Introduction: Guillain-Barré syndrome is an immune-mediated polyneuropathy that is routinely initially treated with either intravenous immunoglobulin or plasmapheresis. To the best of our knowledge, no association between plasmapheresis treatment and acute onset of facial neuropathy has been reported. Case presentation: A 35-year-old Caucasian man with no significant prior medical history developed ascending motor weakness and laboratory findings consistent with a diagnosis of Guillain-Barré syndrome. Plasmapheresis was initiated. Acute facial palsy developed during the plasma exchange that subsequently resolved and then acutely recurred during the subsequent plasma exchange. Conclusion: To the best of our knowledge, no prior cases of acute facial palsy developing during plasmapheresis treatment are known. Although facial nerve involvement is common in typical Guillain-Barré syndrome, the temporal association with treatment, near-complete resolution and later recurrence support the association. The possible mechanism of plasmapheresis-induced worsening of peripheral nerve function in Guillain-Barré syndrome is unknown

Bogorad, Ilya V.

Stevenson, Mary L.

Weimer, Louis H.

English

PubMed

Mary L Stevenson

Louis H Weimer

Ilya V Bogorad

Springer - Publisher Connector

CASE REPORT Open Access
Development of recurrent facial palsy during
plasmapheresis in Guillain-Barré syndrome: a
case report
Mary L Stevenson1, Louis H Weimer2*, Ilya V Bogorad3
Abstract
Introduction: Guillain-Barré syndrome is an immune-mediated polyneuropathy that is routinely initially treated
with either intravenous immunoglobulin or plasmapheresis. To the best of our knowledge, no association between
plasmapheresis treatment and acute onset of facial neuropathy has been reported.
Case presentation: A 35-year-old Caucasian man with no significant prior medical history developed ascending
motor weakness and laboratory findings consistent with a diagnosis of Guillain-Barré syndrome. Plasmapheresis was
initiated. Acute facial palsy developed during the plasma exchange that subsequently resolved and then acutely
recurred during the subsequent plasma exchange.
Conclusion: To the best of our knowledge, no prior cases of acute facial palsy developing during plasmapheresis
treatment are known. Although facial nerve involvement is common in typical Guillain-Barré syndrome, the
temporal association with treatment, near-complete resolution and later recurrence support the association. The
possible mechanism of plasmapheresis-induced worsening of peripheral nerve function in Guillain-Barré syndrome
is unknown.
Introduction
Guillain-Barré syndrome (GBS) is an immune-mediated
acute polyneuropathy typically characterized by ascend-
ing weakness and areflexia. An association with Campy-
lobacter jejuni infection is most common; however,
numerous associations are known [1]. Now recognized
as a heterogeneous syndrome, different variants exist
including demyelinating and axonal forms; the demyeli-
nating variant is most common in the USA. Based on
considerable clinical trial evidence, the American Acad-
emy of Neurology currently recommends treatment with
either intravenous immunoglobulin (IVIG) or plasma-
pheresis within two to four weeks [2]. Although the dis-
ease may continue to advance during treatment, acute
focal worsening is not a recognized treatment complica-
tion. We report a case of acute facial palsy that devel-
oped during plasma exchange, subsequently resolved,
and then acutely recurred during the subsequent plasma
exchange.
Case presentation
A 35-year-old Caucasian man, with no significant prior
medical history, developed symmetric ascending weak-
ness and paresthesia. Six days prior to admission he
noted bilateral foot and then posterior leg numbness.
Chiropractic manipulation provided no relief. Two days
later, he developed progressively ascending lower extre-
mity weakness and increasing leg and foot tingling and
numbness. Hand weakness and paresthesia began two
days prior to admission and this spread to involve his
shoulder girdle. His gait became unsteady, prompting
him to come to our emergency department. Prior to
admission, he also noted shortness of breath with exer-
tion but not while at rest, feeling as though his heart
was racing during exertion, and night sweats, all of
which were unusual for him. He was an avid runner
prior to the onset of his symptoms. His wife is a nurse
practitioner and her home physical examination was
described to show areflexia. He had no notable
* Correspondence: lhw1@columbia.edu
2Neurological Institute of New York, Columbia University Medical Center, 710
W. 168th Street, Unit 55, New York, NY 10032, US
Full list of author information is available at the end of the article
Stevenson et al. Journal of Medical Case Reports 2010, 4:253
http://www.jmedicalcasereports.com/content/4/1/253 JOURNAL OF MEDICAL
CASE REPORTS
© 2010 Stevenson et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative
Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and
reproduction in any medium, provided the original work is properly cited.
respiratory or gastrointestinal viral prodrome prior to
the onset of his neurological symptoms. However, he
described a mild, transient occipital, throbbing headache
one morning at the start of his symptoms that resolved
within two hours of onset. One day prior to admission,
he noted mid-tongue numbness. Sensation in his extre-
mities seemed altered and he had difficulty distinguish-
ing hot from cold objects. His weakness and numbness
were progressively worsening on the day of his admis-
sion. On the morning of admission he also noted one
period of blurry vision, which spontaneously resolved
within two hours. He denied nausea, diarrhea, dysuria,
presyncope, vertigo, hearing loss, rash, diplopia, facial
asymmetry, tremor, dysphagia, or dysarthria. He denied
recent vaccinations.
On admission to our hospital, he had normal orienta-
tion and cognition. His extraocular movements were full
without nystagmus. His visual fields were full; no papil-
ledema was evident. His pupils reacted briskly without
afferent defect. His facial strength and sensation was full
and symmetric. Despite the subtle symptoms, his face
was symmetric upon smiling, eyebrow wrinkling, lip
pursing, and eye closure. Light touch and cold percep-
tion in his trigeminal nerve territories was normal. His
hearing was intact. His palate elevated well, although he
had an odd sensation in the back of his throat. His ton-
gue was midline on protrusion. No dysarthria was
noted. His limb strength demonstrated bilateral weak-
ness ranging from Medical Research Council scale 4- to
4+/5 in his upper and lower extremities. Deep tendon
reflexes were hypoactive in his arms and absent in both
of his legs; he had decreased light touch, temperature,
and pin-prick sensation bilaterally from his feet to his
thighs, and in his hands ascending to his shoulders. No
specific cerebellar abnormalities were evident. His gait
was unsteady and wide-based and he displayed an
inability to tandem walk.
Cerebral spinal fluid showed cytoalbuminologic disso-
ciation with a protein of 51 mg/dL and two white blood
cells per mm3. His serology was negative for IgG anti-
GQ1b and anti-GM1 ganglioside and related antibodies.
No human immunodeficiency virus antibodies were pre-
sent. He had positive titers of cytomegalovirus IgG and
IgM, and he had a borderline reactive cerebrospinal
fluid Lyme antibody study though negative serum anti-
bodies suggested a false positive result. Over the ensuing
days, weakness continued to progress slowly in his arms
and legs to a point at which he was no longer able to
walk or raise his arms without difficulty. His face, how-
ever, remained uninvolved. No cranial sensory or motor
deficits developed.
Plasmapheresis was initiated on day nine of his symp-
toms following insertion of a vascular catheter. Near the
end of the first treatment, he developed severe right-
sided facial weakness with dysgeusia, and an obvious
facial droop appeared. The remainder of his neurological
examination, including contralateral facial strength,
remained unchanged. A brain magnetic resonance ima-
ging (MRI) scan was performed two hours later and
showed no restricted diffusion. This deficit completely
resolved within thirty minutes and did not recur that
day. Two days later, a second round of plasmapheresis
was initiated. Calcium gluconate was given prior to the
procedure because of mildly low-ionized calcium mea-
sures. Approximately half-way into the second treat-
ment, his facial weakness reemerged, this time without
resolution, and he developed a persistent right-sided
facial droop, an asymmetric smile, and weak closure of
the right eye. The plasma exchange was discontinued
mid-treatment and he was closely observed. His fore-
head was asymmetric but notably less involved. His
frontalis strength improved and was symmetric the day
after this second round of plasmapheresis, though the
remainder of his facial paralysis persisted.
Because of the association of recurrent acute facial
weakness during plasmapheresis, the therapy was dis-
continued and a decision was made to substitute with a
course of IVIG. He received a conventional dose of
IVIG, which was a total dose of 2.0 mg/kg given as a 5-
day treatment course (0.4 mg/kg per day of 6 percent
IVIG). He tolerated the infusions without complication.
Despite treatment, the weakness in his extremities
continued to slowly progress and he later developed
left-sided facial weakness, first noted four days after his
second plasmapheresis treatment. Additionally, on day
12 of his symptoms his difficulty chewing prompted a
change to a soft mechanical diet; on day 14 of his
course he failed a swallowing evaluation indicating prob-
able pharyngeal weakness. His symptoms continued to
progress and seemed to nadir by week five of his course.
Facial motor nerve conductions were performed on
the day of the second plasmapheresis (day 11 of his
symptoms). Normal distal latencies and normal and
symmetric evoked amplitudes were found from common
facial nerve stimulation and recording from his bilateral
orbicularis oculi, nasalis, and orbicularis oris muscles. In
all likelihood, insufficient time had elapsed for Wallerian
degeneration to occur. Blink reflex studies demonstrated
an increased R1 latency (16.1 ms) and absent ipsilateral
and normal contralateral R2 responses following right-
sided supraorbital stimulation. Left-sided stimulation
demonstrated a mildly-increased R1 latency but normal
ipsilateral and absent contralateral R2 responses.
Nerve conduction studies of his right median, ulnar,
peroneal, and tibial nerves were performed on days
nine, 18 and 26 of his course and showed a demyelinat-
ing pattern with axonal involvement that progressively
worsened with each examination. Increased distal motor
Stevenson et al. Journal of Medical Case Reports 2010, 4:253
http://www.jmedicalcasereports.com/content/4/1/253
Page 2 of 4
latencies, serially-reduced evoked motor amplitude,
reduced sensory responses, and loss of F-waves ensued;
conduction velocity remained relatively unaffected. Focal
conduction block or significant temporal dispersion was
not evident at any point. Abundant fibrillations were
evident in multiple muscles in the studies performed on
day 26.
His facial droop improved by the third week of his
course, and continued to improve through week four.
His face became symmetric. His clinical course was
complicated by pneumonia, respiratory failure requiring
intubation, and a tracheotomy. He was discharged on
day 46 in a stable condition to an acute rehabilitation
facility. At that point he had mild facial weakness, was
able to symmetrically produce a small smile and could
fully but not forcefully close his eyes.
One year later he has almost fully recovered, following
extended rehabilitation and physical therapy, and he has
returned to work. He continues to report numbness in
his big toes, and partial numbness in his second and
third toes bilaterally, with sporadic neuropathic pain
occurring two to three times per week but not requiring
the use of pain medications. His facial symptoms ulti-
mately resolved.
Conclusions
GBS typically produces relatively symmetric ascending
weakness and depressed deep tendon reflexes or are-
flexia [3]. Plasmapheresis and IVIG are the mainstays of
acute GBS treatment [2]. Conventional plasmapheresis
is not recognized to induce acute worsening including
facial neuropathy. Only one previous similar report, of
two clinical cases, was identified. Chida et al. reported
in 1998 two cases of bilateral facial palsy developing in
Miller Fisher Syndrome, a GBS variant associated with
GQ1b antibodies, which occurred in the setting of
immunoadsorption plasmapheresis (IAP) therapy. In
these cases, bilateral facial palsy developed after either
three of three or three of five IAP treatments while
other neurological deficits were improving [4]. IAP is a
newer form of plasmapheresis that selectively removes
IgG without removal of significant albumen and other
blood components. It should be noted that the process
does not remove notable amounts of IgM antibodies.
This process has been shown to be efficacious in Miller
Fisher Syndrome [5].
Our patient twice developed acute onset right-sided
facial weakness during conventional plasmapheresis for
GBS, with resolution of his symptoms in the first inci-
dence and persistence of them in the latter. This close
temporal association of his facial weakness onset is sup-
portive of a direct relationship with the plasma exchange
treatment. The remainder of his symptoms continued to
gradually and slowly progress over days without acute
changes. Plasmapheresis is proven to decrease the dura-
tion and severity of deficits [6]. It is believed that ante-
cedent infection leads to the production of humoral and
cellular immune effectors that cross-react with certain
nerve or myelin epitopes [7]. More recent work invol-
ving immunoadsorption, which selectively removes spe-
cific antibodies, shows that IAP is also an efficacious
treatment which removes specific anti-myelin antibodies
associated with GBS [8,9]. Our case is highly suggestive
of a direct relationship between plasma exchange and
development of facial palsy. It is conceivable that pro-
tective antibodies were removed by this treatment lead-
ing to the acute facial neuropathy. Additionally, other
unknown large molecular weight proteins serving to
modulate the immune response may have been
removed. The etiology of the peripheral nerve dysfunc-
tion is unknown at this stage. The mildly low-ionized
calcium during the first exchange and calcium gluconate
infusion during the second treatment is not likely a sig-
nificant factor. Sudden improvement of neurological
function is reported in some cases associated with
plasma exchange; such improvement is thought to occur
faster than that explicable by neuroregenerative pro-
cesses such as remyelination. In these settings, antibody-
mediated changes in ion channel function that restores
neural transmission is proposed. Ultimately the affected
side of the face had the same outcome as the later more
conventionally-affected side. Plasmapheresis units should
be watchful for acute changes in strength during
exchange treatments that may be exacerbated by further
treatment.
Consent
Written informed consent was obtained from the patient
for publication of this case report. A copy of the written
consent is available for review by the Editor-in-Chief of
this journal.
Author details
1P & S Box 485, 630 West 168th Street, New York, NY 10032, US.
2Neurological Institute of New York, Columbia University Medical Center, 710
W. 168th Street, Unit 55, New York, NY 10032, US. 391 Highgate Street,
Needham, MA 02492, US.
Authors’ contributions
MLS, IVB, and LHW reviewed the current literature and patient presentation
to compile this case report. LHW analyzed the nerve conduction studies. All
authors read and approved the final manuscript.
Competing interests
The authors declare that they have no competing interests.
Received: 10 February 2010 Accepted: 6 August 2010
Published: 6 August 2010
References
1. Rees JH, Soudain SE, Gregson NA, Hughes RA: Campylobacter jejuni
infection and Guillain-Barré syndrome. N Engl J Med 1995, 333:1374-1379.
Stevenson et al. Journal of Medical Case Reports 2010, 4:253
http://www.jmedicalcasereports.com/content/4/1/253
Page 3 of 4
2. Hughes RA, Wijdicks EF, Barohn R, Benson E, Cornblath DR, Hahn AF,
Miller RG, Sladky JT, Stevens JC: Practice parameter: immunotherapy for
Guillain-Barré syndrome: report of the Quality Standards Subcommittee
of the American Academy of Neurology. Neurology 2003, 61:736-740.
3. Ropper AH: The Guillain-Barré syndrome. N Engl J Med 1992,
326:1130-1136.
4. Chida K, Takase S, Itoyama Y: Development of facial palsy during
immunoadsorption plasmapheresis in Miller Fisher Syndrome: a clinical
report of two cases. J Neurol Neurosurg Psychiatry 1998, 64:399-401.
5. Ohtsuka K, Nakamura Y, Tagawa Y, Yuki N: Immunoadsorption therapy for
Fisher syndrome associated with IgG anti-GQ1b antibody. Am J
Ophthalmol 1998, 125:403-406.
6. Raphaël JC, Chevret S, Hughes RA, Annane D: Plasma exchange for
Guillain-Barré syndrome. Cochrane Database Syst Rev 2001, 2:CD001798.
7. Hahn AF: Guillain-Barré syndrome. Lancet 1998, 352:635-641.
8. Haupt WF, Rosenow F, van der Ven C, Birkmann C: Immunoadsorption in
Guillain-Barré syndrome and myasthenia gravis. Ther Apher 2000,
4:195-197.
9. Willison HJ, Townson K, Veitch J, Boffey J, Isaacs N, Andersen SM, Zhang P,
Ling CC, Bundle DR: Synthetic disialylgalactose immunoadsorbents
deplete anti-GQ1b antibodies from autoimmune neuropathy sera. Brain
2004, 127:680-91.
doi:10.1186/1752-1947-4-253
Cite this article as: Stevenson et al.: Development of recurrent facial
palsy during plasmapheresis in Guillain-Barré syndrome: a case report.
Journal of Medical Case Reports 2010 4:253.
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Development of recurrent facial palsy during plasmapheresis in Guillain-Barré syndrome: a case report

Columbia University Academic Commons

CASE REPORT Open AccessDevelopment of recurrent facial palsy duringplasmapheresis in Guillain-Barré syndrome: acase reportMary L Stevenson1, Louis H Weimer2*, Ilya V Bogorad3AbstractIntroduction: Guillain-Barré syndrome is an immune-mediated polyneuropathy that is routinely initially treatedwith either intravenous immunoglobulin or plasmapheresis. To the best of our knowledge, no association betweenplasmapheresis treatment and acute onset of facial neuropathy has been reported.Case presentation: A 35-year-old Caucasian man with no significant prior medical history developed ascendingmotor weakness and laboratory findings consistent with a diagnosis of Guillain-Barré syndrome. Plasmapheresis wasinitiated. Acute facial palsy developed during the plasma exchange that subsequently resolved and then acutelyrecurred during the subsequent plasma exchange.Conclusion: To the best of our knowledge, no prior cases of acute facial palsy developing during plasmapheresistreatment are known. Although facial nerve involvement is common in typical Guillain-Barré syndrome, thetemporal association with treatment, near-complete resolution and later recurrence support the association. Thepossible mechanism of plasmapheresis-induced worsening of peripheral nerve function in Guillain-Barré syndromeis unknown.IntroductionGuillain-Barré syndrome (GBS) is an immune-mediatedacute polyneuropathy typically characterized by ascend-ing weakness and areflexia. An association with Campy-lobacter jejuni infection is most common; however,numerous associations are known [1]. Now recognizedas a heterogeneous syndrome, different variants existincluding demyelinating and axonal forms; the demyeli-nating variant is most common in the USA. Based onconsiderable clinical trial evidence, the American Acad-emy of Neurology currently recommends treatment witheither intravenous immunoglobulin (IVIG) or plasma-pheresis within two to four weeks [2]. Although the dis-ease may continue to advance during treatment, acutefocal worsening is not a recognized treatment complica-tion. We report a case of acute facial palsy that devel-oped during plasma exchange, subsequently resolved,and then acutely recurred during the subsequent plasmaexchange.Case presentationA 35-year-old Caucasian man, with no significant priormedical history, developed symmetric ascending weak-ness and paresthesia. Six days prior to admission henoted bilateral foot and then posterior leg numbness.Chiropractic manipulation provided no relief. Two dayslater, he developed progressively ascending lower extre-mity weakness and increasing leg and foot tingling andnumbness. Hand weakness and paresthesia began twodays prior to admission and this spread to involve hisshoulder girdle. His gait became unsteady, promptinghim to come to our emergency department. Prior toadmission, he also noted shortness of breath with exer-tion but not while at rest, feeling as though his heartwas racing during exertion, and night sweats, all ofwhich were unusual for him. He was an avid runnerprior to the onset of his symptoms. His wife is a nursepractitioner and her home physical examination wasdescribed to show areflexia. He had no notable* Correspondence: lhw1@columbia.edu2Neurological Institute of New York, Columbia University Medical Center, 710W. 168th Street, Unit 55, New York, NY 10032, USFull list of author information is available at the end of the articleStevenson et al. Journal of Medical Case Reports 2010, 4:253http://www.jmedicalcasereports.com/content/4/1/253 JOURNAL OF MEDICALCASE REPORTS© 2010 Stevenson et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the CreativeCommons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, andreproduction in any medium, provided the original work is properly cited.respiratory or gastrointestinal viral prodrome prior tothe onset of his neurological symptoms. However, hedescribed a mild, transient occipital, throbbing headacheone morning at the start of his symptoms that resolvedwithin two hours of onset. One day prior to admission,he noted mid-tongue numbness. Sensation in his extre-mities seemed altered and he had difficulty distinguish-ing hot from cold objects. His weakness and numbnesswere progressively worsening on the day of his admis-sion. On the morning of admission he also noted oneperiod of blurry vision, which spontaneously resolvedwithin two hours. He denied nausea, diarrhea, dysuria,presyncope, vertigo, hearing loss, rash, diplopia, facialasymmetry, tremor, dysphagia, or dysarthria. He deniedrecent vaccinations.On admission to our hospital, he had normal orienta-tion and cognition. His extraocular movements were fullwithout nystagmus. His visual fields were full; no papil-ledema was evident. His pupils reacted briskly withoutafferent defect. His facial strength and sensation was fulland symmetric. Despite the subtle symptoms, his facewas symmetric upon smiling, eyebrow wrinkling, lippursing, and eye closure. Light touch and cold percep-tion in his trigeminal nerve territories was normal. Hishearing was intact. His palate elevated well, although hehad an odd sensation in the back of his throat. His ton-gue was midline on protrusion. No dysarthria wasnoted. His limb strength demonstrated bilateral weak-ness ranging from Medical Research Council scale 4- to4+/5 in his upper and lower extremities. Deep tendonreflexes were hypoactive in his arms and absent in bothof his legs; he had decreased light touch, temperature,and pin-prick sensation bilaterally from his feet to histhighs, and in his hands ascending to his shoulders. Nospecific cerebellar abnormalities were evident. His gaitwas unsteady and wide-based and he displayed aninability to tandem walk.Cerebral spinal fluid showed cytoalbuminologic disso-ciation with a protein of 51 mg/dL and two white bloodcells per mm3. His serology was negative for IgG anti-GQ1b and anti-GM1 ganglioside and related antibodies.No human immunodeficiency virus antibodies were pre-sent. He had positive titers of cytomegalovirus IgG andIgM, and he had a borderline reactive cerebrospinalfluid Lyme antibody study though negative serum anti-bodies suggested a false positive result. Over the ensuingdays, weakness continued to progress slowly in his armsand legs to a point at which he was no longer able towalk or raise his arms without difficulty. His face, how-ever, remained uninvolved. No cranial sensory or motordeficits developed.Plasmapheresis was initiated on day nine of his symp-toms following insertion of a vascular catheter. Near theend of the first treatment, he developed severe right-sided facial weakness with dysgeusia, and an obviousfacial droop appeared. The remainder of his neurologicalexamination, including contralateral facial strength,remained unchanged. A brain magnetic resonance ima-ging (MRI) scan was performed two hours later andshowed no restricted diffusion. This deficit completelyresolved within thirty minutes and did not recur thatday. Two days later, a second round of plasmapheresiswas initiated. Calcium gluconate was given prior to theprocedure because of mildly low-ionized calcium mea-sures. Approximately half-way into the second treat-ment, his facial weakness reemerged, this time withoutresolution, and he developed a persistent right-sidedfacial droop, an asymmetric smile, and weak closure ofthe right eye. The plasma exchange was discontinuedmid-treatment and he was closely observed. His fore-head was asymmetric but notably less involved. Hisfrontalis strength improved and was symmetric the dayafter this second round of plasmapheresis, though theremainder of his facial paralysis persisted.Because of the association of recurrent acute facialweakness during plasmapheresis, the therapy was dis-continued and a decision was made to substitute with acourse of IVIG. He received a conventional dose ofIVIG, which was a total dose of 2.0 mg/kg given as a 5-day treatment course (0.4 mg/kg per day of 6 percentIVIG). He tolerated the infusions without complication.Despite treatment, the weakness in his extremitiescontinued to slowly progress and he later developedleft-sided facial weakness, first noted four days after hissecond plasmapheresis treatment. Additionally, on day12 of his symptoms his difficulty chewing prompted achange to a soft mechanical diet; on day 14 of hiscourse he failed a swallowing evaluation indicating prob-able pharyngeal weakness. His symptoms continued toprogress and seemed to nadir by week five of his course.Facial motor nerve conductions were performed onthe day of the second plasmapheresis (day 11 of hissymptoms). Normal distal latencies and normal andsymmetric evoked amplitudes were found from commonfacial nerve stimulation and recording from his bilateralorbicularis oculi, nasalis, and orbicularis oris muscles. Inall likelihood, insufficient time had elapsed for Walleriandegeneration to occur. Blink reflex studies demonstratedan increased R1 latency (16.1 ms) and absent ipsilateraland normal contralateral R2 responses following right-sided supraorbital stimulation. Left-sided stimulationdemonstrated a mildly-increased R1 latency but normalipsilateral and absent contralateral R2 responses.Nerve conduction studies of his right median, ulnar,peroneal, and tibial nerves were performed on daysnine, 18 and 26 of his course and showed a demyelinat-ing pattern with axonal involvement that progressivelyworsened with each examination. Increased distal motorStevenson et al. Journal of Medical Case Reports 2010, 4:253http://www.jmedicalcasereports.com/content/4/1/253Page 2 of 4latencies, serially-reduced evoked motor amplitude,reduced sensory responses, and loss of F-waves ensued;conduction velocity remained relatively unaffected. Focalconduction block or significant temporal dispersion wasnot evident at any point. Abundant fibrillations wereevident in multiple muscles in the studies performed onday 26.His facial droop improved by the third week of hiscourse, and continued to improve through week four.His face became symmetric. His clinical course wascomplicated by pneumonia, respiratory failure requiringintubation, and a tracheotomy. He was discharged onday 46 in a stable condition to an acute rehabilitationfacility. At that point he had mild facial weakness, wasable to symmetrically produce a small smile and couldfully but not forcefully close his eyes.One year later he has almost fully recovered, followingextended rehabilitation and physical therapy, and he hasreturned to work. He continues to report numbness inhis big toes, and partial numbness in his second andthird toes bilaterally, with sporadic neuropathic painoccurring two to three times per week but not requiringthe use of pain medications. His facial symptoms ulti-mately resolved.ConclusionsGBS typically produces relatively symmetric ascendingweakness and depressed deep tendon reflexes or are-flexia [3]. Plasmapheresis and IVIG are the mainstays ofacute GBS treatment [2]. Conventional plasmapheresisis not recognized to induce acute worsening includingfacial neuropathy. Only one previous similar report, oftwo clinical cases, was identified. Chida et al. reportedin 1998 two cases of bilateral facial palsy developing inMiller Fisher Syndrome, a GBS variant associated withGQ1b antibodies, which occurred in the setting ofimmunoadsorption plasmapheresis (IAP) therapy. Inthese cases, bilateral facial palsy developed after eitherthree of three or three of five IAP treatments whileother neurological deficits were improving [4]. IAP is anewer form of plasmapheresis that selectively removesIgG without removal of significant albumen and otherblood components. It should be noted that the processdoes not remove notable amounts of IgM antibodies.This process has been shown to be efficacious in MillerFisher Syndrome [5].Our patient twice developed acute onset right-sidedfacial weakness during conventional plasmapheresis forGBS, with resolution of his symptoms in the first inci-dence and persistence of them in the latter. This closetemporal association of his facial weakness onset is sup-portive of a direct relationship with the plasma exchangetreatment. The remainder of his symptoms continued togradually and slowly progress over days without acutechanges. Plasmapheresis is proven to decrease the dura-tion and severity of deficits [6]. It is believed that ante-cedent infection leads to the production of humoral andcellular immune effectors that cross-react with certainnerve or myelin epitopes [7]. More recent work invol-ving immunoadsorption, which selectively removes spe-cific antibodies, shows that IAP is also an efficacioustreatment which removes specific anti-myelin antibodiesassociated with GBS [8,9]. Our case is highly suggestiveof a direct relationship between plasma exchange anddevelopment of facial palsy. It is conceivable that pro-tective antibodies were removed by this treatment lead-ing to the acute facial neuropathy. Additionally, otherunknown large molecular weight proteins serving tomodulate the immune response may have beenremoved. The etiology of the peripheral nerve dysfunc-tion is unknown at this stage. The mildly low-ionizedcalcium during the first exchange and calcium gluconateinfusion during the second treatment is not likely a sig-nificant factor. Sudden improvement of neurologicalfunction is reported in some cases associated withplasma exchange; such improvement is thought to occurfaster than that explicable by neuroregenerative pro-cesses such as remyelination. In these settings, antibody-mediated changes in ion channel function that restoresneural transmission is proposed. Ultimately the affectedside of the face had the same outcome as the later moreconventionally-affected side. Plasmapheresis units shouldbe watchful for acute changes in strength duringexchange treatments that may be exacerbated by furthertreatment.ConsentWritten informed consent was obtained from the patientfor publication of this case report. A copy of the writtenconsent is available for review by the Editor-in-Chief ofthis journal.Author details1P & S Box 485, 630 West 168th Street, New York, NY 10032, US.2Neurological Institute of New York, Columbia University Medical Center, 710W. 168th Street, Unit 55, New York, NY 10032, US. 391 Highgate Street,Needham, MA 02492, US.Authors’ contributionsMLS, IVB, and LHW reviewed the current literature and patient presentationto compile this case report. LHW analyzed the nerve conduction studies. Allauthors read and approved the final manuscript.Competing interestsThe authors declare that they have no competing interests.Received: 10 February 2010 Accepted: 6 August 2010Published: 6 August 2010References1. Rees JH, Soudain SE, Gregson NA, Hughes RA: Campylobacter jejuniinfection and Guillain-Barré syndrome. N Engl J Med 1995, 333:1374-1379.Stevenson et al. Journal of Medical Case Reports 2010, 4:253http://www.jmedicalcasereports.com/content/4/1/253Page 3 of 42. 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Haupt WF, Rosenow F, van der Ven C, Birkmann C: Immunoadsorption inGuillain-Barré syndrome and myasthenia gravis. Ther Apher 2000,4:195-197.9. Willison HJ, Townson K, Veitch J, Boffey J, Isaacs N, Andersen SM, Zhang P,Ling CC, Bundle DR: Synthetic disialylgalactose immunoadsorbentsdeplete anti-GQ1b antibodies from autoimmune neuropathy sera. Brain2004, 127:680-91.doi:10.1186/1752-1947-4-253Cite this article as: Stevenson et al.: Development of recurrent facialpalsy during plasmapheresis in Guillain-Barré syndrome: a case report.Journal of Medical Case Reports 2010 4:253.Submit your next manuscript to BioMed Centraland take full advantage of: • Convenient online submission• Thorough peer review• No space constraints or color figure charges• Immediate publication on acceptance• Inclusion in PubMed, CAS, Scopus and Google Scholar• Research which is freely available for redistributionSubmit your manuscript at www.biomedcentral.com/submitStevenson et al. Journal of Medical Case Reports 2010, 4:253http://www.jmedicalcasereports.com/content/4/1/253Page 4 of 4

Annane D: Plasma exchange for Guillain-Barré syndrome. Cochrane Database Syst Rev

Birkmann C: Immunoadsorption in Guillain-Barré syndrome and myasthenia gravis. Ther Apher

Bundle DR: Synthetic disialylgalactose immunoadsorbents deplete anti-GQ1b antibodies from autoimmune neuropathy sera. Brain

Campylobacter jejuni infection and Guillain-Barré syndrome.

Guillain-Barré syndrome. Lancet

Immunoadsorption therapy for Fisher syndrome associated with IgG anti-GQ1b antibody.

Itoyama Y: Development of facial palsy during immunoadsorption plasmapheresis in Miller Fisher Syndrome: a clinical report of two cases.

The Guillain-Barré syndrome.

http://www.pubmedcentral.nih.gov/articlerender.fcgi?artid=2923178

Development of recurrent facial palsy during plasmapheresis in Guillain-Barré syndrome: a case report

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