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QJM: An International Journal of Medicine, 2016, 319323

doi: 10.1093/qjmed/hcv190
Advance Access Publication Date: 15 October 2015
Original paper

ORIGINAL PAPER

Outcome of GuillainBarre syndrome patients with


respiratory paralysis
J. Kalita, A. Ranjan and U.K. Misra
From the Department of Neurology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, UP,
226014, India
Address correspondence to U.K. Misra, Department of Neurology, Sanjay Gandhi Post Graduate Institute of Medical Sciences, Raebareily Road, Lucknow,
UP, 226014, India. email: drukmisra@rediffmail.com,ukmisra@sgpgi.ac.in

Summary
Background and Aims: To evaluate the outcome of patients with GuillainBarre syndrome (GBS) having respiratory failure
treated with modified intubation policy.
Design and Methods: Consecutive patients with GBS having single breath count below 12 and respiratory rate >30/min were
included and their clinical details noted. The patients were intubated and mechanically ventilated (MV) if their PaO2
was <60 mmHg on venturi mask, PaCo2 > 50 mmHg or pH < 7.3. Their electrophysiological subtypes and complications were
noted. The hospital mortality and 3 months outcome were compared in MV and those could be managed without MV even
with respiratory compromise.
Results: Out of 369 patients, 102 (27.6%) patients had respiratory compromise who were included in this study. Of the pa-
tients with respiratory compromise, 44 (43.1%) were intubated and mechanically ventilated after a median of 4 days of hos-
pitalization. The median duration of MV was 21 (range 188) days. The patients with autonomic dysfunction (56.8% vs. 19%),
facial weakness (78% vs. 36.2%), bulbar weakness (81.8% vs. 31%), severe weakness (63.8% vs. 31%) and high transaminase
level (47.7% vs. 25.9%) needed MV more frequently. In our study, 6.8% patients died and 26.6% had poor outcome which was
similar between MV and non-MV patients. The MV patients had longer hospitalization and more complications compared
with non-MV group.
Conclusion: In GBS patients with respiratory compromise, conservative intubation does not increase mortality and
disability.

Introduction complication of GBS and occurs in up to 40% patients.35 Severely


GuillainBarre syndrome (GBS) is rapidly progressive symmetrical affected GBS patients need close monitoring in intensive care
weakness of upper and lower limbs with or without sensory or unit (ICU) and artificial ventilation may be lifesaving.
autonomic disturbances associated with hyporeflexia or areflexia In the GBS patients with impending respiratory failure and
in absence of cerebrospinal fluid (CSF) pleocytosis. GBS is the bulbar weakness, elective intubation and artificial ventilation
commonest cause of acute flaccid paraplegia and its worldwide have been suggested.6 Respiratory paralysis is more frequent in
incidence is 1.23/100 000 population per year.1 The incidence of the patients with acute motor axonal neuropathy (AMAN)
GBS increases with age from 0.8 in those below 18 years to 3.2 in which has been reported commonly from South East Asia.79 In
those 60 years or more.2 Respiratory paralysis is a dreaded the developing countries, there is scarcity of ICU, moreover the

Received: 12 June 2015; Revised (in revised form): 24 September 2015. Accepted: 29 September 2015
C The Author 2015. Published by Oxford University Press on behalf of the Association of Physicians.
V
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319
320 | QJM: An International Journal of Medicine, 2016, Vol. 109, No. 5

available ICUs are beyond the reach of common patients. The patients were defined to have respiratory compromise
Mechanical ventilation (MV) has inherent complications such by the clinical criteria; single breath count <12 and respiratory
as ventilator associated pneumonia (VAP), sepsis, pneumo- rate >30/min. These patients were closely monitored in ICU and
thorax and line infection, which occur in 1540% patients.10 those patients were intubated and mechanically ventilated who
These complications may result in prolonged ICU stay and mor- had the following features on ABG analysis (a b or c).
tality.10 Many patients with respiratory impairment may also
(a) Inability to maintain PaO2 > 60 mmHg on ventimask.
recover without MV.
(b) PaCO2 > 50 mmHg
In our practice, we follow conservative policy for MV because
(c) pH < 7.3
of scarcity of ventilators.11 In the present study, we therefore
compare the clinical, neurophysiological characteristics and Those patients that not meet the criteria for intubation
outcome of the patients with respiratory impairment who were stated above were not intubated. The patients were closely fol-
mechanically ventilated based on alteration of arterial blood lowed up in ICU. Pulse, blood pressure, respiration and pulse
gas (ABG) parameters with those who were not mechanically oxymetry were continuously monitored in all. The ABG analysis
ventilated although they fulfilled the criteria for elective venti- was done daily or more frequently if needed. Blood counts,
lation in GBS.6 The study may help in defining the characteris- hemoglobin and serum electrolytes were done twice weekly
tics of the patients with GBS with respiratory involvement in and X-ray chest weekly or earlier if indicated. Blood culture,
whom MV can be deferred without compromising the safety. endotracheal or tracheostomy tube aspirate culture and urine
culture were carried out as indicated. Pneumonia, pneumo-
thorax, urinary infection, pressure sore, sepsis, deep vein
Subjects and methods
thrombosis and pulmonary thromboembolism were closely
Study design and setting monitored. Tracheostomy was done after 12 weeks of intub-
ation based on the clinical condition.
Consecutive patients with GBS having respiratory involvement
The patients admitted within 14 days of illness were pre-
were prospectively evaluated. The study has been approved by
scribed IVIg 400 mg/kg/day for 5 days if they could afford.
Institute Ethics Committee.
Calories, fluid, electrolytes and nutrition were maintained by
intravenous and/or nasogastric feeding. Heparin prophylaxis
Selection of patients was given to the patients who had severe weakness (MRC
The diagnosis of GBS was based on National Institute of grade <2). The patients weaned from ventilator when they
Neurological and Communicative Disorders and Stroke could maintain normal saturation on continuous positive air-
(NINCDS) criteria.12 The patients with periodic paralysis, por- way pressure (CPAP) or T pies and extubated when they main-
phyria, viral myositis, polio and non-polio enteroviral diseases, tained saturation for 2448 hr on room air.
diphtheria, botulism, rabies and toxic neuropathy were
excluded. Outcome
Outcome was defined as hospital mortality, duration of hospital
Evaluation stay and disability at 3 months. Three months functional out-
Detailed medical history including preceding events such as come was defined as complete (independent for activities of
flue like illness, rash, diarrhea, vaccination, trauma, pregnancy, daily living), partial (dependent for activities of daily living) and
childbirth and surgery were noted. The demographic variables, poor (wheel chair bound or bedridden).16
season of occurrence, duration of illness and onset to peak dis-
ability were noted. Presence of cranial nerve palsy was re-
Statistical analysis
corded. Muscle weakness was graded on a 05 MRC (Medical
Research Council) scale. Muscle tone and tendon reflexes were The patients with respiratory impairment with or without
categorized into decreased, absent or normal. Autonomic func- MV were compared for demographic details, triggers, peak
tions such as tachycardia or bradycardia, sinus arrhythmia, disability, onset to peak duration, duration of hospital stay,
fluctuation of blood pressure, sweating abnormality and bowel complications, death and 3 months disability using X2 for
and bladder dysfunction were noted. Postural hypotension was categorical and independent t test or MannWhitney U-test
assessed in the patients who could sit or stand. Peak disability for continuous variables. The predictors of MV were eval-
was assessed on a 010 scale.13 uated by logistic regression analysis. The variables having a
Blood counts, urinalysis, hemoglobin, ESR for the first hour, P-values of <0.05 were considered significant. The statistical
fasting blood sugar, serum creatinine, blood urea nitrogen, serum analysis was done by SPSS version 16 software and GraphPad
sodium, potassium, bilirubin, transaminase and creatinine kinase prism 5.
were measured. Urine porphobilinogen, HIV serology and chest
radiograph were done in all the patients. CSF was examined for
protein, cell and sugar. Motor nerve conduction of median, ulnar
Results
and peroneal and sensory nerve conduction study of median, ulnar During last 14 years, 369 patients with GBS were admitted; 102
and sural nerves were carried out bilaterally. Based on the motor (27.6%) of them had respiratory compromise requiring ICU ad-
nerve conduction parameters, i.e. distal motor latency, nerve con- mission and they were included in the present study. Their me-
duction velocity, conduction block (>20%) and minimal F wave la- dian age was 25 (range 270) years and 27 were females. The
tency, the patients were categorized into different subgroups of patients were admitted after a median of 6 (125) days of illness.
GBS.14 On the basis of nerve conduction studies, the GBS were cate- The triggering events were present in 65 (63.7%) patients and
gorized in to (i) acute inflammatory demyelinating polyradiculo- included diarrhea in 15 (23.1%), flu like illness in 42 (64.6%), sur-
neuropathy (AIDP), (ii) AMAN, (iii) acute motor sensory axonal gery in 1 (1.5%), vaccination in 1 (1.5%) and others in 6 (9.2%) pa-
neuropathy (AMSAN) and (iv) equivocal.14,15 tients. The disability peaked after a median duration of 7 (range
J. Kalita et al. | 321

224) days. The median peak disability grade was 8 (range 59). relative risk of death in the ventilated group was 1.7 (95% CI
Facial weakness was present in 65 (63.7%), bulbar weakness in 1.022.91; P 0.23). The cause of death in the ventilated patients
57 (55.9%) and autonomic disturbances in 36 (35.3%) patients. was pneumonia in two and dysautonomia in three patients. In
After nerve conduction studies, the patients were categorized the non-ventilated group, one died because of sudden cardiac
into AIDP in 65 (63.7%), AMAN in 19 (18.6%), AMSAN in 4 (3.9%), arrest and the other due to dysautonomia. The median duration
unclassifiable in 5 (4.9%) and inexcitable nerves in 5 (4.9%) pa- of MV was 21 (range 188) days. Prolonged ventilation (>15
tients. Intravenous immunoglobulin was given to 58 (56.9%) days) was needed in 25 (56.8%) patients. The median duration of
patients. hospital stay of the MV patients was longer compared with
Out of 102 GBS patients with respiratory compromise, those of non-MV patients [38 (range 3105) vs. 12 (range 331)
44 (43.1%) needed mechanical ventilation based on ABG criteria days; P < 0.001]. The ventilator-related complications were
after a median duration of 4 (120) days of hospitalization. noted in 19 (43.2%) patients and included VAP in 16 (34.4%), lung
There was no significant difference in the demographic vari- collapse in 2 (4.5%), pneumothorax in 1 (2.3%) and sepsis in 1
ables in the MV and non-MV patients. Comparing the clinical (2.3%) patient. In the non-MV group, only three (5.2%) patients
data between the MV and non-MV patients, there was no sig- developed complications which included pneumonia in two
nificant difference in the day of admission from onset of illness (3.4%) and urinary tract infection in one (1.7%) patient (Table 2).
(median 5 vs. 7 days; P 0.18), the antecedent events (27 vs. 38; At 3 months follow up, 32 (43%) patients recovered com-
P 0.40), frequency of transient bladder involvement (5 vs. 8; pletely, 37 (39.4%) partially and 25 (26.6%) had poor recovery.
P 0.71), radicular pain (29 vs. 45; P 0.19), simultaneous in- The GBS patients with respiratory compromise who did not re-
volvement of upper and lower limbs (6 vs. 4; P 0.25) and CSF quire MV had insignificantly higher frequency of complete re-
protein (median 66 vs. 63.5 mg/dl; P 0.35). The ventilated pa- covery (23/55; 41.8%) compared with those who were
tients had insignificantly shorter median duration of onset to mechanically ventilated [9/39 (23%; P 0.08)]. The details are
peak disability [5 (range 216) vs. 7 (range 314); P 0.24]. The shown in Figure 2.
MV patients had more frequent dysautonomia (56.8% vs. 19%,
P < 0.001), facial weakness (75% vs. 36.2%, P 0.04), bulbar weak-
ness (81.8% vs. 31.0%; P 0.001), severe weakness [(MRC 02)
Discussion
63.8% vs. 31% P 0.001)] and high SGPT level (47.7% vs. 25.9%, In our study, 102 (27.6%) patients with GBS had respiratory com-
P 0.01). The details are summarized in Table 1. AMAN type of promise, 43% of them needed MV on the basis of ABG criteria.
GBS although needed mechanical ventilation more frequently Severity of weakness (MRC grade 2) was an independent pre-
(9/19, 47.4%) compared with AIDP (22/65, 33.8%) but the differ- dictor of mechanical ventilation. The patients with respiratory
ence was not statistically significant (P 0.18). The details are compromise who were mechanically ventilated had higher
shown in Figure 1. On multiple regression analysis, lower limb complications and more frequent deaths or disability compared
power MRC grade 2 (OR 6.8; 95% CI 00.29; P 0.02) was inde- with those who were not mechanically ventilated although ful-
pendently associated with need of mechanical ventilation. filled criteria of elective MV in GBS. We have followed

Outcome
In this study, seven (6.8%) patients died during the hospital; five
(11.4%) in the MV and two (3.4%) in the non-MV group. The

Table 1. Comparison of admission parameters of the patients with


GuillainBarre syndrome having respiratory involvement who
needed mechanical ventilation (MV) with those who did not based
on arterial blood gas analysis

Parameters MV (n 44) Non-MV (n 58) P

Age (yrs) 30.5 6 15.9 28 6 21.1 0.62


Male 34 (77.3%) 41 (70.7%) 0.30
Day of admission (days) 7.2 6 6.1 8.2 6 6.5 0.18
Autonomic dysfunction 25 (56.8%) 11 (19%) <0.001
Antecedent illness 27 (61.4%) 38 (65.5%) 0.40
Radicular pain 29 (65.9%) 45 (77.6%) 0.19
Figure 1. Subtypes of GuillainBarre syndrome with respiratory compromise
Cranial nerve palsy 27 (61.4%) 34 (58.6%) 0.75
who needed mechanical ventilation and those who did not.
Facial palsy 33 (75%) 32 (36.2%) 0.04
Bulbar weakness 36 (81.8%) 21 (36.2%) <0.001
Power <3 UL 31 (70.5%) 18 (31%) <0.001 Table 2. Complications during hospital stay between mechanically
Wrist dorsiflexion Gr 0 16 (36.4%) 5 (8.6%) <0.001 ventilated (MV) and non-ventilated GuillainBarre patients
Lowe limb power <3 32 (72.7%) 29 (50%) <0.02
Complications MV N 44 Non-MV N 58
Foot dorsiflexion Gr 0 18 (40.9%) 10 (17.2%) 0.005
Power in all limbs <3 28 (63.6%) 18 (31%) 0.001 Pneumonia 16 (34.4%) 2 (3.4%)
Liver dysfunction 21 (47.7%) 15 (25.9) 0.012 Lung collapse 2 (4.5%) 0 (0%)
CSF protein mg/dl 66 (16200) 63.5 (18422) 0.35 Pneumothorax 1 (2.3%) 0 (0%)
IVIg treatment 32 (72.7%) 26 (44.8%) 0.001 Fever without localization 1 (2.3%) 0 (0%)
Urinary tract infection 1 (2.3%) 1 (1.7%)
CSF, cerebrospinal fluid; IVIg, intravenous immunoglobulin.
322 | QJM: An International Journal of Medicine, 2016, Vol. 109, No. 5

study, hypokalemia was not an important predictor of mechan-


ical ventilation or outcome. In our study, only seven patients
died; five in MV and two in non-MV group. The results of our
study regarding predictors of MV and outcome are different
from the reported literature because we have included the pa-
tients with respiratory compromise and compared those requir-
ing MV with those who did not based on ABG. Had we followed
elective ventilation criteria, all these patients would have been
mechanically ventilated. This may be the reason why the mor-
tality and 3 month functional outcome were not significantly
different between MV and non-MV patients in our study. The re-
spiratory complications were significantly more in the MV pa-
tients compared with the non-MV patients (40.9% vs. 3.4%).
In this study, AIDP constituted 63.7% and AMAN in 18.6% pa-
tients. Half the patients with AMAN needed MV whereas ii was
one-third in AIDP group. Higher frequency of AMAN although
have been reported in the developing countries of Asia and
Figure 2. The mechanically ventilated (MV) GuillainBarre patients had insignifi-
America but on detailed nerve conduction studies, we have
cantly higher mortality and poor outcome compared with the patients who did
found AIDP as a predominant subtype of GBS.3,7,16,20,21
not require MV although had respiratory compromise.

Limitations
conservative criteria for mechanical ventilation, i.e. we relied
on ABG and have deferred intubation in those with respiratory The present study is limited by lack of spirometric assessment.
impairment but maintained ABG and those with bulbar We have relied on single breath count for defining respiratory
weakness. compromise instead of spirometry because it is not easily avail-
In GB syndrome, elective intubation has been recommended able bedside and even many mildly affected patients had diffi-
based on 20, 30 and 40 rule (vital capacity <20 ml/kg, maximal in- culty in performing spirometry test. Single breath count has
spiratory pressure <30 cm of water and maximum expiratory pres- been found to be a useful bedside pointer and correlated with
sure <40 cm of water).5 Delaying ventilation by waiting for hypoxia peak expiratory flow rate and forced expiratory pressure in 1 s
and hypercarbia to occur may result in emergent intubation result- in the adults.22 Similar observation has also been reported in
ing in complications.6 Intubation and artificial ventilation have in- children.23 Our study is a retrospective analysis in a tertiary
herent complications of pneumonia, pneumothorax and sepsis care teaching hospital and all the patients were examined by at
resulting in higher mortality and morbidity. In MGH study, 83% of least two of the authors. Our findings suggest that using ABG
ventilated patients had pneumonia, 5% each had pulmonary em- criteria, MV may be deferred in 55% patients with GBS who
bolism and tracheal stenosis but none died. In the Mayo clinic ser- otherwise would have been ventilated. This protocol seems to
ies out of 13 ventilated GBS patients; 38% had pneumonia, 5% be safe, reduce ventilator-related complications and may be ap-
pulmonary embolism, 7% tracheal stenosis and 15% patients died. plicable especially in resource poor countries. Further prospect-
Timely ventilation can reduce mortality by protecting airway and ive study is needed to confirm these observations.
minimizing atelectasis.6 In this study, the criteria of ventilation
were any of the following: ventilatory failure with reduced vital
Acknowledgements
capacity of <1215 ml/kg, pO2 below 70 mm of Hg on room air or
severe oropharyngeal paresis with difficulty in clearing secretion or We acknowledge Mr Rakesh Kumar Nigam and Deepak
repeated coughing or aspiration after swallowing. In the study by Kumar Anand for secretarial help.
Lawn et al.5, progression to respiratory failure is likely in the pa-
tients with rapid progression of weakness, bulbar dysfunction and
dysautonomia. Progression to respiratory failure is likely in those Ethical approval
patients having vital capacity <20 ml/kg, maximum inspiratory
The study PGI/BE/80/2014 has been approved by institu-
pressure <30 cm of water and maximum expiratory pressure <40
tional ethics committee, SGPGIMS, Lucknow.
cm of water or reduction of >30% in vital capacity, maximum in-
spiratory pressure or expiratory pressure. The predictors of respira- Conflict of interest: None declared.
tory failure in GBS have been reported in a number of studies and
include single breath count, neck weakness, bulbar weakness, limb
power <3 (MRC grade) on admission, simultaneous upper and
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