Guillain-Barré syndrome : Causes, Symptoms and Prognosis

Background

Guillain-Barré syndrome is a medical emergency, with predominantly motor features; requiring constant monitoring and support of vital functions, often in ITU

Introduction

  • A heterogeneous group of immune-mediated processes characterised by motor (and some sensory, and autonomic) dysfunction. In its classic form, GBS is a rapidly progressive acute inflammatory demyelinating polyneuropathy characterised by a progressive symmetrical ascending LMN muscle weakness, paralysis, and hyporeflexia; with or without sensory or autonomic symptoms
  • Typically, it starts in the lower limb (with reduced tendon reflexes); and progresses into the arms; with mild distal sensory loss. 50% have cranial nerve involvement
  • Most common acquired inflammatory neuropathy. Although the cause is not fully understood, it is thought to be autoimmune; and in most patients, is associated with antecedent infection
  • Several variants. In some, there is an inflammatory demyelinating polyradiculoneuropathy; others affect the axon
  • Diagnosis is clinical; as the classical LP findings (v high protein) may not appear for a week
  • Treatment includes immunoglobulin OR plasma exchange; and, for severe cases, mechanical ventilation
  • Prognosis: 2% mortality; 5-10% require ventilation; 10% cannot walk independently at 1 yr; 30% have some deficit at 3 yrs; 2-5% recur
  • Complete paralysis is compatible with a full recovery

Epidemiology/risk factors

  • 1-2/100,000 pa.  Increased incidence in males. Peak ages: 15-35 and 50-75 yrs
  • Vaccination
  • Lymphomas – especially Hodgkin’s disease
  • Pregnancy – incidence decreases during pregnancy but increases in the months after delivery

Variants (6)

  • Acute inflammatory demyelinating polyneuropathy (AIDP) – is the most common form of GBS, and the term is often used synonymously with GBS. It is caused by an auto-immune response directed against Schwann cell membrane
  • Miller-Fisher Syndrome (MFS) – is a rare variant of GBS and manifests as a descending paralysis, proceeding in the reverse order of the more common form of GBS. It usually affects the ocular muscles first and presents with the triad of ophthalmoplegia, ataxia, and areflexia. There is little significant limb weakness
  • Acute motor axonal neuropathy (AMAN) (or Chinese Paralytic Syndrome)
  • Acute motor sensory axonal neuropathy (AMSAN)
  • Bickerstaff’s brainstem encephalitis (BBE)
  • Acute panautonomic neuropathy

Did you know?

  • The disorder was first described by the French physician Jean Landry in 1859. In 1916, Georges Guillain, Jean Alexandre Barré, and Andre Strohl diagnosed two soldiers with the illness; and discovered the key diagnostic abnormality of increased spinal fluid protein production, but normal cell count
  • GBS is also known as: Guillain-Barré-Strohl syndrome; and, Landry’s paralysis, Landry’s ascending paralysis, Kussmaul-Landry syndrome, Landry’s syndrome, Landry-Guillain-Barré syndrome, Landry-Kussmaul syndrome, acute inflammatory demyelinating polyneuropathy (AIDP), acute idiopathic polyradiculoneuritis, acute idiopathic polyneuritis and French Polio
  • Various famous people have had GBS: Joseph Heller, Franklin D. Roosevelt (paralysis long attributed to poliomyelitis), Markus Babbel, William “The Refrigerator” Perry, Tony Benn

Aetiology

  • In about 2/3rds of patients, the syndrome begins 5 days to 3 wk after a banal infectious disorder, surgery, or vaccination. Infection is the trigger in > 50% of patients
  • Common pathogens include Campylobacter jejuni (especially Penner serogroup 19), enteric viruses, herpesviruses, HIV, cytomegalovirus, Epstein-Barr virus, and Mycoplasma sp
  • A cluster of cases followed the swine flu vaccination program in 1975
  • Antibodies to ganglioside GM1 are found in 20-30% of cases. There is a strong association with ganglioside GQ1b and the Miller-Fisher syndrome

Symptoms

  • Can start with paraesthesis in toes; rapidly (hours) followed by LMN symmetrical weakness, usually beginning in the legs; ascends to arms; 90% maximal weakness at 3 wks
  • More marked proximally than distally
  • May involve intercostal or bulbar muscles, or muscles of mastication; causing respiratory failure, dysphasia or dysphagia (can be life-threatening)
  • Muscle wasting develops if axonal degeneration has occurred
  • Sensory complaints; also frequent (less so than motor though)
  • A few patients (possibly with a variant form) have significant, life-threatening autonomic dysfunction causing labile BP, inappropriate ADH secretion, tachycardia, cardiac arrhythmias, disturbed sweating, GI stasis (paralytic ileus), sphincter problems (eg urinary retention), and pupillary changes
  • Muscle/back pain (so differential diagnosis wide); it is important to not make light of the pain a patient with GBS experiences; gabapentin (or a similar neuropathic analgesic) is often required +/- a referral to the ‘pain team’
    Notes: (less commonly): disease affects upper limbs only; or cranial nerves alone; 50% facial and eyelid weakness; sphincter problems rare; can start in head or arms, then progress down; or, sensory symptoms minimal or absent; or asymmetrical; acute onset of bilateral facial palsy is usually due to GBS

Key questions

  • “When did the weakness start?”
  • “Have you had an infection recently?”

Signs

  • Fever normally absent
  • Sensory symptoms but limited signs
  • Flaccid paralysis of lower limbs, then upper limbs (usually)
  • Deep tendon reflexes are typically absent; plantars normal
  • Facial palsy (VII), ptosis
  • Tender muscles
    Note: if spinal level, assume spinal cord compresion, until otherwise proven

 

Investigation

Very high LP protein may not appear for 1 week

Blood

  • FBC, ESR, CRP
  • U+E, LFTs, Bone, Glucose ± ABG, if hypoxic (on sats)
  • Hep B/C/HIV
  • Protein electrophoresis

Other

  • LP (usually very high protein, 3-10 g/L; no/few WC; but it may not appear for up to 1 wk and does not develop in 10% of patients)
  • MRI (brain/spine; to exclude other diagnoses)
  • ECG (many different abnormalities may be seen, eg 2nd and 3rd degree AV block, T-wave abnormalities, ST depression, QRS widening, and a variety of arrhythmias)
  • CXR

Key Investigation

  • LP

Specialist Investigations

  • Ganglioside antobodies
  • Nerve conduction studies:
    – Initial testing detects slow nerve conduction velocities and evidence of segmental demyelination in 2/3 of patients
    – However, normal results do not exclude the diagnosis and should not delay treatment
    – Electrophysiologic studies may reveal marked slowing of motor and sensory conduction velocity, or evidence of denervation and axonal loss
    – The time course of the electrophysiologic changes does not necessarily parallel any clinical developments

Differential Diagnoses (including all causes of rapid paralysis)

Neuromuscular (causes of paralysis)

  • Brain: brainstem CVA (rapid onset), encephalitis
  • Spinal cord: spinal cord compression (usually UMN); transverse myelitis (sphincter disturbance and sensory level);  poliomyelitis (LMN; usually occurs in epidemics); tick paralysis (causes ascending paralysis but spares sensation); West Nile virus (causes headache, fever, and asymmetric flaccid paralysis but spares sensation)
  • Peripheral nerve: autoimmune, toxic and metabolic neuropathies (vasculitis, toxins, porphyria)
  • Neuromuscular junction: myasthenia gravis (intermittent and worsened by exertion); botulism (LMN; may cause fixed dilated pupils (in 50%) and prominent cranial nerve dysfunction with normal sensation)
  • Muscle: hypokalaemia, hyperkalaemia, myositis

Non-neuromuscular

  • Causes generalised muscle/joint pain (myalgia predominates; eg viral illness)

 

Treatment

Ventilate sooner rather than later

Treatment (first line)

Drugs

  • IV NORMAL IMMUNOGLOBULIN 400 mg/kg od, for 5 days; it has some benefit up to 1 month from disease onset (OR plasma exchange) – senior specialist decision
  • SC ENOXAPARIN 40 mg od (prophylaxis)

Procedures

  • IV line (+ fluids, if dry, or dysphagic)
  • OXYGEN, if hypoxic
    Note: therapy is otherwise symptomatic, the aim being to prevent such complications as respiratory failure or vascular collapse; eg volume replacement or teratment with pressor agents is sometimes required to counter hypotension

Nursing issues

  • Compression stockings (DVT/PE prevention). Careful nursing, with physio, to prevent pressure sores, chest infection, contractures – and maintain morale

Treatment (second line)

  • NG feeding, if dysphagic
  • Ventilation (if suspect paralysis ascending to respiratory muscles)
  • Early tracheostomy (muscle activity can take weeks-months to recover)
  • Temporary cardiac pacing (if bradycardic)

Prescribing issues

  • Steroids of no value

Key management decisions

  • Immunosuppression
  • Ventilation

Admit?

  • Yes

Bed plan

  • Medical admission, then neurology ward ± ITU

Referrals

  • Neurology ± ITU

 

The Rest

Don’t ignore people when they say they ‘cannot walk’. There are several treatable causes (GBS, sc compression, hyperkalaemia etc)

Complications

  • Urinary retention and extreme constipation can occur; so, catheterise, and pay attention to bowels
  • Respiratory failure (respiratory muscle weakess; 25% patients); 5-10% require ventilation
  • DVT/PE
  • Autonomic dysfunction

Follow-up

  • Neurology

Risk stratification

  • Respiratory involvement is very serious

Prognosis

  • Mortality < 2%.  About 25% of deaths occur during the first week and about 50% during the first month. 10% cannot walk independently at 1 yr
  • Most patients improve considerably over a period of months, but about 30% of adults and even more children have some residual weakness at 3 yr. Patients with residual defects may require retraining, orthopedic appliances, or surgery
  • 2-5% recur; some have further acute episodes; other develop chronic inflammatory demyelinating polyneuropathy

2° Prevention + Health promotion

  • Warn about recurrence, and progression to chronic form

Don’t forget

  • Give immunogloblins ASAP
  • Respiratory involvement is serious
  • VENTILATE SOONER > LATER; assess respiratory function 2xday
  • Manage on ITU if any concern
  • Examine regularly (progression)
  • If worried, get MRI to exclude sc compression
  • Complete paralysis compatible with full recovery

Red flags

  • Respiratory involvement

 

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