Guillain-Barre Syndrome

GBS is an autoimmune, monophasic, acute or subacute symmetrical predominantly motor infective polyneuropathy involving more than one peripheral nerve, also known as acute idiopathic demyelinating polyneuropathy (AIDP).

Clinically disease reaches a peak of disability by 4 weeks.

It is an acute ascending polyneuropathy which generally starts with gastrointestinal or respiratory infection at the age of 15-25 and usually ends with recovery. Otherwise having unknown etiology.

Campylobacter jejuni and cytomegalovirus infections are well-recognized causes of severe GBS.

Synonyms-

  • Acute infective polyneuropathy (AIP)
  • Landry Guillain-BarrĂ©-strohl syndrome (LGBSS)
  • Acute idiopathic polyneuropathy (AIP)
  • Acute idiopathic demyelinating polyneuropathy (AIDP)
  • Acute inflammatory demyelinating polyradiculoneuropathy (AIDP)

Predisposition factors-

  • Age- 15-25 years
  • Infection- Viral in the form of Epstein Barr virus cytomegalovirus, bacterial in the form of mycoplasma pneumonia Campylobacter jejuni.
  • Vaccination- Rabies, typhoid, tetanus, or influenza vaccination.
  • Surgery- After 4 to 5 weeks of major surgery patient may show signs of GBS.
  • Drugs- Prolonged use of antidepressant drugs like zimelidine or gold therapy which are neurotoxins are found to cause GBS.
  • Autoimmune- Due to the presence of an antigen CD +ve T cells.
  • Idiopathic

Types-

GBS spectrum extends to an acute motor axonal neuropathy and the Miller Fisher syndrome (a rare proximal form causing ocular muscle palsies, ataxia and areflexia).

Clinical features-

  • Symmetrical weakness or numbness in distal limb that progress proximally. Weakness is of LMN type.
  • Lower limbs are involved first preceding the upper limb and may progress to the trunk and cranial nerves which can lead to paralysis.
  • Wasting, hypotonia and a partial or complete loss of the associated deep tendon reflexes.
  • Sensory symptoms range from mild pain to paresthesia and tenderness on deep pressure but total loss of sensation is usually not seen.
  • The disease usually spreads within 30 minutes to 4 weeks. It may take on an average of 4 to 5 days for the weakness to reach its peak, then it remains at a plateau or it maintains a plateau for 15 to 20 days following which the patient recovers in 4 to 6 months generally. In rare cases the recovery may extend up to 2 years.
  • Sphincter and autonomic disturbance- orthostatic hypotension.
  • Visual impairment is rare, though papilledema is seen occasionally.

Pathology-

The disease influences the spinal roots and nerve processes, principally including the Schwann cell and this results in segmental demyelination of the nerve interaction at first, later there is a proliferation of Schwann cells. The axon stays intact however demyelinated and can conduct impulses with a much decreased speed.

Recovery takes place by remyelination of peripheral axons, though the myelin sheath recovered is thinner than before and the internodal distances less, but eventually the conduction velocity returns to normal limits.

Complications-

  • Respiratory tract infection
  • Deep vein thrombosis
  • Cardiac arrhythmias
  • Retention of urine (uncommon)

Investigation-

  • Nerve conduction velocity test
  • EMG
  • Antibody test against ganglioside
  • Raised CSF and normal glucose level

Differential diagnosis-

  • Botulism
  • Cord compression
  • Myasthenia
  • Myopathy

Management-

Medical management

  • Ventilatory support required in case of progression of paralysis when there is respiratory muscle weakness.
  • IvIg in the first 2 weeks reduces duration and severity of disease.
  • Plasmapheresis
  • Steroid therapy

Physiotherapy management-

Divided in 2 stages –

  1. Progressive stage
  2. Recovery stage

1.Progressive stage-

When the patient is in intensive care, main aims of treatment are-

  • Maintain tracheobronchial hygiene and prevent infection.
  • Maintain joint range of motion and prevent damage and deformity.
  • Maintain muscles properties
  • Pain relief
  • Prevention of pressure sores
  • Prevention of postural hypotension

Maintain tracheobronchial hygiene-

  • Intermittent positive pressure ventilation via a cuffed tracheostomy tube along postural drainage with change in position every two hourly
  • Suctioning by suction catheter till cough reflex reappears
  • Cough drainage by manual assistance by vibration, rib springing, shaking, percussion and manual mobilization with the help of ambu-bag.
  • When the patient is not on a ventilator, forced expiratory techniques like huff and cough and active breathing techniques like ACBT (active cycle of breathing technique) and AD (autogenic drainage).

  • Nebulization in case of respiratory infection.

Maintain joint range of motion and prevent damage and deformity-

  • Passive range of motion of all joints with 3 sets of 5 repetitions in a day, hip joint must be fully extended daily in side-lying.
  • Ankle, wrist, hands and feet need accurate passive movement as these areas will be least and last to recover.
  • Gradual stretching especially of biarticular muscles to prevent tightness
  • Static or dynamic splints to prevent contractures as well as support weak structures to prevent injury
  • Maintain hand and foot in neutral position by the help of splints or pillow.

Maintain muscle properties-

As muscles begin to atrophy very rapidly functional electrical stimulation or passive range of motion is given to maintain muscle property.

Pain relief-

  • TENS
  • Massage
  • Positioning joints in suspension

Prevention of pressure sores-

  • Change patient’s position in every 2 hour
  • Use of air and water beds
  • Gentle massage over pressure prone areas
  • Use of soft, clean and crease free bed sheet
  • Pressure sore management- ice cube massage around wound, UVR radiation, LASER etc. to treat the sores.

Prevention of postural hypotension-

Prevention by stimulation of vascular reflex by getting the patient into erect position by use of a tilt bed with elastocrepe bandage.

2.Recovery stage-

When a patient can maintain his own airway and ventilation and some motor recovery is occurring, the recovery stage begins.

In this stage motor improvement occurs more rapidly than sensory.

Although every patient has variable forms of recovery a basic treatment is-

  • To maintain ventilation
  • To improve joint range of motion
  • To strengthen and re-educate muscles
  • To improve sensory awareness
  • Restoration of normal function

To maintain ventilation-

Various breathing techniques such as ACBT, AD along with forced expiratory techniques along with deep breathing and thoracic mobility exercises.

To improve joint range of motion-

Peripheral joints require passive movement and splintage for considerable time. Overall body range of motion-

  • Chest expansion exercises
  • Shoulder blade squeeze
  • Shoulder shrugging
  • Scapation
  • Protraction-retraction
  • Elbow bending
  • Thigh supported pronation- supination of forearm
  • Wrist pumps
  • Straight leg raise SLR in supine prone and side lying
  • Knee bending
  • Pre gait training
  • Ankle toe movements
  • Clam shell
  • Pelvic tilts
  • Bridging 
  • Cat-cow
  • Wall squats
  • Sit-ups
  • Quadriceps drills
  • Buttock walking
  • Kneel standing
  • Kneel walking
  • Standing

To strengthen and re-educate muscles-

  • Neuromuscular facilitation techniques
  • Afferent stimulation of skin
  • Equilibrium and righting reactions
  • Progressive resistance exercises
  • Hydrotherapy
  • Suspension
  • springs/pulleys
  • Circuit training
  • Isometrics

To improve sensory awareness-

  • Cutaneous stimulation which assist in perceptual re-education
  • Proprioception
  • Visual and sensory improvement

Restoration of normal functions-

  • Use of walking aids, toilet training and gait training
  • Splinting or use of orthosis to enable the patient in his personal care e.g. hygiene, feeding and dressing

Reference-

  • Susan B. O’Sullivan, Thomas J. Schmitz and George D. Fulk (Physical rehabilitation)
  • Glady Samuel Raj ( Physiotherapy in neuro conditions)
  • Patrica A. Downie ( Cash’s textbook of neurology for physiotherapists )
  • Jayant Joshi and Prakash Kotwal ( Orthopaedics and applied physiotherapy)