Guillain-Barre Syndrome or Is It?

Sarenthia Epps, MD, MBA, Fellow, Department of Pulmonary and Critical Care, University of
Arkansas for Medical Sciences (UAMS), Little Rock, Arkansas. Email: smepps@uams.edu

Lovepreet Singh, MD, Resident, Hospitalist, Department of Internal Medicine, Baptist Health,
Little Rock, Arkansas. Email: lovepreetsingh7006@gmail.com

Harmeen Goraya, MD, Assistant Professor, Department of Pulmonary and Critical Care,
University of Arkansas for Medical Sciences (UAMS), Little Rock, Arkansas. Email:
hgoraya@uams.edu

Rajani Jagana, MD, Assistant Professor, Department of Pulmonary and Critical Care, University
of Arkansas for Medical Sciences (UAMS), Little Rock, Arkansas. Email: rjagana@uams.edu

Conflicts of Interest and Disclosures: None

Grant Support and Writing Assistance: None
Keywords: GBS, AIDP, West Nile Virus, Guillian-Barre Syndrome
Address for Correspondence:
Sarenthia McClelland, MD, MBA
Department of Pulmonary and Critical Care
4301 West Markham Street Slot 547-19, Little Rock, AR 72205
Email: smmcclelland@uams.edu
Abstract
West Nile Virus has become a common cause of meningoencephalitis in humans in North
America. The virus is transmitted from infected birds to humans via mosquitoes and typically
manifests during the summer months in North America. West Nile Virus can be difficult to
diagnose as signs and symptoms of West Nile Virus encephalitis can range from non-specific
symptoms, asymptomatic, and even death. West Nile Virus encephalitis can also mimic
neuromuscular disorders, and patients can present with encephalitis, respiratory weakness, and
ascending paralysis as in this case below.

Introduction

Respiratory failure secondary to neuromuscular weakness is difficult to diagnose when patients

present with acute respiratory illness requiring mechanical ventilation. A high degree of
suspicion and extensive workup is needed in most of these cases. Broad differential diagnoses
should be part of the workup. We present a case of neuromuscular weakness secondary to
infectious encephalitis, that presented as typical Guillain-Barre syndrome (GBS). We present a
case with an uncommon presentation of West Nile Meningo-Encephalitis with limited treatment
options. 

Case Presentation

55-year-old African American man with hypertension and polysubstance abuse presented to an
outside hospital for evaluation of multiple falls. The patient was found to have rhabdomyolysis,
dehydration, acute kidney injury, and a urinary tract infection and was admitted to a general
medicine floor. The patient developed mental status changes, respiratory distress, and atrial
flutter with RVR was subsequently intubated and transferred to the intensive care unit. CT PE
was negative for pulmonary embolism but showed a right lower lobe consolidation. The patient
remained intubated for eight days and was extubated, but subsequently re-intubated on the same
day secondary to respiratory distress. Sputum culture was positive for multi-drug resistant
Acinetobacter baumannii, which prompted the initiation of tobramycin and tigecycline as per
susceptibilities. While at the outside hospital, there was a concern for neuromuscular weakness
being the etiology of failure to wean from the ventilator. Guillain-Barre syndrome (GBS) was
suspected due to flaccid paralysis of all four extremities post-viral illness. The patient was started
on pyridostigmine and ACh antibodies were obtained, and later returned negative, along with
ANA and ANCA. The patient was transferred to a tertiary hospital for a higher level of care.
 
The patient continued to have leukocytosis with left shift, hyperkalemia, hyperphosphatemia
with non-oliguric kidney failure. The patient continued to require high FiO2 on mechanical
ventilation. Repeat CT chest showed extensive multifocal pneumonia with mucous plugging
causing partial occlusion of the left lower lobe bronchus. The patient underwent bronchoscopy
with bronchoalveolar lavage that returned positive for multi-drug resistant Acinetobacter
baumanii. The Infectious Disease team recommended continuing tigecycline, tobramycin, and
start inhaled tobramycin. Given concern for a neuromuscular disorder and failure to wean from
the ventilator, neurology was consulted. MRI of the cervical spine obtained and showed
enhancement and subtle enlargement of the anterior lumbar nerve roots, consistent with Guillain-
Barre syndrome. Lumbar puncture was also consistent with GBS and the patient was initiated on
plasma exchange (PLEX) for five sessions. After a few days, West Nile IgM and IgG antibodies
on spinal fluid returned positive. The patient’s hospital course remained complicated by
difficulty weaning from the ventilator, hyperkalemia, metabolic acidosis, and quadriparesis with
decreased tone and intact reflexes. Tracheostomy was placed as the patient was unable to wean
from ventilator support.
 
The patient was diagnosed with acute flaccid paralysis associated with West Nile Virus
encephalitis. After completed five PLEX sessions, the patient was found to have some
improvement in his neurological status and neurology recommended five additional PLEX
sessions. The patient’s neurological status continued to improve in terms of mental status;
however, there was only mild improvement in muscle weakness. Motor power in distal upper
extremities improved compared to lower extremities and the patient was referred to a spinal
rehabilitation program at the time of hospital discharge.

 Discussion

West Nile Virus (WNV), is a zoonotic arbovirus of the Flaviviridae family.1 It transmits itself
from infected birds to humans via mosquitoes.1 It was first isolated in Uganda in 1937 from the
blood of a febrile woman.2 WNV has become the common cause of epidemic
meningoencephalitis in humans in North America.2 Mortality and incidence of neurological
sequelae among affected patients is very high. At present, there is no effective therapy for the
treatment of WNV encephalitis.2

Patients who present with altered mental status or neurological symptoms, suspicion should be
high for viral encephalitis in appropriate settings. Signs or symptoms of WNV encephalitis can
be very nonspecific and can vary from asymptomatic to death. Neuro-invasive disease accounts
for less than 1% of WNV cases but can result in severe neurological sequelae and even death.1
Acute flaccid paralysis is a rare but serious complication of West Nile virus infection.3 The
prognosis of acute flaccid paralysis in such patients is unclear at this time.3 The literature is very
limited regarding the role of therapeutic plasma exchange in the management of WNV
encephalitis with opsoclonus myoclonus syndrome (which is not present in this patient), with
rare reports of benefit after five sessions.4  There are case reports showing a benefit of IVIG in
the literature but this benefit was not found to be statistically significant when larger studies were
performed.5

Conclusions

This case illustrates the importance of maintaining a broad differential diagnosis in patients
presenting with altered mental status and acute respiratory failure requiring mechanical
ventilation. History, physical exam, and laboratory analysis remain critical elements in
formulating the diagnosis of West Nile Virus Encephalitis. In this case, the lumbar puncture was
vital in identifying the true cause of the patient’s ascending paralysis and respiratory failure as
the pathophysiology for West Nile Virus Encephalitis differs from Guillan-Barre Syndrome
References

1. Hart, J., Tillman, G., Kraut, M. A., Chiang, H.-S., Strain, J. F., Li, Y., … Whitley, R. J.
(2014). West Nile virus neuroinvasive disease: neurological manifestations and
prospective longitudinal outcomes. BMC Infectious Diseases, 14(1). doi: 10.1186/1471-
2334-14-248
2. Debiasi, R. L., & Tyler, K. L. (2006). West Nile virus meningoencephalitis. Nature
clinical practice. Neurology, 2(5), 264–275. doi:10.1038/ncpneuro0176

3. Johnstone, J., Hanna, S. E., Nicolle, L. E., et al. (2011). Prognosis of West Nile virus
associated acute flaccid paralysis: a case series. Journal of Medical Case Reports, 5(1),
395. https://doi.org/10.1186/17521947-5-395

4. Cooper, C. J., & Said, S. (2014). West Nile virus encephalitis induced opsoclonus-
myoclonus syndrome. Neurology International. https://doi.org/10.4081/ni.2014.5359

5. Beckham, J. D., & Tyler, K. L. (2015). Arbovirus Infections. CONTINUUM Lifelong

Learning in Neurology. https://doi.org/10.1212/CON.0000000000000240