PNS Guidelines GBS Flashcards

(40 cards)

1
Q

What is Guillain-Barré Syndrome (GBS)?

A

Acute immune-mediated polyradiculoneuropathy

GBS is characterized by rapid onset of muscle weakness and is considered an emergency due to potential respiratory failure.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What is the global incidence of GBS?

A

1–2 per 100,000 person-years

This statistic reflects the estimated frequency of GBS in the general population.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What is the peak age for GBS incidence?

A

50–70 years

Males are affected more frequently than females, with a ratio of 1.5:1.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What are the typical variants of GBS?

A

Motor-sensory and pure motor

Motor-sensory is the most common in Europe and North America, while pure motor is more prevalent in Asia.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

List some atypical variants of GBS.

A
  • Pharyngeal-cervical-brachial
  • Bilateral facial weakness with paresthesias
  • Paraparetic
  • Pure sensory
  • Miller Fisher syndrome
  • Bickerstaff brainstem encephalitis

These variants can present with different symptoms and may require specific diagnostic considerations.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What are the required diagnostic features for GBS?

A
  • Progressive limb weakness
  • Hypo/areflexia in affected limbs
  • Progression ≤4 weeks

The nadir of symptoms is usually reached by 2 weeks.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What supportive features may indicate GBS?

A
  • Symmetry
  • Mild sensory symptoms
  • Cranial nerve involvement
  • Autonomic dysfunction
  • Recent infection (≤6 weeks)

These features can help strengthen the diagnosis of GBS.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What laboratory finding is characteristic of GBS in CSF analysis?

A

Albuminocytologic dissociation (↑ protein, WBC <50/μL)

This finding is a hallmark of GBS but may not be evident in the early stages.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What are some red flags that may suggest alternative diagnoses instead of GBS?

A
  • Asymmetric weakness
  • Sensory level
  • Hyperreflexia/extensor plantars
  • Abdominal pain/vomiting
  • Nystagmus
  • Alterations to consciousness
  • Fever at onset

Recognizing these signs is crucial for differential diagnosis.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What is the role of electrodiagnostics in GBS?

A
  • Demyelinating/axonal patterns may be observed
  • Absent H-reflexes
  • Sensory conduction abnormalities

Initial findings can be normal, and specific patterns help to confirm the diagnosis.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What immunotherapy treatments are recommended for GBS?

A
  • Plasma Exchange (PE)
  • Intravenous Immunoglobulin (IVIg)

Both treatments are effective but have specific protocols and contraindications.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What is the recommended plasma exchange protocol for non-ambulatory patients?

A

4–5 exchanges (12–15 L total) over 1–2 weeks within 4 weeks of onset

This is a strong recommendation for patients with GBS DS grade 3 or more.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What should be avoided in the treatment of GBS?

A
  • Oral corticosteroids
  • Intravenous methylprednisolone
  • Eculizumab, alemtuzumab, cyclophosphamide

These treatments have been shown to be ineffective or harmful in GBS management.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What factors predict a poor outcome in GBS?

A
  • Older age
  • Preceding diarrhea
  • Severe weakness at onset
  • Low CMAP amplitudes (<20% LLN)

These factors can help clinicians assess prognosis.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What is the purpose of mEGOS in GBS?

A

Predicts inability to walk at 4/26 weeks

This scoring system helps in assessing the severity and potential recovery of patients.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What does mEGRIS predict in GBS patients?

A

Need for mechanical ventilation

This tool assists in identifying high-risk patients who may require intensive care.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Fill in the blank: The presence of _______ is a hallmark feature of Miller Fisher syndrome.

A

Anti-GQ1b antibodies

These antibodies are strongly associated with MFS and help in confirming the diagnosis.

18
Q

What is the initial treatment for pain in GBS?

A
  • Gabapentin
  • Carbamazepine
  • Tricyclic antidepressants

These medications can help manage neuropathic pain in GBS patients.

19
Q

What supportive care strategies should be employed in GBS patients?

A
  • ICU monitoring
  • Pain management
  • Fatigue management

Supportive care is critical in managing complications and improving quality of life.

20
Q

True or False: Corticosteroids are recommended for the treatment of GBS.

A

False

Corticosteroids have been shown to be ineffective and may worsen outcomes in GBS.

21
Q

What is A-CIDP and when should it be suspected?

A

A-CIDP (Acute Chronic Inflammatory Demyelinating Polyneuropathy) should be suspected if progression >8 weeks or ≥3 treatment-related fluctuations

Recognizing A-CIDP is important for appropriate management.

22
Q

What is a key takeaway regarding GBS diagnosis?

A

Diagnose clinically + CSF/electrodiagnostics; reserve antibodies/MRI for atypical cases

This approach ensures timely and accurate diagnosis.

23
Q

What are the diagnostic criteria for motor-sensory or motor GBS?

A

Features required: Progressive weakness of arms and legs; Tendon reflexes absent or decreased in affected limbs; Progressive worsening ≤ 4 weeks.

None

24
Q

What features support the diagnosis of GBS?

A

Relative symmetry; Relatively mild/absent sensory symptoms and signs; Cranial nerve involvement; Autonomic dysfunction; Respiratory insufficiency; Pain; Recent history of infection; CSF: protein increased; white cells: usually < 5x10€ /1; Electrodiagnosis: nerve conduction studies consistent with polyneuropathy.

None

25
What are some findings that make GBS less likely?
Asymmetric weakness; Severe respiratory dysfunction with mild limb weakness; Predominant sensory signs with mild weakness; Fever; Hyperreflexia; Upper motor neuron involvement; Bladder/bowel dysfunction; Abdominal pain or vomiting; Nystagmus; Alteration of consciousness (except in BBE); CSF: >50×105 white cells/l; Blood: abnormal routine tests; No further worsening after 24 h; Relatively slow worsening (period 2-4 weeks); Continued worsening > 4 weeks (consider A-CIDP). ## Footnote None
26
What are the criteria for GBS variants or MFS spectrum?
GBS variants: Criteria as for motor-sensory GBS but neurological deficit in a distribution other than weakness of all limbs. ## Footnote None
27
What characterizes Miller Fisher Syndrome (MFS)?
Ophthalmoplegia, ataxia, areflexia = typical (incomplete forms exist); If also limb weakness = GBS/MFS overlap syndrome; Progressive worsening ≤ 4 weeks; CSF: protein may be elevated; usually < 5x10° cells/l; Blood: usually GQ1b antibodies. ## Footnote None
28
What defines Bickerstaff brainstem encephalitis (BBE)?
As for MFS, except impaired consciousness, pyramidal signs; MRI brain may show white matter changes; CSF: white cell count may be raised; Blood: often GQ1b antibodies. ## Footnote None
29
What are the estimated percentages for motor-sensory GBS and motor GBS?
Motor-sensory GBS: 30-85%; Motor GBS: 5-25%. ## Footnote None
30
What are the classifications of GBS variants?
GBS variants (5-10%): Paraparetic; Pharyngeal-cervical-brachial; Pure sensory. ## Footnote None
31
What are suspicious features for A-CIDP?
Slower progression, 23 TRF's, worsening >8 wks; EMG (early phase): motor nerve conduction slowing; Nodo-paranodal antibody testing advised; Consider: nerve ultrasound. ## Footnote See: 'CIDP EAN/PNS Guideline' for diagnosis
32
What is the required clinical presentation for typical CIDP?
Symmetrical proximal and distal weakness of both upper and lower limbs developing over at least 8 weeks, with reduced or absent tendon reflexes.
33
What antibodies are relevant for GBS diagnosis?
Anti-ganglioside antibodies (e.g., GM1, GQ1b), especially in AMAN, MFS.
34
What are 3 regional variants of GBS mentioned in the guideline?
• Pharyngeal–cervical–brachial • Bilateral facial palsy with paresthesias • Paraparetic variant
35
Should anti-ganglioside antibody testing be done in typical GBS?
Generally no, except anti-GQ1b in MFS and nodal/paranodal antibodies in suspected nodopathies
36
What role does nerve imaging (MRI or ultrasound) play in GBS?
Considered in atypical cases to assess nerve root enhancement or thickening
37
What are the early Electrodiagnostic features of GBS ?
Early findings: Absent H-reflexes sensory conduction abnormalities in keeping with a polyneuropathy facial nerve direct responses showing increased motor latencies or decreased CMAPs Blink responses showing absent responses or prolonged R1 and R2 responses and contralateral R2 response.
38
What are the supportive Electrodiagnostic features in GBS ?
Sural sparing Indirect discharges (often multiple and resembling A waves and distinct from F waves) prolonged distal CMAP duration >8.5 ms (time from onset of first negative deflection to return to baseline of last negative deflection, using a filter bandpass of 2 Hz–10 kHz).
39
When should a second IVIG course be considered ?
Only in cases of clear TRF ( Improvement initially followed by deterioration ( 2-4 weeks))
40
When should repeat IVIG not be given ?
Poor MEGOS score of >6 at Day 7 Deterioration despite IVIG or PE after first course