A case of fulminant Guillain-Barré syndrome presenting as brain death

INTRODUCTION

Guillian Barré syndrome has an incidence of 0.3 per 100,000 in the general population which increases to 30.4 per 100,000 following Campylobacter jejuni infection.¹ Polyradiculopathy following Campylobacter jejuni gastroenteritis is often severe, necessitating ventilator and intensive care management. Fulminant forms of Guillain Barré syndrome present with respiratory paralysis and progress relentlessly despite intubation and life support with an uncertain outcome as is seen in this case report. There are only twenty cases reported in the literature so far, hence this case illustrates the problems encountered during the treatment of a rare disease.

CASE REPORT

A 15-year-old boy presented in January 2025 with fever, cough, hoarseness of voice followed by rapidly progressive quadriparesis and bulbar involvement over four hours. He had a history of recovery from Guillain Barré syndrome in 2024 with nerve conduction studies revealing demyelinating polyneuropathy with conduction blocks in bilateral lower limbs (Table 1).

On day 1, at casualty admission, there were grade 3/ 5 power of all four limbs, neck, trunk weakness, areflexia and reduced respiratory excursions requiring ventilation. As there was rapid onset bulbar involvement, investigations were done to exclude recurrent Guillain Barré syndrome, botulism, brainstem encephalitis, tick paralysis and organophosphorus poisoning.

On day 2, there was bilateral total ophthalmoplegia, fixed and dilated pupils, absent corneal and oculocephalic reflexes, hypotonia and areflexia with flexor plantar responses. His lumbar puncture was normal and the nerve conduction studies revealed the sensory motor axonal polyneuropathy (Table 1). On day 10, there was no improvement in his clinical condition, he had a low GCS E1M1VET, pulse 110/min, BP 150/100 mmHg, Temperature 37⁰C, respiratory rate 20/min on ventilatory support. Pupils were fixed and dilated with absent brainstem reflexes and no papilledema. He had flaccid, areflexic quadriparesis with grade 0/5 power of all four limbs, bilateral flexor plantars and no meningeal irritation. Urgent CT angiography of the brain done from the emergency unit showed normal cerebral perfusion and electroencephalography revealed cerebral dysfunction in bilateral posterior regions (Table 1). On Day 14, his pupils became unequal, right > left, there was dysautonomia with heart rate and blood pressure fluctuations and no spontaneous respiratory effort. Repeat cerebrospinal fluid showed albuminocytological dissociation, with negative autoantibody and antiganglioside antibody profile (Table 1). Contrast MRI of lumbosacral spine sagittal and axial views revealed enhancement of the cauda equina (Figure 1). He remained quadriplegic with no brainstem signs, no trigger on ventilation and he was unresponsive to external stimuli. On day 24, tracheostomy was done. On day 26, there were jaw and vertical eye movements. On day 29, he had eye fluttering with head nodding, jaw and tongue movements. On day 30, there was bilateral finger flexion, yes and no head nodding movements and triggering of the ventilator. On day 40, there were elbow flexion movements and response to simple commands. On day 60, he was on tracheostomy, off ventilator with lateral movements of his upper and lower extremities and on day 90 he was standing with support. There was a history of Guillain Barré syndrome nine months ago from which patient recovered and attended normal school. He was the only child of non-consanguineous parents born following invitro fertilization with normal birth and development history.

Management and outcome

Guillain Barré mimics were excluded, as stool biofire was positive for Campylobacter jejuni and nerve conduction study revealed sensorimotor axonal polyneuropathy (Table 1), patient received intravenous immunoglobulins 20 grams daily for five days followed by intravenous methyl prednisolone 1 gram daily for five days. There was further worsening of neurological status on day 10, so plasmapheresis and supportive treatment were initiated. He received a total of six cycles of plasma exchange on alternate days followed by repeat intravenous immunoglobulin 0.4 gram/ kg body weight for another five days. Tracheostomy was done on day 24 to enable weaning from the ventilator. Meanwhile low molecular weight heparin prophylaxis, antibiotics to prevent ventilator associated pneumonia, Ryles tube feeds, prevention of deep venous thrombosis and bedsores, care of bowel and bladder, chest and limb physiotherapy were done. Patient was extubated and made a slow and steady recovery over three months. At present, he is off tracheostomy and walking with support.

DISCUSSION

Campylobacter jejuni, a gram-negative rod-shaped bacteria causes diarrheal illness in 400 to 500 million cases annually.¹ Campylobacter jejuni causes Guillain Barré syndrome in 33% cases and the organism is detected by stool cultures, polymerase chain reaction or by enzyme immunoassay.¹ Ascending polyradiculoneuropathy occurs three weeks after the diarrhoeal illness.¹ The above-mentioned patient presented with a rapid-onset quadriplegia, respiratory failure and coma mimicking brain death necessitating investigations to exclude brainstem encephalitis, posterior circulation stroke, botulism, poisoning, fulminant myasthenia gravis and Guillain Barré syndrome. Brain death was excluded as no clear etiology was available, there was normal blood flow in CT angiogram of brain and EEG showed a mild posterior cerebral dysfunction. Contrast MRI brain was normal and anti-GQ1 b antibodies were negative disfavoring brainstem encephalitis. Metabolic and toxin etiologies were negative. There was a previous history of Guillain Barré syndrome with rapidly ascending weakness, abnormal nerve conduction studies followed by inexcitable responses, albuminocytological dissociation of cerebrospinal fluid, cauda equina enhancement in MRI spine and Campylobacter jejuni isolated in stool samples, favoring the diagnosis of Guillain Barré syndrome and justifying prompt treatment.

Pathogenic cell wall lipo-oligosaccharides of Campylobacter jejuni trigger an immune response through toll-like-receptor 4 signals which cross react with antibodies against gangliosides [sialic-acid containing glycolipids] located at the nodes of Ranvier and at the nerve terminals.² The proximal nerve roots and distal nerve terminals lack a blood nerve barrier thus increasing the vulnerability to immune mediated injury.³ Nodal-paranodal antibodies interrupt salutatory conduction along the paranodes by detaching terminal myelin loops and interrupting sodium channels.⁴ This causes slowing of nerve conduction, temporal dispersion and reduction of the amplitude of compound motor action potentials.⁴

The 8.8% of patients with fulminant Guillain Barré syndrome follow campylobacter infection and progress rapidly despite immunotherapy.⁴ 3-8% mortality is attributed to the severity of neurological involvement, dysautonomia, cardiac arrhythmias, hypotension and shock.⁵

Brain death caused by motor axonal polyneuropathy has high mortality and morbidity hence identification and timely treatment with intravenous immunoglobulins or plasmapheresis and life support systems are necessary.⁵

Reviewing the literature, a clinically deafferented state caused by fulminant polyneuropathy has been reported by many authors.⁶ Carrol et al described the first case of polyneuropathy induced locked-in state in 1979, in whom there was gradual recovery over twelve months following treatment.⁶ Bakshi et al described the case of a 6-year-old child who responded to treatment with intravenous immunoglobulins after being diagnosed with clinically brain dead state wherein nerve conductions and nerve biopsies were suggestive of demyelinating polyneuropathy.⁶ Vargas et al in 2000 has described the outcome of 13 patients of fulminant polyneuropathy of which two patients died of dysautonomia, one died of anterior wall myocardial infarction, two patients recovered with minimal deficits and the remainder were severely disabled despite appropriate treatment.⁷

Nithyashree et al graded the prognosis of fulminant Guillian Barré syndrome based on the presence of six factors which included, age over sixty years, prodromal diarrhoea, rapid onset respiratory paralysis requiring ventilation in twenty-four hours, severe muscle weakness with grade 0 power in all four limbs, cardiovascular autonomic symptoms and inexcitable peripheral nerve conductions.⁸

CONCLUSION

The treating physician was convinced that this young boy had a recurrence of Guillain Barré syndrome so he persevered with treatment despite absent brainstem reflexes. A high index of suspicion is to be maintained in young onset, aggressive quadriparesis presenting with brain death as treatment can reverse antibody mediated attack and return bulbar and motor functions to normal. Our medical team put their best efforts to revive this patient as there was a past history of Guillain Barré syndrome. This case was an eye-opener, as prompt and aggressive therapy prolonged the youngster’s life and gave a ray of hope to his parents who endured all hurdles to save his life. It is important to improve awareness among clinicians and medical practitioners about such rare cases where medical intervention stands the test of time. The limitations of the management were that this was a rare presentation of Guillain Barré Syndrome with unpredictable results.

Funding: No funding sources

Conflict of interest: None declared

Ethical approval: Not required

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