Diagnosis and management of Guillan Barre Syndrome in 10 steps

Comprehensive and concise approach on GBS with 10 systemically steps.

1. Guillan Barre
Syndrome
Diagnosis and management of Guillain–Barré
syndrome in 10 steps.
24
08
23
Presented By:
Farah Adibah Kasmin
Master in Internal Medicine (MMed)
UITM Malaysia
Neurology
HCTM

2. Agenda
INTRODUCTION
EPIDEMIOLOGY
T
O
D
A
Y S
WHAT ARE THE 10 STEPS?

3. Introduction
A rare, potentially fatal immune mediated disease of the
peripheral nerves and nerve root.
The most common cause of acute flaccid paralysis.
A monophasic illness characterized by symmetrical weakness of the
limbs, and hyporeflexia or areflexia, which reaches a maximum severity
within 4 weeks.
Typically present with weakness and sensory signs in the legs that
progress to the arms and cranial muscles.
However, the disease is heterogeneous and several distinct clinical
variants exist.

4. 70% of cases of GBS were caused by antecedent
infections, most frequently respiratory or
gastrointestinal infections.
Overall incidence of GBS was between
1.1/100,000/year and 1.8/100,000/year.
The incidence of GBS increased with age after 50
years from 1.7/100,000/year to 3.3/100,000/year.
Epidemiology
GBS occurs more frequently in males than in
females.

5. S
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A
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10
Steps
•How to monitor the disease progression?
•How to manage early complications?
•How to manage the clinical progression?
•What is the predicting outcome?
•What is the rehabilitation planning?
When to suspect GBS?
How to diagnose GBS?
When to admit to ICU?
When to start treatment?
What are the treatment options?

6. Rapidly progressive bilateral weakness of the legs and/or
arms.
For classic sensorimotor form of GBS, distal
paraesthesias or sensory loss, accompanied or followed
by weakness that starts in the legs and progresses to the
arms and cranial muscles.
Reflexes are decreased or absent.
Dysautonomia is common: blood pressure or heart rate
instability, pupillary dysfunction, and bowel or bladder
dysfunction.
Disease onset is acute or subacute, typically reach
maximum disability within 2 weeks.
When to suspect?1.
Atypical clinical featuresTypical clinical features
Weakness and sensory signs, though always
bilateral, can be asymmetrical or
predominantly proximal or distal, and can
start in the legs, the arms or simultaneously in
all limbs.
Severe and diffuse pain or isolated cranial
nerve dysfunction can precede the onset of
weakness.

7. Variant of GBS
weakness without sensory signs (pure
motor variant).
Some patients have a distinct and persistent clinical variant of GBS that does not progress to the classic pattern of
sensory loss and weakness.
weakness limited to the cranial nerves
(bilateral facial palsy with paraesthesias).
weakness on upper limbs
(pharyngeal–cervical–brachial
weakness) or lower limbs
(paraparetic variant).

8. Preceding
Two- thirds of patients who develop GBS report
symptoms of an infection in the 6 weeks preceding
the onset of the condition.
Events
Usage of parenteral gangliosides for treating
peripheral neuropathy.
Non-infective associations to GBS

However, the absence of an antecedent illness does
not exclude a diagnosis of GBS.
Vaccines: H1N1 Influenza - have the potential
to induce molecular mimicry.
Immunosuppressive drugs: anti TNF1.

9. Clinical
Course
GBS

10. Two most commonly used sets of diagnostic criteria for GBS
are :
1. National Institute of Neurological Disorders and Stroke
(NINDS) in 1978 (revised in 1990) and further improvised in 2019.
2. The Brighton Collaboration in 2011.
2. How to diagnose?
Diagnosis of GBS is clinical, supported by relevant investigations such as CSF analysis, electrodiagnostic
studies (nerve conduction study), radiological findings (ultrasound/MRI) or anti-gangliosides testing.

11. Modified National Institute of Neurological Disorders and Stroke (NINDS) criteria for
GBS.

12. Modified National Institute of Neurological Disorders and Stroke (NINDS) criteria for
GBS.

13. The Brighton Collaboration GBS Working Group

14. Differential Diagnosis

15. Investigations of GBS
Laboratory studies are helpful in supporting and speeding up the diagnosis,
especially in patients with atypical presentation.
General Lab Ix Anti-gangliosides antibodies
FBC
Anti- GQ1b antibodies are found in up to
90% of patients with MFS.
Renal profile and Electrolytes
A positive test result can be helpful in doubtful
diagnosis.
Liver Funtion Test But a negative test result does not exclude GBS
Zika
C. Jejuni
Identify associated infection
However, do not wait for the results before
starting treatment.

16. CSF examination Electrodiagnostic studies - NCS & EMG
Elevated CSF
protein level and a normal CSF cell count (known as
albumino- cytological dissociation).
Useful supporting the diagnosis of GBS and to classify into
electrophysiology subtypes
of GBS: AIDP, AMAN, and AMSAN.
But, protein
levels are normal in 30–50% of patients during 1st week and 10–
30% in the 2nd week.
Typical for GBS is a ‘sural sparing pattern’ in which the
sural sensory nerve action potential is normal while
the median and ulnar sensory nerve action potentials are
abnormal or even absent
Mild pleocytosis (10–50 cells/μl), compatible with GBS.
For demyelinating form of GBS (AIDP): prolonged latencies, reduced
conduction velocities, abnormal temporal dispersion and/or partial
motor conduction blocks.
Axonal form of GBS (AMAN and AMSAN) shows reduced sensory and
motor evoked amplitudes.
Investigations of GBS

17. Investigations of GBS
Imaging in GBS.
MRI Peripheral nerve Ultrasound
Not part of the routine diagnostic evaluation
of GBS
Enlarged cervical nerve roots early in the
disease course,
indicatin of spinal root inflammation
as an early pathological mechanism.
Mainly to exclude differential diagnosis such
as central nervous system infection,
inflammation or infiltrative lesions.
Cheap, easily performed.

18. Evolving respiratory distress
with imminent respiratory insufficiency
Severe
autonomic cardiovascular dysfunction
Severe swallowing dysfunction or diminished cough
reflex
Rapid progression of weakness
3.When to admit ICU?
Breathless at rest/talk
Inability to count to 15 in a single
Use of accessory respiratory muscles.
Increased respiratory or heart rate
Vital capacity <15–20 ml/kg or
Abnormal arterial blood gas or pulse
oximetry

Imminent sign of respiratory distress:
breath.
<1 litre.
measurements.

Up to 22% of patients with GBS require mechanical1.
ventilation within the first week of admission.
2. The Erasmus GBS Respiratory Insufficiency
Score (EGRIS) prognostic tool was developed to identify and calculates the probability
(1–90%) that a patient will require ventilation within 1 week of assessment.

19. EGRIS

20. Immunomodulatory therapy should be started if patients are unable to walk
independently for 10 metre.
Treatment should be considered, especially if these patients display rapidly
progressive weakness or other severe symptoms such as autonomic
dysfunction, bulbar failure or respiratory insufficiency.
4. When to start treatment?
Clinical trials have demonstrated a treatment effect for intravenous
immunoglobulin (IVIg) when started within 2 weeks ofthe onset of weakness
and for plasma exchange when started within 4 weeks.

21. 5. What are the treatment options?
IVIG (0.4 g/kg daily for 5 days) and plasma exchange (200-
250 ml plasma/kg) alternate day for 5 sessions) are equally
effective.
Plasma exchange followed by IVIG or vice versa is not
more effective than either treatment alone.
Intravenous immunoglobulin (IVIG) is usually the treatment
of choice as it is easier to administer and more widely
available.
Started within the first 4 (preferably 2) weeks from onset
who are unable to walk unaided.
Adverse effects of IVIG include headache, liver dysfunction,
hyponatraemia, acute renal failure, haemolytic anaemia and
exfoliative dermatitis.
Plasma exchange may be unsuitable for patients with
autonomic instability because of the large shift in fluid
balance, and risk of CRBSI.
Several RCT showed had shown no significant benefit
of corticosteroids for GBS.
Insufficient evidence to support the efficacy of add-on
intravenous methylprednisolone to IVIG-treated patients.

22. 6. How to monitor
includes observation of usage of
accessory respiratory muscles,
frequent check of vital capacity and
arterial blood gas if necessary.
Patient is deemed at risk of
respiratory failure if the vital
capacity is <15 ml/kg or <1.5 L
Routine measurement of respiratory
function is mandatory.

Muscle strength in the neck, arms and
legs should be assessed with Medical
Research Council (MRC) grading scale
and functional disability with GBS
disability scale.

Monitor swallowing and coughing
difficulties.

Autonomic dysfunction should be
assessed via ECG and monitoring of
heart rate, BP and bowel and bladder
function.
the disease
progression?
Two-third of the mortality of patients
with GBS occur during the recovery
phase, mostly due to cardiovascular
dysrhythmias and respiratory
dysfuntion.

23. Complications including sacral
sores, pressure ulcers, hospital acquired
infections (for example,
orthostatic pneumonia or urinary
tract infections) and DVT can occur in
bed-bound patients.
Complications more specific
to GBS, for example, the inability
to swallow safely in patients with
bulbar palsy; corneal ulceration in
patients with facial palsy; and limb
contractures, and ossification in
patients with limb weakness.
7. How to manage early
complications?
Standard practice preventive
measures and treatment are
recommended.
Adequate management of
complications is best undertaken by a
multidisciplinary team includes nurses,
physiotherapists, rehabilitation
specialists, occupational therapists,
speech therapists and dietitians

24. Treatment- related fluctuations
8. How to
manage
Clinical
Progression?
Approximately 40% of patients treated with plasma
exchange or IVIG do not improve within the first 4
weeks of treatment.
May consider retreating the patient with the same
treatment or changing to an alternative treatment, but no
evidence to support this approach will improve the
outcome.
Insufficient response to treatment
TRFs can occur in 6–10% of patients with GBS and are
defined as disease deterioration occurring within 2
months following an initial treatment-induced clinical
improvement or stabilization.
This condition might benefit from further treatment. A
common practice is to repeat the full course of IVIg or
plasma exchange in these patients.
In about 5% of patients with GBS, repeated clinical
deteriorations suggest a more chronic form of
disease, termed acute-onset CIDP.
Acute-onset CIDP typically presents with three or
more TRFs and/or clinical deterioration ≥8 weeks
after disease onset.
Chronic Inflammatory Demyelinating
Polyneuropathy (CIDP)

25. 9. What are the predicting outcomes?
80% of patients with GBS regain the ability to walk independently at
6 months after disease onset.
The probability of regaining walking ability can be calculated in
individual patients using the modified Erasmus GBS outcome score
(mEGOS) prognostic tool.
3-10% mortality due to cardiovascular and respiratory complication.
Risk factors for mortality include advanced age and severe disease
at onset.

26. Physical function Fatigue
10. Planning Rehabilitation
Arranging a rehabilitation
programme with a rehabilitation
specialist, physiotherapist and
occupational therapist
Occurs in 60–80% of patients
with GBS and is often one of the
most disabling complaints.
Patients with GBS can experience a range of long- term residual problems.
A graded and supervised exercise
programme might be useful in
reducing fatigue.

27. Planning Rehabilitation
Patients with GBS can experience a range of long- term residual problems.
Pain
Severe pain is reported in one-third of
patients with GBS 1 year after disease
onset.
Management includes encouragement
of mobilization and administration of
drugs for neuropathic pain.
Psychological distress:
Rapid loss of physical function
can be severely traumatised and
cause anxiety and/or depression.
Referral to a psychologist or
psychiatrist will be beneficial.

29. Thank you!
for listening!

30. Leonhard SE, Mandarakas MR, Gondim FAA, Bateman K, Ferreira MLB, Cornblath DR, van Doorn PA, Dourado
ME, Hughes RAC, Islam B, Kusunoki S, Pardo CA, Reisin R, Sejvar JJ, Shahrizaila N, Soares C, Umapathi T,
Wang Y, Yiu EM, Willison HJ, Jacobs BC. Diagnosis and management of Guillain-Barré syndrome in ten steps.
Nat Rev Neurol. 2019 Nov;15(11):671-683. doi: 10.1038/s41582-019-0250-9. Epub 2019 Sep 20. PMID:
31541214; PMCID: PMC6821638.
Resources:
Physician's Booklet on Guillan Barre Syndrome, Clinical Neurophysiology neuromuscular Section