Case Report: Rapid Progressive Quadriparesis in Guillain-Barre Syndrome

Lina Kudinavičiūtė1, Karolina Meyer2, Kamilė Žilinskienė3

1Tarandes family clinic, Vilnius, Lithuania

2Lithuanian University of Health Sciences, Academy of Medicine, Faculty of Medicine, Kaunas, Lithuania

3Lithuanian University of Health Sciences, Academy of Medicine, Faculty of Medicine, Kaunas, Lithuania

Abstract:

Guillain-Barre syndrome (GBS) is an acute immune-mediated polyneuropathy with an incidence of 0.6-2.4 per 100000 annually. GBS affects mostly lower extremities but can ascend to upper extremities and cause weakness of intercostal muscles, disturbing the ability to breathe and swallow. The tendon reflexes will be affected and the cranial nerves can be damaged. GBS extremely affects the autonomous nervous system and causes arrhythmia, blood pressure changes, tachycardia. Treatment varies for every individual, although the acute phase requires careful management of vital functions and instant treatment with plasmapheresis and high-doses of intravenous immunoglobulin. We present a case of a 40-year-old male patient, who was diagnosed with Guillain-Barre syndrome. He suffered from the following symptoms: progressive muscle weakness, dysarthria, upper limbs numbness, and tingling.

Keywords: Guillain-barre syndrome, polyneuropathy, paralysis, rehabilitation.

Journal of Medical Sciences. April 30, 2020 - Volume 8 | Issue 15. Electronic-ISSN: 2345-059
Medical Sciences 2020 Vol. 8 (15), p. 85-90
Case Report: Rapid Progressive Quadriparesis in Guillain-Barre
Syndrome
Lina Kudinavičiūtė
1
, Karolina Meyer
2
, Kamilė Žilinskienė
3
1
Tarandes family clinic, Vilnius, Lithuania
2
Lithuanian University of Health Sciences, Academy of Medicine, Faculty of Medicine, Kaunas, Lithuania
3
Lithuanian University of Health Sciences, Academy of Medicine, Faculty of Medicine, Kaunas, Lithuania
Abstract:
Guillain-Barre syndrome (GBS) is an acute immune-mediated polyneuropathy with an incidence of 0.6-2.4 per
100000 annually. GBS affects mostly lower extremities but can ascend to upper extremities and cause weakness of
intercostal muscles, disturbing the ability to breathe and swallow. The tendon reflexes will be affected and the cranial
nerves can be damaged. GBS extremely affects the autonomous nervous system and causes arrhythmia, blood
pressure changes, tachycardia. Treatment varies for every individual, although the acute phase requires careful
management of vital functions and instant treatment with plasmapheresis and high-doses of intravenous
immunoglobulin. We present a case of a 40-year-old male patient, who was diagnosed with Guillain-Barre syndrome.
He suffered from the following symptoms: progressive muscle weakness, dysarthria, upper limbs numbness, and
tingling.
Keywords: Guillain-Barre syndrome, polyneuropathy, paralysis, rehabilitation.
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Journal of Medical Sciences. April 30, 2020 - Volume 8 | Issue 15. Electronic-ISSN: 2345-059
Introduction
Guillain-Barre syndrome (GBS) is an acute immune-
mediated polyneuropathy, quadriparesis and is a
common cause of respiratory paralysis
[1]
. The incidence
of GBS is reported to be 0.6-2.4 per 100000 annually
[2,3,4]
. It usually is proceeded by upper respiratory tract
infections or gastrointestinal tract infections.
[5]
Clinical
features are very progressive and include fairly
symmetric muscle weakness, absent or depressed deep
tendon reflexes. The muscle weakness might differ from
mild difficulty with walking to nearly complete
paralysis of all extremities, facial, respiratory and bulbar
muscles
[6]
. Studies from the United States and Europe
show that the weakness usually starts in the legs and
only 10% of cases start in the arms or facial muscles.
Severe respiratory muscle weakness with the need for
ventilation develops in 10-30% of patients
[7]
. More than
50% of patients develop facial nerve palsies and
oropharyngeal weakness
[8,9]
. The pain is due to nerve
root inflammation, typically located in the back and
extremities can be a presenting feature and is reported
during the acute phase by two-thirds of patients with all
forms of GBS
[10,11]
. Typical laboratory finding having
increased levels of protein in cerebrospinal fluid with a
normal white blood cell count that might be normal in
the early stages. The acute phase requires careful
management of the disease and is combined with both
supportive therapy and immunotherapy. Every treatment
plan varies for each individual
[12]
. Treatment plans
include plasma exchange, intravenous gamma globulin
therapy, and immunosuppressive therapy based on
immunopathology
[13]
. There is a possibility to recover to
an extent such that independent walking after GBS
treatment. Nevertheless, some patients experience
negative outcomes regarding daily activities and
impaired muscular function even at 3-6 years after the
onset of GBS
[14]
. So, it is very important for GBS
patients to undergo rehabilitation treatment to increase
the likelihood of regaining independence in the daily
lifestyle and to improve muscular strength
[5]
.
Case report:
A healthy 40-year-old male patient, with no history of
chronic diseases, was rushed to the ER with complaints
of numbness in both hands; muscle weakness in all
extremities and disturbed speech.
On the 18
th
of October, the patient developed a dry
cough followed by vomiting. His both children were
sick with upper respiratory tract infection at that
moment. On the 24
th
of October 2018, the
patient’s sleep was interrupted by a sudden pain in his
neck and nausea. He thought the pain was caused by an
improper sleeping position, the patient ended up
throwing up several times. The following morning, the
pain was tolerable, however coughing and vomiting
continued. Around 10 A.M. the patient came back home
from work due to unexpected but progressive fatigue
and vomiting. By midday, getting up from the bed he
perceived muscle weakness in both hands and feet. The
patient called an ambulance and was taken to the nearest
hospital where, during an examination, his speech was
impaired and his hand numbness was getting worse.
Head CT showed light a subcortical hypodense zone
near the caput of the caudate nucleus, but a chance of
ischemia was not excluded. Lung Rö showed a small
amount of liquid in the right pleural sinus. Due to
unclear diagnosis, the patient was transferred to a 3
rd
level hospital.
Findings during the examination :
vesicular breathing and fine crackles during lung
auscultation,
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Journal of Medical Sciences. April 30, 2020 - Volume 8 | Issue 15. Electronic-ISSN: 2345-059
breathing rate – 26 times/min.,
febrile fever (37.6 ),
blood pressure 162/76mm/Hg.,
pulse – 76 bpm.
Neurologically the patient was conscious,
oriented, coughed and his speech was dysarthric.
Inadequate eye convergence was found. Due to throat
secretion, the patient was gagging.
o Hands: only flexible and extensive movements,
muscle strength 4 points. Slight flexion via elbow
joints. No proximal movements. Areflexia.
o Feet: no proximal movements, flexion, and
dorsiflexion 4 points. No patellar reflexes. Achill
tendon reflexes were positive both sides. No
pathological reflexes. The sensation was intact. Stiff
neck. Positive Brudzinski symphysial sign.
The patients’ symptoms were progressing quickly, and
since diagnosis was unclear, he was hospitalized to the
intensive care unit due to quadriparesis and respiratory
failure.
Table 1. shows progress of the symptoms, diagnostic
measures that were used during the time of
hospitalization as well as the treatment.
Table 1. Progress, symptoms, diagnostic measures, treatment
Date Symptoms, diagnostic measures and treatment
October 24
th
,
2018
Urgent CT and CT angiography were performed. No vertebral displacement was shown, hernias
were excluded. Neck blood vessels filled with contrast evenly, no signs of aortic aneurysms or
dissections.
Diagnosis differentiated between acute meningomyelitis and acute demyelinating
polyneuropathy.
The spinal fluid sample was taken – no major changes were found.
At 5 P.M. the patient was intubated due to paralysis of the diaphragm.
CFS – no significant changes (protein - 0,24g/l; WBC – 3l; glucose – 4,99 mmol/l) minimal
changes may be found in the first week.
After the intubation, the patient remained conscious, reactive to commands. Plegia of the hands
only the left-hand fingers were moving. Feet strength 0 points proximal and 4-5 points
distal, absence of reflexes.
Electromyoneurography (EMNG) the changes that were found are most common for axonal-
demyelinating motoric polyneuropathy with conduction blockages in proximal nerve parts.
The diagnosis was confirmed: Guillan-Barre syndrome.
Treatment with i/v IgG was administrated.
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Journal of Medical Sciences. April 30, 2020 - Volume 8 | Issue 15. Electronic-ISSN: 2345-059
October 25
h
,
2018
The patient’s condition remained severe, he was still conscious but responsive to commands.
Hand plegia presented reflexes, feet strength showed regress – 3-4 points distally.
October 27
th
,
2018
Rö – negative dynamics, fluid was detected in left pleural sinus.
October 29
th
,
2018
Treatment with i/v IgG course was finished.
After i/v IgG the patient showed no improvement. Facial nerve damage on both sides occurred
and paralytic lagophtalmus of both eyes.
Rö – negative dynamics. More free fluid in left pleural sinus were detected than before.
Tracheostomy was formed.
October 30
th
,
2018
The patient started breathing spontaneously.
November
5
th
, 2018
Rö – positive dynamics. The amount of free fluid reduced significantly.
November
6
th
, 2018
Central vein punction.
November
7
th
, 2018
The neurologist consulted the patient before another spinal puncture. The patient was
conscious, responsive to commands, unable to talk due to tracheostomy. Conjunctivitis in both
eyes, lagophtalmos was detected. Eye movements were limited (unable to move right eye
laterally), diploplia was found. The patient was unable to close both eyes at the same time.
Facial nerve paralysis was seen on both sides. Sensations in the face were intact.
November
8
th
, 2018
Infectologist consult doubtful IgM for tick bite encephalitis, however, there was a normal
number of cells in the spinal fluid, so it is thought to be cross-reaction. The blood test for
syphilis was negative.
December
3
rd,
2018
The patient’s condition improved, although insignificantly. During the examination - minimal
moves in shoulders and hands proximally, however, there were no movements in the legs.
The patient was transferred to neurology clinic from the ICU for further treatment.
December
4
th
, 2018
Early rehabilitation was started (increasing muscle strength, thromboembolic prophylaxis, a
course of massages for blood flow, logotherapy).
December
12
th
, 2018
Sinus tachycardia approached (pulse – 112bpm on the monitor), so the patient was consulted by
a cardiologist. The patient has no history of previous cardiac illnesses. The cardiologist
prescribed antihypertensive drugs.
December
21
st
, 2018
PM&R consult: biosocial functions remained disturbed. The patient is unable to move or serve
himself. 1
st
stage of rehabilitation was completed. Further rehabilitation is highly recommended.
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Journal of Medical Sciences. April 30, 2020 - Volume 8 | Issue 15. Electronic-ISSN: 2345-059
The patients’ condition had positive dynamics and, on
December 28th, 2018, so he was transferred to the
PM&R clinic. During rehabilitation, a team of PM&R
specialists performed a full set of individually formed
rehabilitation components. Daily functions and
independence of the patient improved significantly: he
was able to feed himself, stand up, sit down on his own
and lay down by himself or with minor help. He was able
to walk short distances with one crutch. Put on upper
clothes by himself, pants with minor help. Muscle
strength improved: upper limbs both proximal and distal
– 4-5 pts; lower limbs proximal – 1-2 pts, distal – 0-2pts.
Pain syndrome was highly expressed due to femoral
nerve damage, it was unresponsive, to peroral
medication. The Pain Clinic Department of Lithuanian
University of Health Sciences performed a femoral nerve
blockade to relieve the pain. Two days after the
procedure, the pain came back but was tolerable. Barthel
index an adapted environment 55 pts. The patient was
discharged on the 1
st
of March, 2019 with the
recommendation for further rehabilitation process in
peripheral facilities. Intensive and continuous
rehabilitation leads to multi-system improvement,
however, the patient is still not fully recovered and is left
with a diagnosis of hypertension and treatment with
antihypertensive medication.
Discussion
We present a 40-year-old male who was diagnosed with
Guillain-Barre syndrome after a complex of symptoms,
which includes progressive muscle weakness, dysarthria,
upper limb numbness, and tingling. GBS is an
acute/subacute onset polyneuropathy typically
manifesting with sensory symptoms and weakness over
several days and leading to quadriparesis
[1]
and affects
0.6-2.4 per 100000 people annually
[2,3,4]
. Our patient’s
case manifested rapidly with progressive muscle
weakness, dysarthria which reached a level of complete
quadriparesis, diaphragm paralysis, and facial nerve
paralysis. Lung ventilation was needed to maintain
breathing as well. Electroneuromyography showed
changes most common for axonal-demyelinating motoric
polyneuropathy which, combined with rapid progression,
suggested the diagnosis of Guillain-Barre syndrome. The
patient was treated in the ICU with intravenous
immunoglobulin G and supportive care was ensured.
After the treatment, our patient underwent a long and
intensive personal rehabilitation program which lead to
recovery.
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