GUILLAIN BARRE SYNDROME/ ACUTE DEMYELINATING

POLYRADICULONEUROPATHY / POLYNEUROPATHY.
Definition.
A disorder in which an immune response within the body attacks the peripheral nervous system
but does not affect the brain or spinal cord.
It is a heterogeneous and inflammatory condition that results in demyelinization of peripheral
nerves and axonal degeneration resulting in acute, ascending, progressive neuropathy.
Guillain Barre syndrome variants.
Historically it was considered a single disorder, but is now recognized as an umbrella term with
several variants.
a) Acute inflammatory demyelinating polyradiculoneuropathy (AIDP).
Is a motor sensory demyelinating disorder.
Common clinically; 85 to 90% of cases.
b) Acute motor axonal neuropathy (AMAN).
Purely motor, high prevalence in children.
c) Acute motor and sensory axonal neuropathy (AMSAN).
Severe form of acute motor axonal neuropathy. Sensory nerves are affected.
d) Miller fisher syndrome.
A rare triad of ophthalmoplegia, ataxia and areflexia. Usually presents as descending
paralysis rather than ascending as seen in other GBS variants.
e) Acute panautonomic neuropathy.
Involves sympathetic and parasympathetic nervous systems.
f) Pure sensory Guillain barre syndrome.
Sensory loss, sensory ataxia and areflexia in a symmetrical and widespread pattern.
g) Pharyngeal-cervical-brachial weakness.
Oropharyngeal, neck and cervical muscle weakness.
Etiology.
This is an autoimmune condition that is commonly triggered by a previous viral or bacterial
infection usually described by an influenza like upper respiratory tract infection or an acute
gastroenteritis with fever;
 Campylobacter jejuni (Most common cause): Increased virulence as has antigens in its
capsule that are similar to myelin.
 Cytomegalovirus (second most common cause), Epstein-Barr virus, HBV, Herpes Zoster
and mycoplasma. HIV is also implicated.
In most cases the causative agent remains unidentified.
Pathophysiology.
There’s an autoimmune attack due to molecular mimicry between antibodies created from a
recent infection. The immune response cross-react and target antigens on peripheral nerve
myelin (that is ganglio-sides and glycolipids) covering the spinal roots and peripheral nerves
(cranial nerves may be involved). Myelin destruction is accompanied by inflammation. Nerve
conduction is reduced and may be blocked completely. This leads to flaccid paralysis.
Clinical manifestation.
 Pain in the lower back is often the first sign due to inflammatory damage to spinal nerve
roots.
 Subacute (over days to weeks) symmetrical ascending muscle weakness (lower to upper)
with areflexia.
 Ascending sensory dysfunction: Loss of proprioception, paresthesia.
 Autonomic dysfunction: arrhythmias, postural hypotension.
 Can ascend to involve the diaphragm (causing respiratory compromise) and cranial nerves
(facial weakness is common.)
Investigations.
a) Lumbar puncture. (Best initial test.)
 shows albuminous cytological dissociation (elevated CSF protein without elevation of
WBC count.)
 Elevated CSF protein reflects widespread inflammation of nerve roots.
b) Nerve conduction studies. (Most accurate test.)
 Decreased conduction velocity due to peripheral nerve demyelination.
c) Spirometry. (Major determinant for the need to admit the patient to the ICU.)
 Measure the forced vital capacity, >20Ml/kg.
d) Electrocardiography.
 Arrhythmias i.e. 2nd / 3rd degree AV block, T- wave abnormalities, etc.
Management.
Treatment of Guillain-Barré syndrome is symptomatic and focuses on lessening the severity and
speeding recovery.
The goal of treatment is to keep the body functioning until the nervous system recovers
I. Plasma exchange.
Involves mechanically removing the liquid portion of blood from the blood cells. The
blood cells are then put back into the body to manufacture more plasma to replace what
was removed. This helps ride plasma off certain antibodies that contribute to the
autoimmune attack on the peripheral nerves.

II. Administration of intravenous immunoglobulins.

 This procedure involves intravenous infusion of immunoglobulin containing healthy
antibodies from blood donors. A blood product that has been derived from large pools of
plasma donors, has the potential to block the damaging antibodies that may contribute to
autoimmune diseases like GBS. This is effective in patients unable to walk 10m unaided.
The major component is immunoglobulin G with a trace amount of immunoglobulin A.
The dose is set at 2g/kg, divided into 5 daily infusions of 400mg/kg for 5 consecutive
days.

III. Admission to the ICU for respiratory assistance.

Intubation - Done to patients with respiratory compromise with decreased neck flexor
muscles strength seen with inability to lift the head hence phrenic nerves are also affected
causing diaphragm paralysis and reduction of the forced vital capacity (FVC)- amount of
air the patient can forcefully exhale with maximum inhalation. Done to achieve good
airway and manage it.

IV. DVT prophylaxis

Subcutaneous heparin 5,000 units twice daily along with antiembolism stocking.

V. Physical therapy may be helpful in preventing complications and promoting motor skill
recovery.
Nursing diagnosis and Nursing management.
I. Impaired physical mobility related to muscle weakness/ Loss of muscle function as
evidenced by inability to move extremities.

Prevention of complications associated with immobility is a central concern and involves

maintaining skin integrity and preventing contractures.

 Interventions include:

 Turning and repositioning every 2 hours.

 Assessing the skin for redness or breakdown.
 Performing range-of-motion exercises.
 Keeping the skin clean and dry.
 Performing range of motion exercises.
 Encouraging coughing and deep breathing every 2 hours and as needed
 Keep head of bed elevated to 35-45°.
 Perform chest physiotherapy.
 Anticipate the need for mechanical ventilation to support pulmonary function
 Adequate oxygenation.
 Suction secretions as appropriate.
II. Ineffective breathing pattern related to ascending paralysis/ decreased lung expansion as
evidenced by altered chest expansion, cyanosis, altered ABGs.
Preventing respiratory complications is the main aim.
 Encouraging coughing and deep breathing every 2 hours and as needed
 Keep head of bed elevated to 35-45°.
 Perform chest physiotherapy.
 Anticipate the need for mechanical ventilation to support pulmonary function
 Adequate oxygenation.
 Suction secretions as appropriate.

III. Acute pain related to inflammation of nerves as evidenced by guarding behavior,

tachycardia.
 Managing pain is the main aim.
IV. Risk for constipation related to immobility / Loss of sensation.
Other nursing management include:
 Enteral feeding or parenteral nutrition may be indicated if swallowing becomes impaired.
 Provide support and education to the parent and child. The rapid onset and long recovery
can be difficult and can cause strain on the family and its finances. If residual disability
occurs, the family will need help adjusting and caring for their child.