonged
distal latency of midline and peroneal nerves, and absent
F‑waves in midline and tibial nerves. Accordingly, based onelectrophysiological testing, the patient was diagnosed ashaving a demyelinating type of childhood GBS.
The patient's clinical course after hospitalization ispresented in Fig. 1. Hughes functional grade score was 4 (difficulty walking 5 m even with support) on admission. However,the patient's breathing difficulty progressed rapidly, and 3 hafter admission, tracheal intubation was performed which
increased his functional grade to 5 (requiring supplementalventilation). The patient was transferred to an Intensive CareUnit, and immediate IVIg therapy (400 mg/kg x 5 days)
and pulse methylprednisolone therapy (500 mg x 3 days)
were prescribed. Ventilator withdrawal and extubation wereattempted on day 24 after some improvement in his neck andupper limb strength (MMT score: 3). However, his breathingdifficulty worsened, and a second tracheal intubation wasnecessary after 3 h. On day 26, it was clear that long‑term
intubation management would be required. After obtaining
written informed consent from the parents of the patient,
an otolaryngologist performed a tracheostomy. Before thetracheostomy, the patient had been dependent on a ventilator
for respiration; however, his respiratory condition improved
gradually and he was transferred from the Intensive Care
Unit to the general ward a few days later. A second courseof IVIg and pulse corticosteroid therapy were performedon day 34. On day 40, the patient's breathing improved andhis tracheostomy was closed when he no longer required
mechanical ventilation. Joint movement remained difficult
because of intense nerve root pain in his ankle; however, thepain disappeared after the second course of IVIg and pulse

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steroid therapy. After continued rehabilitation, he was able towalk long distances (functional grade: 3, 2 and 0 on days 56,66 and 74, respectively), and he was subsequently discharged
on day 89.

(22)

Discussion

GBS, Guillain‑Barré syndrome; IVIg, intravenous immunoglobulin; mPSL, methylprednisolone pulse; Nd, not described.

(19)
(20)
(21)

(18)

(16)
(17)

Kawai et al
2
Male
Expectoration difficulty, hoarseness
Nd8+
NdExtubation and discharge
Minamihara et al3
Female Swallowing difficulty, hoarseness218++Extubation and								discharge on day 58Takeshita et al
4MaleExpectoration difficulty, dysphonia
Nd16+
NdExtubation and								discharge on day 75
Shibata et al
8MaleBreathing difficulty25+
Nd
Extubation and discharge
Arashin et al
10Female Shallow breathing
2Nd
Nd
Nd
Extubation and discharge
Yata et al
10MaleDiplopia, gait disturbance
Nd
Nd+
NdImmunosorbent therapy,								extubation, and discharge
Kawai and Shimizu
11Female Bulbar palsy
Nd
Nd+
Nd
Adenovirus infection,								extubation, and discharge
Present study
12MaleExpectoration difficulty,
415++15 days after tracheotomy,			breathing difficulty					breathing had improved								and the hole was closed	Intubation	
IVIg	mPSL		management (days) treatment treatment
Outcomes
Intubation fromadmission (days)
Age			(years)
Sex
Characteristic initial symptoms
Studies

Table I. Mechanical ventilation management in Japanese pediatric patients with GBS from 2000 to 2015.

Refs.

EXPERIMENTAL AND THERAPEUTIC MEDICINE 19: 1091-1094, 2020

Some studies have presented the need for artificial ventilation
management because of rapid muscle weakness in patientswith GBS (10,11), and childhood‑onset GBS poses a higherrisk of requiring artificial respiration management (12).
Wijdicks (13) has reported that tracheostomy is strongly
recommended after 2 weeks to avoid tracheomalacia andpostintubation stenosis. Principi et al (14) have reported thatelective tracheostomy is rarely performed among children in
Canada; however, 51% of the physicians surveyed consider
that it is underutilized. In addition, Walgaard et al (15) havedeveloped a prediction tool for the selection of GBS patients,
among patients receiving mechanical ventilation, for earlytracheostomy. Thus, physicians should be aware of theindications for both artificial respiratory management andtracheostomy in patients with GBS, especially in children.
Table I presents details on 7 Japanese children patients withGBS who required ventilator management (16‑23). As shownin the Table, the children who ranged in age from 2‑12 years,
had early clinical features, such as dysphonia, difficultyin expectoration, and dysphagia, suggesting a decrease inrespiratory muscle strength. Table I also shows the numberof intubation days required; however, no patients underwent tracheostomy for artificial respiration management.
Moreover, in a clinical study of 91 childhood GBS cases
in Japan, 19 patients (21.6%) required artificial ventilation
management, and none received tracheostomy (20). Anotherreport has shown that among 50 adult patients with GBS,
15 underwent tracheostomy, subsequently resulting in agood clinical course and improved quality of life (5). For thepatient of the present study, IVIg and pulse steroid therapy
did not improve respiration and whole‑body muscle strength.
Given that the attempt of ventilator withdrawal and extubation led to worsened symptoms, it was decided to perform
tracheostomy early during the patient's clinical course, andit was possible to surgically close the tracheostomy soonafterwards, because of the good clinical course that followed.
Childhood GBS may occasionally lead to sudden respiratory failure requiring long‑term respiratory management.
In severe cases, in which the respiratory muscle strength
does not improve with IVIg and pulse steroid therapy, earlytracheostomy should be considered, as it may improve thepatient's quality of life during prolonged artificial respiration
management.
Acknowledgements
We would like to thank Dr Yao Lu (Seiko Sciences) and Jane
Charbonneau, DVM, from Edanz Group, for editing a draft ofthis manuscript.
FundingNo funding was received.

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MIYAMOTO et al: TREATMENT OF GUILLAIN-BARRÉ SYNDROME

Availability of data and materials
The data used and/or analyzed during the current study isavailable from the corresponding author on reasonable request.
Authors' contributions
MM, GIm, GIc and SY were involved in the writing of themanuscript. MM and GIc were the patient's doctors. YS, YK
and KW were involved in the patient's intensive care treatment
and collected the blood test results. TK operated on the patientand planned the patient's clinical course. KF, TN and NK
analyzed the biological data of the patient and evaluated theresults of the physiological peripheral nerve testing. GIm wasresponsible for monitoring the patient's neurological status
in Intensive Care Unit. MM and SY were responsible for themedical strategy for patient's respiratory failure. All authors
read and approved the final manuscript.
Ethics approval and consent to participate
The study was approved by the Ethics Committee of theDokkyo Medical University. Formal written informed consent
was obtained from the parents of the patient.
Patient consent for publication
The authors received formal written informed consent fromthe parents of the patient approving the publication of thesedata.
Competing interests
The authors declare that they have no competing interests.
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