Guillain - Kerala Medical Journal

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Guillain-Barré Syndrome with
Bilateral Facial Nerve Paralysis
Author- Dr. Ranzeeb Rasheed, PG in Gen. Medicine, MGMC&RI, Puducherry
Co-author- Dr. Kalirattiname, Associate Prof., MGMC&RI, Puducherry
Abstract
Guillain-Barré syndrome is an acute demyelinating disorder of the
peripheral nervous system that results from an aberrant immune response
directed at peripheral nerves. It may also involve cranial nerves. The exact
mechanisms which cause the conditions aren't clear, but about 60 per cent
of those affected will have had a throat or intestinal infection, flu or major
stress within the previous two weeks. Bacteria such as Campylobacter and
mycoplasma pneumonia, and viruses such as Epstein Barr virus and
cytomegalovirus are some of the organisms that have been reported to be
involved.
Introduction
Bilateral facial nerve palsy is an extremely rare presentation. Common
causes for bilateral facial nerve palsy are Guillain-Barré syndrome,
leukaemia, Lyme’s disease, sarcoidosis, leprosy, and trauma.
Case Presentation
A 14 year old male student presented with 1 month history of lower limb
weakness with bilateral facial weakness. He was treated for the same in a
different hospital. One week prior to the onset of weakness, he had fever
with sore throat.
One month back the patient had experienced sudden onset of weakness of
lower limb which was progressive in nature with inability to walk along
with difficulty in swallowing and bilateral facial weakness over a period of
1-2 weeks. This was followed by a mild recovery in the weakness of the
lower limb and complete resolution of dysphagia with persistence of facial
weakness.
The whole episode was managed conservatively.
On examination, there was bilateral lower motor neuron type of facial palsy
along with lower limb weakness (2/5 in all muscle groups) while power in
the upper limb was normal. All deep tendon reflexes demonstrated
areflexia. Plantars were flexor. No bladder or bowel involvement with
normal fundoscopy.
Blood investigations were normal. CSF demonstrated elevated protein
content with normal cell counts suggestive of albumino-cytological
dissociation. CSF bacterial antigen detection for H. Influenza type B,
Streptococcus Pneumoniae, Meningitidis Group A, Meningitidis Group B,
and Streptococcus Group B were absent.
MRI brain showed no significant abnormalities.
EMG / NC finding were suggestive of generalised, symmetric,
motor>sensory (axonal > demyelinating) polyradiculonueropathy affecting
lower limbs more than upper limbs
Patient was started on steroids; improvement in lower limb power along
with mild recovery of facial weakness was noted over a period of one week
from admission.
Discussion
Bilateral Facial Nerve Palsy may be the presenting feature of metabolic,
infectious, vasculitic, immunological, neoplastic causes.
Patient with history of tick bite with presence of erythema chronicum
migrans with recent travel abroad should be suspected to have Lyme’s
disease, other infectious disease like HSV and IMN should be ruled out by
doing serology screens , multiple sclerosis should be screened by doing
MRI brain , CSF for oligoclonal bands and visual evoked potential.
Sarcoidosis , SLE , Vasculitic disorders should be ruled out by doing a ANA ,
ACE levels , pANCA, cANCA , other causes like leukemia, lymphoma can be
diagnosed by doing a peripheral smear and bone marrow biopsy.
Guillain-Barré syndrome is an Acute Inflammatory Demyelinating
Polyradiculonueropathy that presents with facial nerve palsy in 27-50% of
cases. Other cranial nerves may also be involved. Diagnosis is confirmed
clinically by the presence of ascending flaccid paralysis, areflexia without
any definite sensory level with CSF showing albumin- cytological
dissociation and nerve conduction studies showing demyelinating pattern.
Management includes IVIG infusion or Plasmapharesis along with
ventilator support in case of respiratory failure due to involvement of
diaphragm.
Conclusion
Guillain-Barré syndrome should be included in the differential diagnosis of
bilateral facial nerve palsy.
Reference
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CAMPYLOBACTER JEJUNI INFECTION AND GUILLAIN–BARRÉ
SYNDROME , THE NEW ENGLAND JOURNAL OF MEDICINE Nov. 23,
1995
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