Guillain Barré Syndrome in the First Trimester of Pregnancy after Tetanus Toxoid Immunization: A Rare Case Report and Review of Literature

Abstract

Guillain Barré syndrome (GBS) is a rare neurological entity that presents as rapidly progressive symmetrical ascending demyelinating polyneuropathy. Its incidence is infrequent during pregnancy at 1.2 to 1.9 cases per 100,000 population. It is associated with very high maternal mortality and pregnancy complications. Its management during pregnancy is challenging as delay in diagnosis occurs due to rarity and overlapping symptoms. We report a case of a 23 year old primigravida who presented at 3 months of gestation with rapidly progressive lower limb paralysis after tetanus toxoid immunization. She was managed by a multidisciplinary team with intensive care admission, ventilator support, and intravenous immunoglobulins. She showed improvement after 4 weeks and was discharged in a stable condition with little residual weakness in her lower limbs. On follow up, she achieved full recovery. The outcome of the pregnancy was vaginal birth of a male child with no postpartum relapse. GBS is a rare entity during pregnancy and has a high maternal mortality rate. Hence, it is important to have a strong clinical suspicion and aggressive treatment for favorable maternal and fetal outcomes.

Keywords

GBS - pregnancy - tetanus toxoid

Introduction

Guillain Barré Syndrome (GBS) is a rare neurological condition that is even rarer in pregnancy, with a reported incidence of 1.2 to 1.9 per 100,000 population yearly.[1] It presents as immune mediated, rapidly progressive symmetrical peripheral neuropathy, with or without sensory or autonomic involvement. The most common type of GBS is acute inflammatory demyelinating polyneuropathy (AIDP). It is also the most common cause of acute flaccid paralysis. GBS is associated with 10% maternal mortality and 35% intensive care unit (ICU) admission rate with ventilator support in pregnant females.[2]

GBS has three subtypes: AIDP, acute motor axonal neuropathy, and Miller Fisher syndrome, a triad of ataxia, areflexia, and ophthalmoplegia.[3] [4]

The exact pathogenesis of GBS is still unclear, but GBS usually follows a triggering event such as infection, vaccination, or trauma. The accepted hypothesis is a molecular similarity between proteins on the cell wall of microorganisms and gangliosides of Schwann cells, resulting in demyelination.

Delayed hypersensitivity is reduced during pregnancy; hence, GBS is less common during pregnancy. Patients initially present with nonspecific symptoms such as malaise, weakness, tingling, and numbness that might mimic changes in pregnancy; hence, delay in diagnosis is common. GBS requires early diagnosis and intervention by a multidisciplinary team; otherwise, it can be detrimental to both mother and fetus.

Case Presentation

A 23 year old primigravida at 14 weeks of gestation presented to emergency with complaints of weakness in all four limbs, inability to stand up, and shortness of breath that initially started as lower limb weakness in the form of slippage of slippers, progressing to knee buckling, and requiring support to stand for the past 6 days. One day later, she noticed upper limb weakness as she was unable to grasp the objects. The next morning, she noticed difficulty in getting up from bed. Two days later, she had complaints of difficulty in opening her right eye and difficulty in swallowing. The patient presented to us when she developed shortness of breath. There were no associated bowel or bladder complaints and urinary or fecal incontinence. Our patient had received an injection of tetanus diphtheria toxoid (Td) at a nearby Anganwadi center 6 days before presenting symptoms. She did not have any antenatal health center visits and no known comorbidities before this illness.

On examination, her Glasgow coma scale was score E4V5M6, her pulse rate was 93 beats per minute, blood pressure was 130/80 mm Hg, respiratory rate was 25/min, SpO₂ was 98% on room air, and her temperature was 98.4°F. Both pupils were reactive to light. Ptosis was noted in the left eye, along with right lower motor neuron facial palsy, hypotonia, areflexia, power of 1/5 in both lower limbs and 2/5 in both upper limbs, flexor plantar response, reduced sensation of pain, and fine touch in both upper and lower limbs. Her cognition and cerebellar functions were normal. The abdomen was soft and nontender on palpation; the uterus was just palpable above the symphysis pubis.

The arterial blood gas analysis showed PCO₂ of 33 mm Hg, pH of 7.4, serum sodium of 135 mEq/L, potassium of 3.7 mEq/L, and HCO₃ of 21 mEq/L. The electrocardiogram (ECG) showed sinus arrhythmia, random blood sugar (RBS) was 127 mg/dL; routine biochemistry was normal. The patient was admitted to the neurology ICU for evaluation and management.

The patient's condition deteriorated further. She was not able to maintain saturation on room air, for which she was put on mechanical ventilation. Nerve conduction studies (NCS) showed increased distal latency, decreased amplitude, and conduction velocity with prolonged F wave latency in the median, ulnar, and tibial nerves on both sides, indicating generalized large fiber sensory motor demyelinating polyneuropathy. Cerebrospinal fluid (CSF) studies revealed reduced white blood cell counts and raised proteins, which were suggestive of albumin–cytological dissociation favoring the possibility of GBS. Her brain magnetic resonance imaging (MRI) was normal. The ganglioside panel was negative. An antenatal ultrasound done for fetal well being showed a 15 week single live intrauterine pregnancy.

The patient was started on physical therapy, thromboprophylaxis, and definitive management of GBS with five doses of intravenous immunoglobulin (IVIG) at 0.4 mg/kg/d. She developed autonomic instability, which was managed with β blockers. The patient developed fever and catheter related sepsis with methicillin resistant coagulase negative Staphylococcus aureus growth, which was treated with broad spectrum intravenous antibiotics. Ultrasound for fetal well being was done at 21 weeks of gestation, which showed a single live intrauterine pregnancy of 21 weeks with no gross congenital anomalies except for mild right renal pyelectasis (4.5 mm). On the same treatment, she gradually improved with decreased requirement of sedation and maintaining oxygen saturation and chest movements on room air.

The patient was discharged 1 week later at 22 weeks with no bed sores. There was hypotonia in all four limbs. The power in the upper limbs was 4/5, while in the lower limbs, proximal strength was 2/5, hip strength was 3/5, knee and ankle strengths were 1/5 and 2/5, respectively, neck flexor was 3/5 and extensor 4/5. Areflexia was observed with a mute bilateral plantar reflex. She was accepting diet orally. Foley's catheter was kept in situ at the time of discharge. She was advised to follow up after 2 weeks with a plan of a repeat NCS.

Follow Up

She underwent routine follow up appointments and achieved complete recovery without any residual weakness. She gave birth to a male baby weighing approximately 2,700 g at term with a vaginal delivery. There have been no relapses or signs of muscle weakness. Currently, her baby is 7 months old, and both mother and child are doing well.

Discussion

Patients of GBS mostly present with complaints of symmetrical ascending progressive muscle weakness after an event of gastrointestinal or respiratory infection within the past 5 to 6 weeks in approximately 60% of patients. A case of GBS in pregnancy after trivalent influenza vaccine administration has been reported. Our patient had a history of Td vaccination 6 days before the onset of symptoms. There was no travel history or signs and symptoms of gastrointestinal and respiratory tract infection. Other possible reasons cited in the literature are gastrointestinal infection, most commonly by Campylobacter jejuni, cytomegalovirus, varicella zoster virus, and Epstein–Barr virus. Owing to the overlapping of initial symptoms of GBS with pregnancy, there is a delay in diagnosis and seeking health care facilities, resulting in high maternal morbidity and mortality.

The diagnosis of GBS is mainly clinical, with the support of serological reports and NCS and CSF studies. The most common investigation is CSF examination, which shows albumin–cytological dissociation. In low resource settings, a diagnosis of GBS can also be made clinically using the criteria by the GBS classification group. Our patient's diagnosis was based on clinical symptoms and CSF examination. GBS and pregnancy can have overlapping symptoms, including general malaise, tingling in the fingers, muscle weakness, and respiratory difficulty. Our patient developed muscle weakness and respiratory difficulty initially.[5]

GBS in pregnancy is managed by supportive care with IVIG or plasmapheresis. One third of patients need respiratory support with mechanical ventilation associated with very high maternal mortality, most commonly from respiratory failure. Along with mechanical ventilation thromboprophylaxis, identification and treatment of infections, prevention of secondary infections, and psychosocial support play an important role in management. Plasmapheresis and IVIG are considered definitive treatment options for GBS. A systematic review in 2012 reported that plasma exchange has better results than supportive treatment alone without an increase in significant adverse events but is associated with a higher rate of relapse within 1 year. Our patient was managed by mechanical ventilation, IVIG, prophylactic low molecular weight heparin, progesterone supplements, antibiotics, analgesics, physiotherapy, and β blockers.

In reported cases, vertical transmission of GBS is not seen and not associated with specific fetal congenital malformations, growth restriction, or intrauterine fetal demise. Also, GBS more commonly occurred in the third trimester and within 2 weeks of delivery in multigravidas.

To conclude, GBS is rarer during pregnancy, and there is a need to keep a very high index of suspicion to make an early diagnosis. Patients should be managed by a multidisciplinary team with plasma exchange and immunoglobulins, along with supportive care. This case was similarly managed with a favorable outcome. GBS is difficult to manage in a low resource setting because of the prolonged hospitalization, mechanical ventilation, and slow recovery. Despite best efforts, there is a very high rate of maternal and perinatal mortality.

Conflict of Interest

None declared.

• References

• 1 Sharma SR, Sharma N, Masaraf H, Singh SA. Guillain-Barré syndrome complicating pregnancy and correlation with maternal and fetal outcome in North Eastern India: a retrospective study. Ann Indian Acad Neurol 2015; 18 (02) 215-218
• 2 Nelson LH, McLean Jr WT. Management of Landry-Guillain-Barré syndrome in pregnancy. Obstet Gynecol 1985; 65 (3, Suppl): 25S-29S
• 3 Mori M, Kuwabara S, Yuki N. Fisher syndrome: clinical features, immunopathogenesis and management. Expert Rev Neurother 2012; 12 (01) 39-51
• 4 Kuwabara S, Yuki N. Axonal Guillain-Barré syndrome: concepts and controversies. Lancet Neurol 2013; 12 (12) 1180-1188
• 5 Pacheco LD, Saad AF, Hankins GD, Chiosi G, Saade G. Guillain-Barré syndrome in pregnancy. Obstet Gynecol 2016; 128 (05) 1105-1110

Publikationsverlauf

Artikel online veröffentlicht:
19. März 2025