Guillain Barre Syndrome

Guillain Barre Syndrome (GBS) is a rare autoimmune disorder in which our body's immune system attacks the protective covering, the myelin sheath, that surrounds the axon of many peripheral nerves resulting to axonal degeneration. The resultant inflammation and demyelination of the nerves decreases the nerve conduction velocity leading to weakness or sometimes paralysis of the affected muscles. This disorder predominantly affect the motor fibers.  GBS is known from other names: 

• Acute Inflammatory demyelinating polyneuropathy
• Landry's Ascending Paralysis

Main Types: 

1. Acute Inflammatory Demyelinating Polyneuropathy (AIDP)

• The most common type of GBS, particularly in the US. The myelin sheath of the nerves is damage preventing signals to travel to the brain causing weakness, paralysis, or numbness. It is characterized by motor weakness in a distal to proximal progression.

2. Acute Motor Axonal Neuropathy (AMAN)

• It is the same with AIDP, but purely motor and has no sensory symptoms. Usual cases occur during pediatric age. 

3. Acute Motor-Sensory Axonal Neuropathy (AMSAN)

• A severe form of GBS to include both motor and sensory symptoms. It is more prevalent in Asia and in North and South America. Typically, seen during adulthood.

4. Miller Fisher Syndrome

• This type of GBS is more common in Asia. Paralysis starts in the eyes.
• Double vision, loss of balance, and unsteady gait are some features.

Causes:

The exact etiology is unknown. It is often preceded by previous respiratory infection, stomach flu, viral infection (Influenza), vaccination, or surgery.

Symptoms:

The first symptoms is rapid onset of symmetrical muscle weakness and sometimes paralysis or an abnormal sensation in the legs that travel up the limb from fingers and toes towards the upper extremities and head. GBS symptoms usually last for a few weeks, usually peaks within 2 to 4 weeks. Severe cases involving the respiratory muscles that interfere with breathing require immediate medical attention. Other findings may include:

1. Deep muscle pain/Severe nerve pain 

2. Absence of deep tendon reflexes - Knee jerk

3. Breathing problems such shortness of breath and dyspnea on exertion

4. Heart and blood pressure problems such as paroxysmal hypertension and orthostatic hypotension.
5. Coordination and balance difficulties 
6. Blurred or double vision

7. Bowel and bladder weakness

8. Swallowing, chewing, or speaking difficulties(slurred speech)

Risk factors:

1. This disorder can occur at any age. Usually common to young adults and older adults starting at their 50's.

2. It can affect both sexes, but slightly greater in male population. 

3. Conditions associated with GBS include:

• Viral infection - HIV, Influenza
• Bacterial infection - Campylobacter
• Mycoplasma Pneumonia
• Cytomegalovirus
• Epstein-Barr Syndrome
• Surgery
• Vaccination

Diagnosis:

1. Lumbar puncture - Cerebrospinal fluids contain high protein with little or no lymphocytes.

2. Electromyography measures muscle response or electrical activity in response to stimulation of the nerves of the muscles to determine neuromuscular problems.

3. Nerve Conduction Studies measures the speed of conduction of an electrical impulse through a nerve. 

4. Physical and Neurologic examination

5.  Muscle Strength Testing

6. Medical History/Surgery

Treatment:

There is no known cure for GBS, but still there are several treatment to lessen the effects of symptoms and to help speed up recovery. Most patient may recover from GBS, while some of them may still experience weakness and fatigue. Immediate hospitalization is highly recommended to receive appropriate treatment and a good chance of recovery.

A. Pharmacologic Approach

1. Plasma Exchange (Plasmapheresis)

• A blood-cleansing procedure in which the whole blood is withdrawn and the liquid portion, the plasma, is separated. The affected plasma is replaced with a new plasma, or a  plasma substitute and then transfused back to the person.

2. Intravenous immunoglobulin (IVIGs) help improve immune system.

3. Analgesics, such as NSAIDs or Acetaminophen with added narcotics, is usually recommended for pain relief. Corticosteroids are not recommended in GBS.

B. Cardiac Monitoring for arrhythmias, heart rate, postural hypotension.

C. Mechanical Ventilation during the acute stage to assist breathing problems.

D. Physical Therapy

Rehabilitation will help to:
1. Regain independence and quality of life.

2. Improve balance, posture, and coordination

3. Promote relaxation

4. Promote recovery

5. Increase muscle strength and endurance

6. Increase joint mobility and flexibility.

The rehabilitation is a team effort among physical, occupational and speech therapists, nurse/caregiver, and family. Physical therapy management include:

• Respiratory care to include regular breathing exercise and airway clearance.
• Passive range of motion exercises to prevent joint stiffness.
• Light exercises may be allowed but should avoid overexertion and fatigue to avoid exacerbation of symptoms.
• Resting splints to support the wrist/hands and ankles.
• As the patient condition improves and is out of bed, approach on increasing the circulation is important. Strengthening and endurance exercises may be given as tolerated.
• Swelling is likely to develop in the ankles and hands if it remained immobile.
• Monitor the blood pressure as well as the possibilities of having orthostatic hypotension.
• Compression stocking helps with swelling, blood clots, and achy leg.
• Abdominal binder help support weak abdominal muscles.
• A shoulder joint support will also help prevent shoulder dislocation or subluxation.
• A wheelchair for functional mobility is also prescribed to help patient to move around.
• Gait training to regain independence in walking.
• Education and home program.