
==== Front
Ann Med Surg (Lond)
Ann Med Surg (Lond)
MS9
Annals of Medicine and Surgery
2049-0801
Lippincott Williams & Wilkins Hagerstown, MD

10.1097/MS9.0000000000000236
00029
3
Case Reports
Guillain-Barré syndrome complicating early pregnancy: a case report from Nepal
http://orcid.org/0000-0002-4689-9264
Aryal Roshan MBBS aroshanaryal7@gmail.com

Adhikari Aayush MBBS aaayushadhikari61@gmail.com

http://orcid.org/0000-0001-9030-1488
Jha Kritika MD bjkrts02@gmail.com

Deo Bikash MBBS adeobikash98@gmail.com

Kafle Yashaswi MBBS yashukafle123@gmail.com
c
Ojha Rajeev MD, DM drajeevnet@hotmail.com

a Maharajgunj Medical Campus, Institute of Medicine
b Department of Obstetrics and Gynecology, Tribhuvan University Teaching Hospital, Maharajgunj, Kathmandu
c Chitwan Medical College, Bharatpur, Chitwan
d Department of Neurology, Tribhuvan University Teaching Hospital, Maharajgunj, Kathmandu, Nepal
* Corresponding author. Address: Institute of Medicine, Maharajgunj Medical Campus, Maharajgunj, Kathmandu, Nepal. P.O. Box: 44600. Tel.: +977-9860012492. E-mail address: roshanaryal7@gmail.com (R. Aryal).
3 2023
9 3 2023
85 3 497500
6 12 2022
25 12 2022
Copyright © 2023 The Author(s). Published by Wolters Kluwer Health, Inc.
2023
https://creativecommons.org/licenses/by-nc-sa/4.0/ This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as the author is credited and the new creations are licensed under the identical terms. http://creativecommons.org/licenses/by-nc-sa/4.0/

Introduction and Importance:

Guillain-Barré syndrome (GBS) is an immunological demyelinating disorder characterized by progressive, ascending flaccid weakness, usually resulting after infection or some immune stimulation. Its occurrence during pregnancy is rare and due to attribution of its symptoms to pregnancy, diagnosis might be delayed.

Case Presentation:

A 39-year-old G4P3L2A0 woman at 13 weeks 6 days period of gestation presented with acute, symmetrical, ascending type of flaccid quadriparesis leading to slurring of speech, swallowing difficulty, and eventually respiratory failure. With the diagnosis of GBS, she was admitted to the intensive care unit, five sessions of plasma exchange were done along with physiotherapy and her symptoms started improving. After discharge she was on regular antenatal care visits and eventually, she delivered a healthy baby at term with an uneventful labor event and postpartum period.

Clinical Discussion:

There is a huge maternal-fetal risk of respiratory failure, aspiration pneumonia, preterm delivery, possible use of forceps or vacuum, and operative interference due to GBS in pregnancy, whose causative agents are similar to that of the general population. The swinging pattern of risk of GBS in pregnancy might be due to immunological changes with predominant Th2 response seen in pregnancy. Treatment measures are similar as in the general population with initial symptomatic care and administration of disease-specific therapy later which consists of intravenous immunoglobulin and/or plasmapheresis.

Conclusion:

A well-timed diagnosis of GBS in pregnancy might allow successful management with the help of intensive monitoring with or without immunotherapy.

Keywords:

Case report
Guillain-Barré syndrome
Neuropathy
Pregnancy
OPEN-ACCESSTRUE
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pmcHighlights

Guillain-Barré syndrome (GBS), an immune-mediated demyelinating neurological disorder is rare in pregnancy and its symptoms can be attributed to pregnancy.

Maternal-fetal risk of respiratory failure, aspiration pneumonia, preterm delivery, likely use of forceps or vacuum is huge.

The fluctuating risk of GBS during various trimester of pregnancy might be due to change in immunogenicity in pregnancy.

Once diagnosed the treatment modality is initial symptomatic care and administration of disease-specific therapy later.

Disease-specific therapy consists of intravenous immunoglobulins (IVIGs) and plasmapheresis.

Introduction

GBS is a rare, heterogeneous group of an immune-mediated demyelinating neurological disorder of peripheral nervous system, the annual global incidence being around one to two per 100 000 person-years1. The typical presentation is lower limb weakness and sensory signs progressing to arms and cranial muscles, however, heterogeneous clinical presentation owing to different variants of this disease do exist1,2. This disease usually results after an infection or some immune stimulation, both of which leads to an anomalous immune response thereby attacking peripheral nerves2. Being a rare condition itself, it is even rarer during pregnancy, however, the prevalence is similar as in the general population3. Especially in the first trimester, the nonspecific complaints of weakness and numbness of limbs are often wrongly attributed to pregnancy which may delay the diagnosis3,4. GBS onset varies in obstetric populations, with around 13% occurring in the first trimester, around 47% in the second trimester and 40% in the third trimester3,4.

Although being rare in pregnancy, GBS can be managed successfully with good outcomes for both mother and child. However, this may not be a situation when timely diagnosis and early intervention is lacking. Here we report a case of GBS complicating the first trimester of pregnancy successfully managed with plasmapheresis and ventilatory support. This case has been reported in line with SCARE criteria5.

Presentation of case

A G4P3L2A0 39-year-old lady at 13 weeks 6 days period of gestation presented to emergency with acute onset of weakness in bilateral lower limbs, preceded by burning sensation. Weakness started on the lower limbs with not being able to stand from sitting position and difficulty to put on slippers and walk properly. It further progressed causing weakness of both upper limbs and then slurring of speech, difficulty in swallowing, and regurgitation of foods. She did not have an altered sensorium, shortness of breath, abnormal body movements, and her bladder and bowel habits were normal. The patient had a history of sore throat 1 week before hospital presentation but there was no history of preceding diarrhea, vaccination, or travel. Prior pregnancies were uneventful and had no similar complaints. Family history was non-contributory.

On physical examination, the patient was conscious, afebrile with heart rate 86 beats/min, blood pressure measuring 130/80 mmHg, respiratory rate 12 breaths/min, and oxygen saturation 96% in room air. There was a complete absence of deep tendon reflexes in all the limbs. Muscle power examination showed a Medical Research Scale (MRC) of 3/5 in proximal, 4-/5 in distal of upper extremities and 1/5 in proximal, 2/5 in distal of lower extremities. Facial nerve examination revealed bilateral lower motor neuron type palsy (House-Brackmann grade 3). No signs of meningeal irritation were present. Pain, fine touch, vibration, and temperature sensation was normal in both upper and lower limbs.

Ultrasonographic evaluation of the fetus showed a single live fetus at 14+6 weeks of gestation. Her blood examinations were as follows: total leukocyte count 12 700/mm3 [neutrophils 80% and lymphocytes 15% (normal: 4000–1100/mm3)]; hemoglobin 15.3 g/dl (normal: 12.5–17 g/dl); red blood cell 4.8 million/mm3 (normal: 4.5–5.5 million/mm3); platelets 197 000/mm3 (normal: 150 000–450 000/mm3); sodium 137 mEq/L (normal: 135–145 mEq/l); potassium 3.5 mEq/L (normal: 3.5–5 mEq/L) and random sugar 5.3 mmol/l (normal: 3.8–7.8 mmol/l). Her prothrombin time was 15 s and international normalized ratio was 1.25 s. Her hepatic and renal function tests were in normal range. Chest radiograph and electrocardiograph findings were normal. Other laboratory tests including hepatitis B and C, HIV, syphilis, and thyroid function tests were unremarkable. Examination of the cerebrospinal fluid showed protein 160 g/dl, sugar 64 mg/dl, and total cells count of 4 (polymorphs 0% and leukocytes 100%). Nerve conduction study could not be performed, due to unavailability in the inpatient settings.

With strong clinical suspicion of GBS and for respiratory monitoring, the patient was admitted directly to the ICU. The patient was strictly monitored for the signs of respiratory failure. She developed respiratory failure on the night of admission and was started on mechanical ventilation after endotracheal intubation and thromboprophylaxis for prevention of deep venous thrombosis. Periodic ultrasound scanning and cardiotocography for fetal assessment revealed expected fetal well-being and growth. Therapeutic plasma exchange was started on the same day of admission as the first line of treatment. Plasma exchange was done on alternate days, with a total of five sessions. The patient had no complications during these sessions. After five sessions of plasma exchange, the patient’s weakness of limbs and respiratory pattern gradually improved and was weaned off the ventilator and physiotherapy maintained. After 20 days of hospital stay the patient was discharged with the power of 4/5 in upper limbs and 4-/5 in lower limbs with improved slurring of speech and swallowing symptoms. She was on her regular antenatal care visits and on follow-up after 2 months of discharge there was no residual weakness. Patient gave birth to a single live male child of 2300 g at 39 weeks 3 days of gestation through vaginal delivery. There was no issue regarding maternal efforts during labor. The postpartum period was also uneventful.

Discussion

GBS in pregnancy carries a huge maternal-fetal risk due to chances of respiratory failure and aspiration pneumonitis in the mother and preterm delivery of the baby6,7. The majority of the patients present with the history of progressive ascending muscle weakness which is preceded mostly by an infection (flu-like illness, gastroenteritis) in the last 6 weeks6. The causative agents mostly incriminated are similar as in the general population and include Mycoplasma pneumonia, Cytomegalovirus, Campylobacter jejuni, and Epstein–Barr virus6. In our case, the patient gave a history of sore throat a week back before development of weakness.

Although GBS can occur during any trimester of pregnancy and postpartum period, the third trimester and the initial 2 weeks postpartum period are particularly vulnerable as there are relative protective effects of pregnancy during the first months8. Occurrence and worsening during the postpartum period are likely due to increase in the delayed-type hypersensitivity8. However, our patient was diagnosed with GBS in early pregnancy. Vijayaraghavan et al. 9 has reported a case focusing on the management of GBS at 16 weeks of pregnancy. The undulating pattern of risk of GBS in pregnancy could be due to transfer of endogenous antigens whereby fetal cells go to the maternal world and cause immunological response10. Further downregulation of Th1 response with relative temporary enhancement of production of Th2 cytokines during pregnancy could also facilitate the development of immune response as indicated in the pathogenesis of autoimmune diseases like multiple sclerosis and rheumatoid arthritis11,12.

As in the nonpregnant population, diagnosis in the obstetric population is mostly based on pertinent clinical findings of acute progression of weakness beginning in the legs, concurrent with sensory and cranial nerve involvement and the peak clinical deficit in 2–4 weeks2,7. Diagnosis is further confirmed by an acellular elevation of protein in cerebrospinal fluid2,7. Our patient satisfies the level 2 diagnostic certainty of Brighton criteria for GBS with all the features except the nerve conduction findings which was not done in our case13. The Brighton diagnostic criteria for GBS can be found in Table 1 13.

Table 1 Brighton diagnostic criteria for GBS13

	Level of diagnostic certainty	
Diagnostic criteria	Level 1	Level 2	Level 3	Level 4	
Absence of alternative diagnosis for weakness	+	+	+	+	
Diminished or absent deep tendon reflex in weak limbs	+	+	+	+/−	
Monophasic course and time between onset and nadir, 12 hours to 28 days	+	+	+	+/−	
Bilateral and flaccid weakness of limbs	+	+	+	+/−	
CSF cell count <50 cells/μl	+	+*	−	+/−	
CSF protein concentration>normal value	+	+/−*	−	+/−	
NCS findings consistent with one of the subtypes of GBS	+	+/−	−	+/−	
* If CSF is not collected or results not available, nerve electrophysiology results must be consistent with the diagnosis GBS.

+ indicates present; −, absent; +/−, present or absent; CSF, cerebrospinal fluid; GBS, Guillain-Barré syndrome; NCS, nerve conduction study.

As per the disease severity at the time of diagnosis, patients need to be monitored in general wards or in ICUs for evaluation of the progression of disease, focusing on worsening respiratory status, bulbar, and autonomic dysfunctions7. Treatment measures are similar as in the general population with initial symptomatic care and administration of disease-specific therapy later7. Erasmus GBS Respiratory Insufficiency Score (EGRIS) on hospital admission can help with this decision-making as it delineates those requiring artificial ventilation14. In our patient, the onset and hospital admission were less than 3 days with the presence of bulbar symptoms and MRC sum falling between 0 and 20, leading to EGRIS score of 7 pointing to the risk of 88% respiratory failure in the first week after onset of symptoms. Around 25% of the patients with GBS have involvement of respiratory muscles/develop respiratory failure requiring endotracheal intubation and mechanical ventilation in the ICU13. Our patient developed respiratory failure on the day of admission and required endotracheal intubation and mechanical ventilation.

We managed our patient on mechanical ventilation, five cycles of plasmapheresis along with supportive treatments and physiotherapy. Respiratory function can be even more compromised in pregnancy due to splinting of the diaphragm15. It has also been found that risk of premature birth increases significantly in cases requiring ventilatory support16. In regards to pregnancy management, however, it was seen that termination of pregnancy did not hasten the recovery of maternal disease nor improved maternal outcomes, pointing to the fact that GBS itself is not an indication for termination of pregnancy4. Lack of maternal pushing efforts may require use of forceps or vacuum17. Our patient did not require any forceps or vacuum assistance and gave birth through spontaneous vaginal delivery. Pain control is essential regarding the notion of exaggerated hemodynamic response to labor pain in patients with GBS17. About two-thirds of the patients can have very severe pain which can last for months, however, there is no proven specific pharmacologic intervention till date18.

Results from several randomized controlled trials studying the role of immunotherapy for GBS have proved both plasmapheresis and IVIG effective2. Earlier administration of these modalities before irreversible nerve damage has occurred should be implicated principally2. Disease-specific therapy including plasmapheresis or IVIG is proven to be of benefit when administered within 4 weeks of onset of symptoms with maximal benefit being found when used within initial 2 weeks4. Being more convenient, IVIG is favored as it does not require placement of a large-bore central venous catheter and it is also associated with less complications7. However, the cost of plasma exchange is much less as compared with IVIG with a similar outcome which becomes significant in Nepalese setup. Hence, we opted for plasmapheresis as the initial choice of specific treatment.

In about 10% of patients achieving disease-specific therapy, there can be further worsening of the condition after initial improvement19. This is referred to as “treatment-related fluctuation” which requires 2 g/kg IVIG over 5 days19. No worsening of the condition was seen in our case after initial improvement with the use of plasma exchange in our patient. In the days to come, agents such as eculizumab may be the newer modalities of treatment19,20.

Conclusion

Depending upon the severity at the time of diagnosis of GBS, pregnant women might have to be admitted to an intensive care unit or general ward for treatment and monitoring. There can be risks of respiratory failure and aspiration pneumonitis in mother and preterm delivery of baby, but GBS can be successfully managed with intensive monitoring with or without immunotherapy if the timely diagnosis is made.

Ethical approval

Not required.

Consent for publication

Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal on request.

Sources of funding

None.

Author’s contribution

K.J. and R.O.: provided counseling and treatment to the patient, and contributed to the collection of case information. R.A., A.A., and K.J.: conceptualization, collected all the required case information, and reports; reviewed the literature and contributed in writing the original draft. R.A., A.A., K.J., Y.K., and B.D.: writing and editing the manuscript. R.O.: senior author and manuscript reviewer. All the authors read and approved the final manuscript.

Conflicts of interest disclosure

The authors declare that they have no financial conflict of interest with regard to the content of this report.

Research registration unique identifying number (UIN)

Not applicable.

Guarantor

Roshan Aryal.

Provenance and peer review

Not commissioned, externally peer-reviewed.

Sponsorships or competing interests that may be relevant to content are disclosed at the end of this article.

Published online 9 March 2023
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References

1 Sejvar JJ Baughman AL Wise M . Population incidence of Guillain-Barré syndrome: a systematic review and meta-analysis. Neuroepidemiology 2011;36 :123–133.21422765
2 Willison HJ Jacobs BC van Doorn PA . Guillain-Barré syndrome. Lancet 2016;388 :717–727.26948435
3 Chan LYS Tsui MHY Leung TN . Guillain-Barré syndrome in pregnancy. Acta Obstet Gynecol Scand 2004;83 :319–325.15005776
4 Nelson LH McLean WT Jr . Management of Landry-Guillain-Barré syndrome in pregnancy. Obstet Gynecol 1985;65 (Suppl ):25S–29S.3919349
5 Agha RA Franchi T Sohrabi C . SCARE Group. The SCARE 2020 Guideline: Updating Consensus Surgical CAse REport (SCARE) Guidelines. Int J Surg 2020;84 :226–230.33181358
6 Sheikh KA . Guillain-Barré Syndrome. Continuum 2020;26 :1184–1204.33002998
7 Pacheco LD Saad AF Hankins GD . Guillain-Barré syndrome in pregnancy. Obstet Anesth Dig 2017;37 :63.
8 Zeeman GG . A case of acute inflammatory demyelinating polyradiculoneuropathy in early pregnancy. Am J Perinatol 2001;18 :213–215.11444365
9 Vijayaraghavan J Vasudevan D Sadique N . A rare case of Guillain-Barre syndrome with pregnancy. J Indian Med Assoc 2006;104 :269–270.17058576
10 Rolfs A Bolik A . Guillain-Barré syndrome in pregnancy: reflections on immunopathogenesis. Acta Neurol Scand 1994;89 :400–402.8085441
11 Raghupathy R . Th1-type immunity is incompatible with successful pregnancy. Immunol Today 1997;18 :478–482.9357139
12 Feldmann M Brennan FM Maini RN . Rheumatoid arthritis. Cell 1996;85 :307–310.8616886
13 Fokke C van den Berg B Drenthen J . Diagnosis of Guillain-Barré syndrome and validation of Brighton criteria. Brain 2014;137 (Pt 1 ):33–43.24163275
14 Walgaard C Lingsma HF Ruts L . Prediction of respiratory insufficiency in Guillain-Barré syndrome. Ann Neurol 2010;67 :781–787.20517939
15 Gautier PE Hantson P Vekemans MC . Intensive care management of Guillain-Barré syndrome during pregnancy. Intensive Care Med 1990;16 :460–462.2269716
16 Brooks H Christian AS May AE . Pregnancy, anaesthesia and Guillain Barré syndrome. Anaesthesia 2000;55 :894–898.10947755
17 Wiertlewski S Magot A Drapier S . Worsening of neurologic symptoms after epidural anesthesia for labor in a Guillain-Barr patient. Anesth Analg 2004;98 :825–827.14980945
18 Ruts L Drenthen J Jongen JLM . Pain in Guillain-Barre syndrome: a long-term follow-up study. Neurology 2010;75 :1439–1447.20861454
19 van den Berg B Walgaard C Drenthen J . Guillain-Barré syndrome: pathogenesis, diagnosis, treatment and prognosis. Nat Rev Neurol 2014;10 :469–482.25023340
20 Halstead SK Zitman FMP Humphreys PD . Eculizumab prevents anti-ganglioside antibody-mediated neuropathy in a murine model. Brain 2008;131 :1197–1208.18184663
