Inflammatory Polyneuropathy (Guillain-Barre Syndrome)
Inflammatory Polyneuropathy (Guillain-Barre Syndrome)
General and supportive measures
G61.0
* Notifiable condition
DESCRIPTION
Guillain-Barré syndrome (GBS) is an acute autoimmune-mediated polyradiculoneuropathy which is precipitated by a preceding viral or other infection. It is the most common acquired polyneuropathy in children.
Different forms or variants of Guillain-Barré syndrome are described depending on the clinical and/or neurophysiological characteristics.
Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP)
- This is the most common form, accounting for 80–90% of cases.
- Characterised mainly by:
- symmetrical, ascending motor weakness,
- areflexia, i.e. absence of tendon reflexes,
- distal sensory alteration,
- pain/paraesthesia.
Acute Motor Axonal Neuropathy (AMAN)
- A purely motor form of GBS.
- It involves predominantly motor nerves and has an axonal pattern on electrophysiology (nerve conduction studies).
- Although there are similarities with AIDP, the clinical picture tends to be more severe with more patients suffering from respiratory failure.
Acute Motor-Sensory Axonal Neuropathy (AMSAN)
- Another axonal form of GBS but with sensory involvement.
- It is not frequently found in children.
Miller-Fisher syndrome
- Patients have external ophthalmoplegia, sensory ataxia, weakness with areflexia.
- Electrophysiological and CSF studies are similar to AIDP.
Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP)
- May be considered a chronic variant of AIDP.
- Most often starts insidiously and progresses slowly, but can have onset like GBS.
- It is managed differently from GBS and should be referred.
DIAGNOSTIC CRITERIA
Clinical
- Preceding respiratory tract or gastrointestinal infection.
- Symmetrical, flaccid muscle weakness with early areflexia.
- The muscle weakness may ascend rapidly upwards to involve the trunk, arms, face and cranial nerves.
- Bulbar paralysis and respiratory failure may develop.
- Autonomic dysfunction.
- Relatively mild, or absence of, sensory signs.
- Exclude the following:
- Acute Disseminated Encephalomyelitis (ADEM),
- poliomyelitis, a rare cause of hypotonia with abrupt onset of weakness (usually asymmetrical) in association with a febrile illness,
- transverse myelitis:
- initial flaccid weakness and areflexia typically involving the lower limbs maximally,
- occasionally with pain at the onset, but rapidly progressing to spasticity and hyperreflexia,
- a sensory level on the trunk,
- bladder and rectal sphincter involvement.
- diphtheria,
- botulism.
Investigations
Follow the Acute Flaccid Paralysis (AFP) investigation protocol
- Send two stool specimens taken 24–48 hours apart to the National Institute of Virology via the local laboratory.
- The stool sample needs to be sent within 14 days of onset of paralysis to exclude poliovirus infection.
CSF
- CSF findings after 1 week show elevated protein and few or no cells, i.e. albumino-cytological dissociation.
- CSF glucose is normal.
GENERAL AND SUPPORTIVE MEASURES
- Notify as AFP.
- Admit to a high or intensive care unit.
- Monitor respiratory and autonomic functions closely:
- peak expiratory flow rate,
- blood pressure,
- respiratory rate,
- heart rate,
- forced vital capacity (FVC),
- bulbar functions,
- arterial blood gases.
- Ventilation is recommended when:
- rapidly progressing ascending paralysis, including shoulder weakness, head lag, weak cough and swallowing difficulties,
- there is a progressive fall in the peak expiratory flow rate,
- tachycardia and sweating occur,
- inspiratory efforts are weak (typical signs of respiratory distress will be absent),
- inability to talk.
- PaCO2 levels start rising.
Note:
These changes precede hypoxaemia detected on blood gas analysis, and ventilation should begin before frank hypoxaemia occurs.
Respiratory care must be meticulous.
- To determine fluid losses from autonomic instability, monitor urine output and degree of sweating.
- Provide adequate nutrition.
- Provide bladder and bowel care as well as pressure-point care.
- Routine physiotherapy for chest and limbs, keep ankles in neutral position (90˚) (may require foot/hand splints).
- Protect eyes and keep moist.
- Communicate with child as awareness is maintained. Staff should remember that children may be very frightened but unable to express their emotions and needs.
MEDICINE TREATMENT
- Immunoglobulin, IV, 1 g/kg/day, slowly over 12–16 hours on two consecutive days or 0.4 g/kg as a single daily dose on 5 consecutive days early in the disease process.
- Use under specialist supervision.
Substantial pain is present (in up to 90%) in the severely affected patients.
Pain in this setting is often unrecognised and underestimated.
Pain management is essential. See: Management of pain.
For neuropathic pain:
- Carbamazepine, oral, 5 mg/kg/dose 12 hourly.
REFERRAL
- Chronic inflammatory demyelinating polyradiculoneuropathy.
- Guillain-Barré syndrome with bulbar paralysis and/or early signs of respiratory failure.
- Patients who have lost or are losing ambulation for management in consultation with a paediatric neurologist.
- Patients with complex Guillain-Barré syndrome.
Click here to access NEMLC Report.
Standard Treatment Guidelines and Essential Medicines List for South Africa, Paediatric Hospital Level_Updated May 2025
Chapter 13: The Nervous System_13.7 Neuromuscular Disorders_13.7.1 Inflammatory Polyneuropathy (Guillain-Barré Syndrome)