Guillain Barre Syndrome is another extremely uncommon disorder of the peripheral nervous system in which paralysis of the body develops. Paralysis may be ascending or descending, however, ascending paralysis is the most common pattern of Guillain-Barre Syndrome. Unlike disorders of central nervous system like multiple sclerosis or amyotrophic lateral sclerosis, Guillain Barre Syndrome is a peripheral nervous system disorder most commonly.
What are the other names for Guillain Barre Syndrome ?
This syndrome is sometimes nameD as;
u Landry's paralysis or Landry's ascending paralysis
u Guillain-Barre-Strohl syndrome
u Acute idiopathic polyradiculoneuritis
u French polio
u Acute idiopathic polyneuritis
u Landry-Guillain-Barre syndrome
When was the first case diagnosed?
In the middle of nineteenth century, a French doctor Jean Landry described the details about this syndrome. In early twentieth century, two soldiers had this type of abnormality. Spinal tap was performed which showed excessive protein but normal cell count. Georges Guillain, Jean Barre and Andre Strohl were the notable physicians working on these patients.
How do we classify this syndrome?
Guillain Barre Syndrome has been classified into six different subtypes according to the pattern of paralysis;
u Acute inflammatory demyelinating polyneuropathy (AIDP) - most common type and occurs due an immune mediated reaction against specialized cells making myelin sheat around the peripheral nerves, the Schwann cells.
u Acute motor axonal neuropathy (AMAN) - commonly occurs in Chinese and Mexicans, so name as Chinese paralytic syndrome. Occurs due to autoimmune reaction against the axoplasm of peripheral nerves. Commonly attacks the nodes of Ranvier. Anti GD1a and anti GD3 antibodies are responsible. It shows seasonal variation and rapid recovery.
u Acute motor sensory axonal neuropathy (ASMAN) - characteristics are similar to acute motor type but recovery is gradual and incomplete.
u Miller Fischer Syndrome (MFS) - a rare subtype of Guillain Barre Syndrome. Occurs in almost five percent of all the cases. Defining feature is descending paralysis characterized by triad of ataxia, ophthalmoplegia and areflexia.
u Acute panautonomic neuropathy - most uncommon subtype. Characterized by autonomic impairment accompanied with encephalopathy and arrhythmias.
u Bickerstaff's brainstem encephalitis (BEE) - in such cases lesion is present in brainstem.
What are the causes of this syndrome?
It is an autoimmune disorder in which the antigens of foreign invading agents are targeted against the host tissues. These most common invading agents are microorganisms like;
u Campylobacter jejuni (most common cause)
u Cytomegalovirus (CMV)
u Influenza virus
Almost sixty percent of the cases do not have any recognized cause (idiopathic).
How does it present clinically?
The most common clinical manifestation of this syndrome is ascending paralysis in which there is weakness of lower limbs followed by weakness of upper limbs, trunk and face within few hours to days.
There is difficulty in swallowing and breathing when lower cranial nerves get involved.
In severe cases, respiratory failure occurs due to paralysis of respiratory muscles including diaphragm.
Although sensory loss is not typical of this syndrome, however, in few selected cases, it may be present.
In severe cases signs of autonomic instability like orthostatic hypotension and arrhythmias may be present.
How to diagonise Guillain Barre Syndrome ?
Important investigations are;
u Lumbar puncture - cerebrospinal fluid shows albumin-cytologic dissociation, i.e., there is increase in protein content without an increase in cell count.
u Nerve conduction studies show slowed conduction
How to treat Guillain Barre Syndrome ?
Emergency - in acute cases when there is respiratory failure, intubation is done and if necessary mechanical ventilation is started.
Routine - cases usually diagnosed without any emergent condition are treated with the help of either plasmapheresis or intravenous immunoglobulins.
Plasmapheresis - it is a process of filtering antibodies from the blood which are going to attack the host tissues.
Robbin's Principles of Internal Medicine 18th Edition