Guillain-Barré Syndrome

By: Brittany Wyger, MD (PGY-1)

Definition

Group of neuropathic conditions characterized by progressive weakness & dimished or absent reflexes.

Inflammatory neuropathy due to cross reactivity between neural antigens & antibodies induced by specific infections Campylobacter (most common) but also

reported following: Mycoplasma Pneumoniae, Hemophilus Influenza, CMV, EBV

Epidemiology

Estimated annual incidence in the United States is 1.65 to 1.79 per 100,000 persons

Incidence increases from 0.62 per 100,000 in those <9 years old to 2.66 per 100,000 in those 80-89 years old

Male-to-female ratio is 3:2

Preceding illness

Most common symptoms reported before onset of Guillain-Barré Syndrome are: fever, cough, sore throat and other upper respiratory symptoms

Infection with Epstein-Barr virus has been linked to milder forms of Guillain-Barré Syndrome

GI symptoms may be more likely to precede Guillain-Barré subtypes that are related to slower recovery and higher risk of residual disability

Guillain-Barré Syndrome Subtypes

Acute inflammatory demyelinating polyradiculopathy Multifocal peripheral demyelination, slow remyelination,

both humoral and cellular immune mechanisms Most common subtype (90% of Guillain-Barré Syndrome in

the US)

Acute motor axonal neuropathy Antibodies against gangliosides GM1, GD1a, GalNAc-GD1a

and GD1b in peripheral motor nerve axons 5-10% of GBS cases Only motor symptoms

Acute motor-sensory axonal neuropathy Similar to acute motor axonal neuropathy but with sensory

axonal degeneration, predominantly sensory involvement

Guillain-Barré Syndrome Subtypes

Miller Fisher syndrome Demyelination, immunoglobulin G antibodies against

gangliosides GQ1b, GD3 and GT1a Rare, 3% of GBS cases in US Bilateral opthalmoplegia, ataxia, areflexia, facial & bulbar

weakness in 50% of cases Trunk, extremity weakness occurs in 50% of cases

Acute autonomic neuropathy Mechanism is unclear Autonomic symptoms, sensory loss Recovery is slow and may be incomplete

Presentation

Presentation: Symmetrical weakness (ascending) Decreased or absent reflexes

Also commonly seen: Weakness, numbness, tingling & pain in the

limbs 25% of patients will have advancing weakness

that compromise the respiratory muscles- will require mechanical ventilation

Presentation

Respiratory failure is more common in patients with rapid progression of symptoms, upper limb weakness, autonomic dysfunction or bulbar palsy

Facial, oropharyngeal & oculomotor muscles may be affected because of cranial neuropathy

Autonomic symptoms include arrythmias, orthostasis, BP instability, urinary retention, decreased GI motility

Pain is reported in 50-89% of Guillain-Barré Syndrome patients Pain is severe, deep, aching or cramping in muscles or back Difficult to control because pain is nociceptive and/or neuropathic

Disease progression

Symptoms typically peak within 4 weeks, then plateau before resolving

Diagnosis

Clinical criteria for diagnosis include: Symmetric motor weakness (bilateral symptoms) Absent or decreased reflexes

Lumbar puncture: Elevated protein in CSF, normal WBC count Protein level may be normal in the 1st week of symptoms,

protein will be elevated in 90% of cases by then end of the 2nd week

Nerve conduction studies: Slowed conduction (<60% normal velocity) or blockage of

nerve conduction will be seen Must test at least 3 motor nerves & 3 sensory nerves, must

avoid sural nerve (often normal in GBS) Can be used to track progression of the illnes

Differential Diagnosis

Brainstem: Infection, stroke

Spinal cord: compression, myelopathy, poliomyelitis, transverse myelitis

Rhabdomyolysis

Myasthenia gravis

Toxicity: industrial chemicals and other toxins

Infectious, inflammatory or toxic myopathy

Lyme disease

Complications

Neuropathic pain

Autonomic dysfunction Hypotension, hypertension, arrythmias,

bladder and bowel dysfuntion

Increased risk of VTE

Bulbar dysfunction & swallow difficulty, risk of aspiration

Complications

Respiratory failure Close respiratory monitoring with frequent

measurement of negative inspiratory force (NIF) should be instituted initially on all all Guillain-Barré Syndrome patients

NIF is a noninvasive method to measure respiratory muscle strength

Acceptable NIF range in Guillain-Barré Syndrome is -20 to -40 at least

Normal NIF is more negative than -60

Complications

Complications

Predicts the need for mechanical ventilation

Bulbar symptoms

Inability to raise the head or flex the arms

Inadequate cough

Maximum expiratory pressure: < 40 cm H2O

Maximum inspiratory pressure: < 30 cm H2O

Vital capacity: < 60 percent of predicted or < 20 mL per kg

Vital capacity, maximum inspiratory pressure, or maximum expiratory pressure reduced by at least 30 percent

Treatment

Disease modifying therapy: IVIg- 400mg/kg/day x 5days OR 2g/kg/day x 2days Plasma exchange (Plasmapheresis) – optimal response

if performed within 7 days onset

Both IVIg and plasma exchange are equally effective according to the literature but combining them is not beneficial

Mild Guillain-Barré syndrome cases benefit from 2 sessions of plasma exchange

Severe disease often requires 4 sessions

Corticosteroids are not recommended and may in fact delay long term recovery

Treatment

Initial response to IVIg does not predict the outcome because patients may stabilize or continue to decline after therapy

Treatment

Supportive care

SQ anticoagulation & SCD’s to reduce risk of VTE

Swallow eval in patients with facial or oropharyngeal weakness- risk of aspiration

ICU monitoring- ANS dysfunction and risk of respiratory failure, patient can become very unstable very quickly

Treatment

Bladder & bowel care Foley catheter, enemas & laxatives, erythromycin for

treatment of ileus

ANS dysfunction Paroxysmal HTN (24%), Orthostatic hypotension (19%),

sustained HTN (3%) Severe HTN- use PRN Labetalol, Esmolol, Nitroprusside Hypotension- IV fluid boluses 1st, then low dose phenylephrine

Arrythmias Sustained sinus tachycardia (37%)- requires no treatment Bradycardia & asystole (4%)

Treatment

Patients with limited mobility should be closely monitored for skin breakdown and treated appropriately

Pain control- neuropathic pain in 40-50% of patients Gabapentin (Neurontin) Carbamazepine (Tegretol) NSAIDs TCA’s Tramadol Epidural morphine

Treatment

Physical therapy and rehabilitation is recommended to decrease residual deficits and increase speed of recovery

A supervised exercise program is also recommended to improve fatigue as well as functional abilities

Prognosis

Even with appropriate treatment, 3% of patients with Guillain-Barré Syndrome will die

25% of patients will require mechanical ventilation which increases mortality risk

Prognosis is worse in patients with rapid onset of symptoms, severe symptoms and in elderly patients

Neurological deficits persist in 20% of patients, half of these remain severely disabled

Up to 80% of patients experience persistent, severe fatigue after resolution of other symptoms

Prognosis

Predicts long-term disability

Absence of motor response

Diarrheal illness

Axonal involvement

Campylobacter jejuni or cytomegalovirus infection

Inability to walk at 14 days

Older age

Rapid progression of symptoms

Severity of symptoms at their peak

CME Questions

Q: A patient presents with leg pain. Which one of the following accompanying findings most consistently suggests Guillain-Barré syndrome? (check one)

A. Prominent bowel or bladder symptoms.

B. Cerebrospinal fluid leukocytosis.

C. Relatively symmetrical weakness of the limbs.

D. Normal results on nerve conduction studies.

CME Questions

Answer: C. Relatively symmetrical weakness of the

limbs

CME Questions

Q: Which one of the following statements about disease-modifying therapy for Guillain-Barre syndrome is correct? (check one)

A. Plasma exchange should be witheld for the first 30 days after symptom onset.

B. Intravenous immune globulin therapy should be started within two weeks of symptom onset, and should be considered for patients who are nonambulatory.

C. Most patients require six sessions of plasma exchange.

D. Corticosteroids are first-line treatment.

CME Questions

Answer: B. Intravenous immune globulin therapy should

be started within two weeks of symptom onset, and should be considered for patients who are nonambulatory.

THANK YOU!!