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Guillain-Barré syndrome
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Guillain-Barré syndrome (GBS) (in French ,
in English , , etc.) is an acute inflammatory demyelinating polyneuropathy (AIDP), an autoimmune disease 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).
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Encyclopedia
Guillain-Barré syndrome (GBS) (in French ,
in English , , etc.) is an acute inflammatory demyelinating polyneuropathy (AIDP), an autoimmune disease 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 by plasmapheresis or intravenous 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 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.
Serum sickness can rarely manifest as the Guillain-Barre syndrome (GBS), one of its most serious complications. The most serious complications of serum sickness are nerve conditions and peripheral neuritis. Serum sickness is of itself a type of delayed allergic response.
GBS is a form of autoimmune disease with a delayed hypersensitivity reaction, or a rare manifestation of serum sickness, or transient syndrome resembling serum sickness with loss of appetite, nausea, vomiting, and stomach pain accompanied by weakness (tired feeling), chills, low grade fever and possible evidence of brain involvement, indicated by lethargy and migraine headaches, although one theory of the cause of migraine is a central nervous system (CNS) disorder, or Bickerstaff's brain stem encephalitis, a regional variant of GBS. Typical pain is occipital or in the back of the head. Alterations of consciousness go with this headache type affecting the brainstem implicated in the maintenance of arousal, but is a worrisome feature of this type of headache called a Bickerstaff migraine. Other features of Bickerstaff migraine go with involvement primarily of the brainstem including clumsiness and gait unsteadiness, ie. "pulling to the right"..
Signs and symptoms
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 dysesthesias (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.
Acute paralysis in Guillain-Barre syndrome is usually related to Na+ channel blocking factor in the cerebrospinal fluid. Morbid and iatrogenic events involving IV salt and water may occur unpredictably in this patient group, resulting in SIADH. It results from a deficit of sodium, or surplus of water due to iatrogenic fluid overload. It occurs in patients with Guillian Barre syndrome, meningitis, encephalitis, pneumonia, septicemia, severe malaria, bronchiolitis, RSV infection, or as a direct result of clinical insult. SIADH is often the first symptom of Guillain–Barré syndrome. Na overload is almost always iatrogenic. Rapid correction of hyponatremia can cause osmotic brain demyelination.
The symptoms are similar to those for progressive inflammatory neuropathy.
Clinical variants
Six different subtypes of Guillain-Barre syndrome (GBS) exist:
- Acute inflammatory demyelinating polyneuropathy (AIDP) is the most common form of GBS, and the term is often used synonymously with GBS. It is caused by an auto-immune response directed against Schwann cell membranes.
- 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 with the triad of ophthalmoplegia, ataxia, and areflexia. Anti-GQ1b antibodies are present in 90% of cases.
- Acute motor axonal neuropathy (AMAN), aka. Chinese Paralytic Syndrome, attacks motor nodes of Ranvier and is prevalent in China and Mexico. It is likely due to an auto-immune response directed against the axoplasm of peripheral nerves. The disease may be seasonal and recovery can be rapid. Anti-GD1a antibodies 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. Like AMAN, it is likely due to an auto-immune response directed against the axoplasm of peripheral nerves. Recovery is slow and often incomplete.
- Acute panautonomic neuropathy is the most rare variant of GBS. It is associated with a high mortality rate, due to cardiovascular involvement, and associated dysrhythmias.
- Bickerstaff’s brainstem encephalitis (BBE), is further variant of Guillain-Barré syndrome. It is characterized by acute onset of ophthalmoplegia, ataxia, disturbance of consciousness, hyperreflexia or Babinski’s sign (Bickerstaff, 1957; Al-Din et al.,1982). Large, irregular hyperintense lesions located mainly in the brainstem, especially in the pons, midbrain and medulla are described in the literature. BBE despite severe initial presentation usually has a good prognosis. MRI plays a critical role in the diagnosis of BBE.
A considerable number of BBE patients have associated axonal Guillain–Barré syndrome, indicative that the two disorders are closely related and form a continuous spectrum.
Diagnosis
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.
There currently is no cure for Guillain-Barre syndrome. However, there successful treatments have been proven effective against this syndrome.
- typical CSF findings include albumino-cytological dissociation, this is, an elevated protein level (100 - 1000 mg/dL) without an accompanying pleocytosis (increased cell count), as opposed to infectious causes. A sustained pleocytosis may indicate an alternative diagnosis such as infection.
Researchers speculate as to why protein is elevated without pleocytosis in the CSF. Infilitration of WBCs in the myelin are responsible for demyelination in the peripheral nerves. Specific, 2D gel electrophoresis showed proteins unspecifically affected in different inflammatory as well as non-inflammatory neurological diseases and may be of limited value as disease-related biochemical markers in GBS. [Proteome analysis of cerebrospinal fluid in Guillain–Barré syndrome]
- electromyography (EMG) and nerve conduction study (NCS) may show prolonged distal latencies, conduction slowing, conduction block, and temporal dispersion of compound action potential in demyelinating cases. In primary axonal damage, the findings include reduced amplitude of the action potentials without conduction slowing.
Diagnostic criteria
Required
- Progressive, relatively symmetrical weakness of 2 or more limbs due to neuropathy
- Areflexia
- Disease course < 4 weeks
- Exclusion of other causes (see below)
Supportive
- relatively symmetric weakness accompanied by numbness and/or tingling
- mild sensory involvement
- facial nerve or other cranial nerve involvement
- absence of fever
- typical CSF findings obtained from lumbar puncture
- electrophysiologic evidence of demyelination from electromyogram
Differential diagnosis
Treatment
Supportive care with monitoring of all vital functions is the cornerstone of successful management in the acute patient. Of greatest concern is respiratory failure due to paralysis of the diaphragm. Early intubation should be considered in any patient with a vital capacity (VC) <20 ml/kg, a Negative Inspiratory Force (NIF) <-25 cmH2O, more than 30% decrease in either VC or NIF within 24 hours, rapid progression of disease, or autonomic instability.
Once the patient is stabilized, treatment of the underlying condition should be initiated as soon as possible. Either high-dose intravenous immunoglobulins (IVIg) at 400 mg/kg for 5 days or plasmapheresis can be administered, as they are equally effective and a combination of the two is not significantly better than either alone. Therapy is no longer effective 2 weeks after the first motor symptoms appear, so treatment should be instituted as soon as possible. IVIg is usually used first because of its ease of administration and safety profile, with a total of five daily infusions for a total dose of 2 g/kg body weight (400 mg/kg each day). The use of intravenous immunoglobulins is not without risk, occasionally causing hepatitis, or in rare cases, renal failure if used for longer than five days. Glucocorticoids have not been found to be effective in GBS. If plasmapheresis is chosen, a dose of 40-50 mL/kg plasma exchange (PE) is administered four times over a week.
Following the acute phase, the patient may also need rehabilitation to regain lost functions. This treatment will focus on improving ADL (activities of daily living) functions such as brushing teeth, washing and getting dressed. Depending on the local structuring on health care, there will be established a team of different therapists and nurses according to patient needs. An occupational therapist can offer equipment (such as wheel chair and cutlery) to help the patient achieve ADL independence. A physiotherapist would plan a progressive training programme, and guide the patient to correct, functional movement, avoiding harmful compensations which might have a negative effect in the long run. A Speech and Language Therapist would be essential in the patient regaining speaking and swallowing ability if they were intubated and received a tracheostomy. The Speech and Language Therapist would also offer advice to the medical team regarding the swallowing abilities of the patient and would help the patient regain their communication ability pre- Dysarthria. There would also be a doctor,nurse and other team members involved depending on the needs of the patient. This team contribute with their knowledge to guide the patient towards his or her goals, and it is important that all goals set by the separate team members are relevant for the patient's own priorities. After rehabilitation the patient should be able to function in his or her own home and attend necessary training as needed.
Prognosis
Most of the time recovery starts after 4th week from the onset of the disease. Approximately 80% of patients have a complete recovery within a few months to a year, although minor findings may persist, such as areflexia. About 5–10% recover with severe disability, with most of such cases involving severe proximal motor and sensory axonal damage with inability of axonal regeneration. However, this is a grave disease and despite all improvements in treatment and supportive care, the death rate among patients with this disease is still about 2–3% even in the best intensive care units. Worldwide, the death rate runs slightly higher (4%), mostly from a lack of availability of life support equipment during the lengthy plateau lasting 4 to 6 weeks, and in some cases up to 1 year, when a ventilator is needed in the worst cases. About 5–10% of patients have one or more late relapses, in which case they are then classified as having chronic inflammatory demyelinating polyneuropathy (CIDP).
In the UK, one of the fastest recoveries known to date has been that of Anna Liquorish, who was submitted to Epsom General Hospital on 3 May 2008 after experiencing weakness firstly in the legs and left arm. Her deterioration was quick and she was moved to ICU on 7 May after suffering breathing difficulties, before being transferred to Atkinson Morley, a specialist hospital for people with brain, spine and nerve disorders. By this point, Anna was completely paralysed from the neck down and narrowly avoiding assisted breathing. Dr S Wilson of Atkinson Morley, who has diagnosed many GBS patients, noted the recovery as the fastest he had ever seen, after Anna returned to full time work on 2 June 2008, with little or no visible effects of the disease.
History
The disease was first described by the French physician Jean Landry in 1859. In 1916, Georges Guillain, Jean Alexandre Barré, and Andre Strohl diagnosed two soldiers with the illness and discovered the key diagnostic abnormality of increased spinal fluid protein production, but normal cell count.
GBS is also known as acute inflammatory demyelinating polyneuropathy, acute idiopathic polyradiculoneuritis, acute idiopathic polyneuritis, French Polio, Landry's ascending paralysis and Landry Guillain Barre syndrome.
Notable sufferers
- Morten Wieghorst, International Danish footballer who once won the Scottish Football Championship with Celtic FC. He was diagnosed in 2000, and missed a year of his footballing career before returning in November 2001. He played 2 games for Celtic before returning to Denmark with Brøndby IF.
- Andy Griffith, actor on Andy Griffith Show, and Matlock. He contracted Guillain-Barré in 1983.
- Rachel Chagall, actress, contracted GBS in 1982. In 1987 she portrayed Gabriela Brimmer, a notable disabilities activist.
- Joseph Heller, author, contracted GBS in 1981. This episode in his life is recounted in the autobiographical No Laughing Matter, which contains alternating chapters by Heller and his good friend Speed Vogel.
- Franklin D. Roosevelt, U.S. president. In 2003, a peer-reviewed study found that it was more likely that Roosevelt's paralysis--long attributed to poliomyelitis--was actually Guillain-Barré syndrome.
- Markus Babbel, former international footballer, contracted GBS in 2001, following a period suffering from the Epstein-Barr virus. He lost almost an entire year of his footballing career between the two illnesses and never again demonstrated the same level of ability that won him over 50 caps for Germany.
- Serge Payer, Canadian-born professional hockey player. After battling and overcoming the syndrome, He set up the Serge Payer foundation, which is dedicated to raising money for research into new treatments and cures for Guillain-Barré syndrome.
- Lucky Oceans, Grammy Award winning musician with Asleep at the Wheel was diagnosed with GBS in 2008.
- William “The Refrigerator” Perry, former professional American football player with the Chicago Bears was diagnosed with GBS in 2008.
- Tony Benn, British politician.
- Len Pasquarelli, sports writer and analyst for ESPN and resident of the Pro Football Writers of America, diagnosed in 2008.
External links
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- from GBS Association of New South Wales (AU)
- - Oceans is interviewed by Dr Norman Swan on ABC Radio National - (8th March 2009)
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