International Journal of Physical Medicine & Rehabilitation
Open Access
ISSN: 2329-9096
ISSN: 2329-9096
Daniel T Kuo
Tufts Medical Center, USA
Scientific Tracks Abstracts: Int J Phys Med Rehabil
Introduction: Guillain-Barré syndrome (GBS) is an acute, inflammatory autoimmune polyradiculoneuropathy
characterized by abrupt onset progressive limb weakness and diminished reflexes. Heterogeneous subtypes of GBS
are further differentiated by clinical, pathological and electrophysiological criteria. The predominant trigger is
antecedent infection that induces aberrant immune-mediated responses targeting peripheral nerves and spinal
roots. Campylobacter jejuni (C. jejuni) is the pathogen most frequently associated with Acute Motor Axonal
Neuropathy (AMAN), a sub-group of GBS characterized by axonal degeneration of motor nerves. There is a strong
association between AMAN and GM1, GD1a, GD1b and Ga1NAc-GD1a antibodies. Here we describe a patient
who developed AMAN with elevated anti-GM1 and anti-GD1a antibody titers but negative C. jejuni serology.
Case Description: A 59-year-old male presented with rapidly progressing bilateral upper and lower extremity
weakness. He was previously admitted 12 days prior for suspected viral gastroenteritis and treated with intravenous
fluids and discharged home at baseline. Two days prior to this admission, he developed right hand and foot
weakness, which quickly progressed to the contra lateral side. Initial exam revealed rapidly progressing flaccid
quadriparesis without sensory loss. Bilateral biceps, triceps, brachioradialis, patellar and Achilles muscle tendon
reflexes were absent. Cranial nerves were intact. Nerve Conduction Study (NCS) performed three days following
symptom onset revealed reduced compound muscle action potential amplitudes/velocities in right-sided median,
ulnar, peroneal and tibial nerves with preserved sensory responses. Electro physiologic findings were suggestive of
axonal degeneration with evidence of demyelinating features. Six weeks later, repeat NCS demonstrated worsening
motor response and evidence supporting a generalized axonal motor polyneuropathy consistent with AMAN.
Cerebrospinal fluid analysis did not show albuminocytologic dissociation. Serology tested negative for Salmonella,
Shiga toxin 1/2, and Campylobacter. Anti-GM1 and anti-GD1a antibody titers were high. Patient received two
5-day courses of intravenous immunoglobulin (IVIG) following symptom onset which halted, but did not reverse,
progression of weakness. After 3 months of inpatient rehabilitation, he was discharged home at supervision level
for ambulation.
Discussion: The role of anti-ganglioside antibodies in the immuno pathogenesis of GBS is not clearly understood. It
has been proposed that antibody formation is triggered by molecular mimicry between C. jejuni lipopolysaccharides
and neural tissue, which induces a cross-reactive immune response producing axonal degeneration. This explains
the association between anti-GM1 antibody and antecedent C. jejuni infection; however, there have been several
studies which have found only a 50% concordance between serologically positive C. jejuni and anti-GM1 antibody.
The low sensitivity of anti-C. jejuni assay and the presence of other microorganisms that triggers anti-ganglioside
responses and AMAN could explain this discordance.
Conclusion: Despite negative C. jejuni serology, patient presented with high anti-ganglioside antibody titers and
AMAN. Electrodiagnostic studies showed evidence of axonal degeneration, which is thought to be an indicator
of poor prognosis. However, after intensive inpatient rehabilitation, patient demonstrated significant functional
recovery at 3-months following symptom onset.
Daniel T Kuo has completed his Doctor of Osteopathic Medicine degree from Touro University, Nevada College of Medicine in Las Vegas, NV in 2016. He has completed his Internship at Nassau University Medical Center in East Meadow, NY. He is currently a Physical Medicine and Rehabilitation Resident at Tufts Medical Center in Boston.
E-mail: dkuo@tuftsmedicalcenter.org