Guillain-Barré syndrome

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Guillain-Barré syndrome 
Gullain-Barré syndrome (GBS) (in French pronounced [ɡilɛ̃ baˈʁe]
[1]
, in English 
pronounced /ˈgiːlæn ˈbɑːreɪ/
[2]
, /giːˈæn bəˈreɪ/,
[3]
 etc.
[4]
) is an acute, autoimmune, 
polyradiculoneuropathy affecting the peripheral nervous system, usually triggered by 
an acute infectious process. It is included in the wider group of peripheral 
neuropathies. There are several types of GBS, but unless otherwise stated, GBS refers 
to the most common form, acute inflammatory demyelinating polyneuropathy 
(AIDP). It is frequently severe and usually exhibits as an ascending paralysis noted by 
weakness in the legs that spreads to the upper limbs and the face along with complete 
loss of deep tendon reflexes. With prompt treatment of plasmapheresis followed by 
immunoglobulins and supportive care, the majority of patients will regain full 
functional capacity. However, death may occur if severe pulmonary complications 
and dysautonomia are present. 
P A T H O P H Y S I O L O G Y 
All forms of Guillain-Barré syndrome are due to an immune response to foreign 
antigens (such as infectious agents or vaccines) but mistargeted to host nerve tissues 
instead (a form of antigenic mimicry). The targets of such immune attack are thought 
to be gangliosides, which are complex glycosphingolipids present in large quantities 
on human nerve tissues, especially in the nodes of Ranvier. An example is the GM1 
ganglioside, which can be affected in as many as 20-50% of cases, especially in those 
preceded by Campylobacter jejuni infections. Another example is the GQ1b 
ganglioside, which is the target in the Miller Fisher syndrome variant (see below). 
The end result of such autoimmune attack on the peripheral nerves is inflammation of 
myelin and conduction block, leading to a muscle paralysis that may be accompanied 
by sensory or autonomic disturbances. 
However, in mild cases, axonal function remains intact and recovery can be rapid if 
remyelination occurs. In severe cases, such as in the AMAN or AMSAN variants (see 
below), axonal degeneration occurs, and recovery depends on axonal regeneration. 
Recovery becomes much slower, and there is a greater degree of residual damage. 
Recent studies on the disease have demonstrated that approximately 80% of the 
patients have myelin loss, whereas, in the remaining 20%, the pathologic hallmark of 
the disease is indeed axon loss. 
S I G N S A N D S Y M P T O M S  
The disease is characterized by weakness which affects the lower limbs first, and 
rapidly progresses in an ascending fashion. Patients generally notice weakness in their 
legs, manifesting as "rubbery legs" or legs that tend to buckle, with or without 
dysthesias (numbness or tingling). As the weakness progresses upward, usually over 
periods of hours to days, the arms and facial muscles also become affected. 
Frequently, the lower cranial nerves may be affected, leading to bulbar weakness, 
(oropharyngeal dysphagia, that is difficulty with swallowing, drooling, and/or 

maintaining an open airway) and respiratory difficulties. Most patients require 
hospitalization and about 30% require ventilatory assistance. Facial weakness is also 
commonly a feature, but eye movement abnormalities are not commonly seen in 
ascending GBS, but are a prominent feature in the Miller-Fisher variant (see below.) 
Sensory loss, if present, usually takes the form of loss of proprioception (position 
sense) and areflexia (complete loss of deep tendon reflexes), an important feature of 
GBS. Loss of pain and temperature sensation is usually mild. In fact, pain is a 
common symptom in GBS, presenting as deep aching pain usually in the weakened 
muscles, which patients compare to the pain from overexercising. These pains are 
self-limited and should be treated with standard analgesics. Bladder dysfunction may 
occur in severe cases but should be transient. If severe, spinal cord disease should be 
suspected. 
Fever should not be present, and if it is, another cause should be suspected. 
In severe cases of GBS, loss of autonomic function is common, manifesting as wide 
fluctuations in blood pressure, orthostatic hypotension, and cardiac arrhythmias. 
The symptoms are similar to those for progressive inflammatory neuropathy.
[5] 
Clinical variants 
Although ascending paralysis is the most common form of spread in GBS, other 
variants also exist. 
  Miller Fisher Syndrome (MFS) is a rare variant of GBS and manifests as a 
descending paralysis, proceeding in the reverse order of the more common 
form of GBS. It usually affects the ocular muscles first and presents as 
ophthalmoplegia, ataxia, and areflexia. Anti-GQ1b antibodies are present in 
90% of cases. 
  Acute motor axonal neuropathy (AMAN)
[6]
, aka. Chinese Paralytic 
Syndrome, attacks motor nodes of Ranvier and is prevalent in China and 
Mexico. The disease may be seasonal and recovery can be rapid. Anti-GD1a 
antibodies
[7]
 are present. Anti-GD3 antibodies are found more frequently in 
AMAN 
  Acute motor sensory axonal neuropathy (AMSAN) is similar to AMAN but 
also affects sensory nerves with severe axonal damage. Recovery is slow and 
often incomplete
[8]
. 
D I A G N O S I S  
The diagnosis of GBS usually depends on findings such as rapid development of 
muscle paralysis, areflexia, absence of fever, and a likely inciting event. CSF and 
ECD is used almost every time to verify symptoms, but because of the acute nature of 
the disease, they may not become abnormal until after the first week of onset of signs 
and symptoms. 

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Guillain-Barré syndrome Gullain-Barré syndrome (GBS) (in French pronounced [ɡilɛ̃ baˈʁe][1], in English pronounced /ˈgiːlæn ˈbɑːreɪ/[2], /giːˈæn bəˈreɪ/,[3] etc.[4]) is an acute, autoimmune, polyradiculoneuropathy affecting the peripheral nervous system, usually triggered by an acute infectious process. It is included in the wider group of peripheral neuropathies. There are several types of GBS, but unless otherwise stated, GBS refers to the most common form, acute inflammatory demyelinating polyneuropathy (AIDP). It is frequently severe and usually exhibits as an ascending paralysis noted by weakness in the legs that spreads to the upper limbs and the face along with complete loss of deep tendon reflexes. With prompt treatment of plasmapheresis followed by immunoglobulins and supportive care, the majority of patients will regain full functional capacity. However, death may occur if severe pulmonary complications and dysautonomia are present. Pathophysiology All forms of Guillain-Barré syndrome are due to an immune response to foreign antigens (such as infectious agents or vaccines) but mistargeted to host nerve tissues instead (a form of antigenic mimicry). The targets of such immune attack are thought to be gangliosides, which are complex glycosphingolipids present in large quantities on human nerve tissues, especially in the nodes of Ranvier. An example is the GM1 ganglioside, which can be affected in as many as 20-50% of cases, especially in those ...
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