Wikia

Psychology Wiki

Changes: Guillain-Barré syndrome

Edit

Back to page

(-L)
(update wp)
 
Line 1: Line 1:
 
{{ClinPsy}}
 
{{ClinPsy}}
{{DiseaseDisorder infobox |
+
{{Infobox disease
Name = Guillain-Barré syndrome |
+
| Name = Guillain–Barré syndrome
ICD10 = G61.0 |
+
| Image =
ICD9 = {{ICD9|357.0}} |
+
| Caption =
  +
| DiseasesDB = 5465
  +
| ICD10 = {{ICD10|G|61|0|g|60}}
  +
| ICD9 = {{ICD9|357.0}}
  +
| ICDO =
  +
| OMIM = 139393
  +
| MedlinePlus = 000684
  +
| eMedicineSubj = emerg
  +
| eMedicineTopic = 222
  +
| eMedicine_mult = {{eMedicine2|neuro|7}} {{eMedicine2|pmr|48}} {{eMedicine2|neuro|598}}
  +
| MeshID = D020275
 
}}
 
}}
   
'''Guillain-Barré syndrome''' ('''GBS'''), is an acquired [[autoimmune disorder|immune-mediated inflammatory disorder]] of the [[peripheral nervous system]] (i.e. ''not'' the [[brain]] or [[spinal cord]]). It is also called ''acute inflammatory demyelinating polyneuropathy'', ''acute idiopathic polyradiculoneuritis'', ''acute idiopathic polyneuritis'', ''French Polio'' and ''Landry's ascending paralysis''.
+
'''Guillain–Barré syndrome''' ('''GBS''') ({{IPA-fr|ɡiˈlɛ̃ baˈʁe}}, {{IPAc-en|lang|pron|ˈ|ɡ|iː|l|æ|n |ˈ|b|ɑr|eɪ}}), sometimes '''Landry's paralysis''' or '''Guillain–Barré–Strohl syndrome''', is an acute [[polyneuropathy]], a disorder affecting the [[peripheral nervous system]]. [[Ascending paralysis]], weakness beginning in the feet and hands and migrating towards the trunk, is the most typical symptom, and some subtypes cause change in sensation or pain as well as [[dysautonomia|dysfunction of the autonomic nervous system]]. It can cause life-threatening complications, in particular if the [[Muscles of respiration|respiratory muscles]] are affected or if there is [[autonomic nervous system]] involvement. The disease is usually triggered by an infection.
   
== Overview ==
+
The diagnosis is usually made by [[nerve conduction study|nerve conduction studies]] and with studies of the [[cerebrospinal fluid]]. With prompt treatment by [[intravenous immunoglobulin]]s or [[plasmapheresis]], together with supportive care, the majority will recover completely. Guillain–Barré syndrome is rare, at 1–2 cases per 100,000 people annually, but is the most common cause of acute non-trauma-related paralysis in the world. The syndrome is named after the French physicians [[Georges Guillain]] and [[Jean Alexandre Barré]], who described it in 1916.
The pathologic hallmark of the disease is loss of [[myelin]] in peripheral nerves due to an acute and progressive inflammation of unknown cause. It is suggested that it is an [[autoimmune]] disease, in which the sufferer's immune system is triggered into damaging the nerve covering. There is some support for this in that half of all cases occur soon after a microbial infection or respiratory or gastrointestinal viral infection. Many cases developed in people who received the [[1976]] swine [[flu vaccine]].
 
   
Peripheral nerves originate in the spinal cord and proceed to their target tissues (mainly muscle, skin and all internal organs). Their most proximal parts emerging from the spinal cord are called nerve roots and the inflammation in most (but not all) typical Guillain-Barré syndrome cases starts in these roots. Therefore, this condition is also referred to as ''acute polyradiculoneuritis''.
+
==Classification==
  +
Six different subtypes of Guillain–Barré syndrome exist:{{citation needed|date=January 2011}}
  +
* '''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. Accounting for approximately 5% of GBS cases, it manifests as a descending [[paralysis]], proceeding in the reverse order of the more common form of GBS.<ref>{{cite web|last=Davids|first=H|title=Guillain-Barre Syndrome|url=http://emedicine.medscape.com/article/315632|publisher=Medscape Reference|accessdate=3 Jan 2012}}</ref> It usually affects the [[human eye|eye]] muscles first and presents with the triad of [[ophthalmoplegia]], [[ataxia]], and [[areflexia]]. The ataxia predominantly affects the gait and trunk, with the limbs relatively spared. [[Anti-ganglioside antibodies#Anti-GQ1b|Anti-GQ1b]] antibodies are present in 90% of cases.
  +
* '''[[Acute motor axonal neuropathy]]''' (AMAN),<ref name="McKhann1991">{{Cite journal|author=McKhann GM, Cornblath DR, Ho T, ''et al'' |year=1991 |month= |title=Clinical and electrophysiological aspects of acute paralytic disease of children and young adults in northern China |journal=Lancet |volume=338 |issue=8767 |pages=593–7 |pmid=1679153 |url= |accessdate= |quote= |doi=10.1016/0140-6736(91)90606-P }}</ref> also known as '''Chinese paralytic syndrome''', attacks motor [[nodes of Ranvier]] and is prevalent in [[China]] and [[Mexico]]. It is probably due to an auto-immune response directed against the [[axoplasm]] of [[peripheral nervous system|peripheral nerves]]. The disease may be seasonal and recovery can be rapid. Anti-GD1a antibodies<ref name="Ho1995">{{Cite journal|author=Ho TW, Mishu B, Li CY, ''et al'' |title=Guillain-Barré syndrome in northern China. Relationship to Campylobacter jejuni infection and anti-glycolipid antibodies |journal=Brain |volume=118 |issue= 3|pages=597–605 |year=1995 |pmid=7600081 |doi= 10.1093/brain/118.3.597}}</ref> are present. [[Anti-ganglioside antibodies#Anti-GD3|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 probably due to an auto-immune response directed against the [[axoplasm]] of peripheral nerves. Recovery is slow and often incomplete.<ref name="Griffin1995">{{Cite journal|author=Griffin JW, Li CY, Ho TW, ''et al'' |title=Guillain–Barré syndrome in northern China. The spectrum of neuropathological changes in clinically defined cases |journal=Brain |volume=118 |issue= 3|pages=577–95 |year=1995 |pmid=7600080 |doi= 10.1093/brain/118.3.577}}</ref>
  +
* '''Acute panautonomic neuropathy''' is the most rare variant of GBS, sometimes accompanied by [[encephalopathy]]. It is associated with a high mortality rate, owing to cardiovascular involvement, and associated [[cardiac arrhythmia|dysrhythmias]]. Frequently occurring symptoms include impaired sweating, lack of tear formation, [[photophobia]], dryness of nasal and oral mucosa, itching and peeling of skin, [[nausea]], [[dysphagia]], and constipation unrelieved by laxatives or alternating with diarrhea. Initial [[nonspecific symptoms]] of lethargy, fatigue, headache, and decreased initiative are followed by autonomic symptoms including orthostatic lightheadedness, blurring of vision, abdominal pain, diarrhea, dryness of eyes, and disturbed [[micturition]]. The most common symptoms at onset are related to orthostatic intolerance, as well as gastrointestinal and sudomotor dysfunction (Suarez et al. 1994). [[Parasympathetic]] impairment (abdominal pain, vomiting, constipation, ileus, urinary retention, dilated unreactive pupils; loss of accommodation) may also be observed.
  +
* '''Bickerstaff's brainstem encephalitis''' (BBE) is a further variant of Guillain–Barré syndrome. It is characterized by acute onset of ophthalmoplegia, ataxia, disturbance of consciousness, hyperreflexia or [[Plantar reflex|Babinski's sign]]. The course of the disease can be monophasic or remitting-relapsing. Large, irregular hyperintense lesions located mainly in the brainstem, especially in the [[pons]], [[midbrain]] and [[Medulla oblongata|medulla]], are described in the literature. Despite severe initial presentation, BBE usually has a good prognosis. [[Magnetic resonance imaging]] (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.
   
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. The cases indicating the demyelinating form (AIDP) are called "acute motor and sensory axonal neuropathy" (AMSAN); the cases showing only motor symptoms (diffuse weakness) are called "acute motor axonal neuropathy" (AMAN). In a different and infrequent variant called [[Miller Fisher syndrome]], patients develop [[ataxia]], loss of [[tendon]] reflexes, and difficulty moving eye muscles but not weakness or sensory loss. All variants of Guillain-Barré syndrome are now supposed to be an [[autoimmune]] disease caused by [[antibody|antibodies]] against a variety of [[ganglioside]]s found in abundant amounts in the peripheral nerve tissue.
+
==Signs and symptoms==
  +
The disorder is characterized by symmetrical weakness that usually 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]] (drooling, or difficulty swallowing and/or maintaining an open airway) and respiratory difficulties. Most patients require hospitalization and about 30% require [[mechanical ventilation|ventilatory assistance]] for treatment of Type II [[respiratory failure]].<ref>{{cite journal|last=Burt|first=Christiana C.|coauthors=Arrowsmith, Joseph E.|title=Respiratory failure|journal=Surgery (Oxford)|date=1 November 2009|volume=27|issue=11|pages=475–479|doi=10.1016/j.mpsur.2009.09.007}}</ref> Facial weakness is also common. Eye movement abnormalities are not commonly seen in ascending GBS, but are a prominent feature in the Miller-Fisher variant.
   
== Prevalence ==
+
Sensory loss, if present, usually takes the form of loss of [[proprioception]] (position sense) and [[hyporeflexia|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 may be treated with standard [[analgesic]]s. [[Urinary bladder|Bladder]] dysfunction may occur in severe cases but is usually transient.
GBS is a rare disease affecting about 1 to 2 people in every 100,000 annually. It does not discriminate with regard to the age or sex of sufferers. When diagnosed in young teenagers, it generally does not recur for many years, although when it does, it often does so in the fourth or fifth decade of life, long after the patients may have forgotten the details of the original episode.
+
  +
In severe cases of GBS, loss of [[autonomic nervous system|autonomic]] function is common, manifesting as wide fluctuations in [[blood pressure]], [[orthostatic hypotension]] (a fall in blood pressure on standing, leading to an increased risk of collapse), and [[cardiac arrhythmia]]s.
  +
  +
Acute paralysis in Guillain–Barré syndrome may be related to [[sodium channel]] blocking factor in the [[cerebrospinal fluid]] (CSF). Significant issues involving intravenous salt and water administration may occur unpredictably in this patient group, resulting in [[SIADH]], a cause of low sodium levels in the blood.
   
 
==Cause==
 
==Cause==
About one half of patients have a history of preceding viral infection within two to four weeks prior to exhibiting the onset of Guillain-Barré syndrome. Guillain-Barré syndrome may also be associated with certain [[immunization]]s, recent surgery or trauma, pregnancy, [[Hodgkin's disease]], chemo-therapy, and connective tissue diseases. The most frequently associated viral agents are [[cytomegalovirus]] (CMV), [[HIV]], [[measles]] and [[herpes simplex virus]]. A bacterium called ''[[Campylobacter jejuni]]'' has recently been shown to be closely related with certain subtypes of the disease: the risk of developing Guillain-Barré syndrome following campylobacter infection is less than 2 per 10,000 in the following two months.<ref>{{cite journal | title=Incidence of Guillain-Barré Syndrome among patients with campylobacter infection: a general practice research database study | author=Tam CC, Rodrigues LC, Petersen I, ''et al.'' | journal=J Infect Dis | year=2006 | volume=194 | pages=95&ndash;7 | url=http://www.journals.uchicago.edu/JID/journal/issues/v194n1/36123/brief/36123.abstract.html }}</ref> On the other hand, about 25 to 30 percent of people diagnosed with Guillain-Barré syndrome have had a recent infection with the campylobacter bacterium.
+
{{Neuron map|Neuron}}
  +
All forms of Guillain–Barré syndrome are [[Autoimmune_disease|autoimmune diseases]], due to an immune response to foreign antigens (such as infectious agents) that is mistargeted at host nerve tissues instead, a phenomenon called [[molecular mimicry]].<ref>{{cite journal|last=CW|first=Ang|coauthors=Jacobs BC, Laman JD|title=Guillain-Barré syndrome: a true case of molecular mimicry.|journal=Trends in Immunoology|date=1|year=2004|month=February|volume=25|issue=2|pages=61–66|url=http://www.cell.com/trends/immunology/abstract/S1471-4906(03)00385-5}}</ref> The targets of such immune attack are thought to be [[gangliosides]], compounds naturally present in large quantities in human peripheral nerve tissues. The most common antecedent infection is the bacterium ''[[Campylobacter jejuni]]'',<ref name="pmid18540372">{{Cite journal| author = Yuki N | title = [Campylobacter genes responsible for the development and determinant of clinical features of Guillain-Barré syndrome] | language = Japanese | journal = Nippon Rinsho. Japanese Journal of Clinical Medicine | volume = 66 | issue = 6 | pages = 1205–10 | year = 2008 | month = June | pmid = 18540372 | doi = | url = | issn = }}</ref><ref>{{Cite journal | journal = Neurology | title = Does Campylobacter jejuni infection elicit "demyelinating" Guillain-Barré syndrome? | author = Kuwabara S. et al. | volume = 63 | issue = 3 | publisher = Lippincott Williams & Wilkins | pages = 529–33 | date = 2004-08-10 | pmid = 15304587}}</ref> followed by [[cytomegalovirus]] (CMV).<ref name="pmid21427390">{{Cite journal| author = Orlikowski D | title = Guillain–Barré Syndrome following Primary Cytomegalovirus Infection: A Prospective Cohort Study | language = | journal = Clin Infect Dis. | volume = 52| issue = 7 | pages = 837–844 | year = 2011| month = April | pmid = 21427390| doi = 10.1093/cid/cir074 | url = http://cid.oxfordjournals.org/content/52/7/837.long| issn = }}</ref> However, 60% of cases do not have a known cause. Some cases may be triggered by the [[influenza virus]], or by an immune reaction to the influenza virus.<ref>{{Cite journal | doi = 10.1086/594124 | journal = Clinical Infectious Diseases | title = Guillain-Barré syndrome and influenza virus infection | author = Sivadon-Tardy V. et al. | volume = 48 | issue = 1 | publisher = The University of Chicago Press | pages = 48–56 | issn = | date = Jan. 1 2009 | pmid = 19025491}}</ref> There was increased incidence of Guillain-Barré syndrome following influenza immunization during the 1976-1977 swine flu pandemic;<ref>{{Cite journal | doi = 10.1001/jama.292.20.2478| journal = JAMA | title = Guillain-Barré syndrome following influenza vaccination | author =Haber P et al. | volume = 292 | issue = 20 | publisher = | pages = 2478–81 | issn = | date = Nov 24, 2004 | pmid = 15562126}}</ref> however, epidemiological studies since then have demonstrated either an extremely small increased risk following immunization (under 1 additional case per million vaccinations) or no increased risk.<ref>{{Cite journal | doi = 10.1016/S1473-3099(10)70140-7| journal = Lancet Infect Dis| title = Guillain-Barré syndrome following influenza vaccination | author =Lehmann HC P et al. | volume = 10 | issue = 9| publisher = | pages = 643–51 | issn = | date = Sept 2010 | pmid = 20797646}}</ref><ref name="h1n1China">{{Cite journal| author = Liang et al | title = Safety of Influenza A (H1N1) Vaccine in Postmarketing Surveillance in China | language = | journal = New England Journal of Medicine | volume = 364| issue = | pages = 638–647 | year = 2011 | month = February | pmid = 21288090 | doi = 10.1056/NEJMoa1008553| url = http://www.nejm.org/doi/full/10.1056/NEJMoa1008553 | issn = }}</ref>
   
== Signs and symptoms ==
+
The end result of this [[autoimmune]] attack on the peripheral nerves is damage to the [[myelin]], the fatty insulating layer of the nerve, and a nerve conduction block, leading to muscle paralysis that may be accompanied by sensory or [[autonomic nervous system|autonomic]] disturbances.
Extensive damage of myelin causes disturbances in peripheral nerve functions, which can be classified as motor (affecting the muscle), sensory (affecting the skin) or autonomic (affecting the internal organs). Therefore, patients usually show two or more of the following symptoms: weakness (often symmetrical, in ascending fashion, leading to respiratory failure in one-third of cases), decreased sensation (numbness, loss of position sense), severe fluctuations in blood pressure, irregularities of heart rate, constipation and incontinence. Additional symptoms may be blurred vision, difficulty moving facial muscles, difficulty swallowing, and drooling.
 
   
The symptoms are ascending weakness with abnormal sensations and then paralysis of the legs, arms, face and possibly breathing muscles. Miller-Fisher Syndrome, however, is a descending weakness, proceeding in the reverse order of the more common form of Guillain-Barré syndrome. Guillain-Barré syndrome is rarely fatal but there is no direct cure and recovery may need care in an intensive care unit and can take years (although people can recover in a few weeks as well).
+
In mild cases, nerve [[axon]] (the long slender conducting portion of a nerve) function remains intact and recovery can be rapid if remyelination occurs. In severe cases, axonal damage occurs, and recovery depends on the regeneration of this important tissue. Approximately 80% of patients have [[myelin]] loss; in the remaining 20%, the pathological hallmark is [[axon]] loss.
   
== Diagnosis ==
+
Guillain–Barré, unlike disorders such as [[multiple sclerosis]] (MS) and [[Amyotrophic lateral sclerosis|Lou Gehrig's disease]] (ALS), is a peripheral nerve disorder and does not in general cause nerve damage to the brain or spinal cord.
The diagnosis is established by [[electromyography]] examination, [[nerve conduction study|nerve conduction studies]] (NCS), and [[cerebrospinal fluid]] (CSF) examination. Electromyography and NCS show slowing of [[Nerve conduction velocity|conduction velocities]], indicating myelin loss; CSF examination reveals high [[protein]] content with usually normal or slightly elevated cell count, indicating severe nerve damage. These findings are usually prominent after the first week of the disease, so the clinical symptoms and findings are more valuable in the early stages.
 
   
== Treatment ==
+
==Diagnosis==
Supportive care with monitoring of all vital functions is the cornerstone of successful management. 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 cmH<sub>2</sub>O, more than 30% decrease in either VC or NIF within 24 hours, rapid progression of disease, or autonomic instability.
+
The diagnosis of GBS usually depends on findings such as rapid development of muscle paralysis, areflexia, absence of fever, and a likely inciting event. Cerebrospinal fluid analysis (through a [[lumbar puncture|lumbar spinal puncture]]) and electrodiagnostic tests of nerves and muscles (such as nerve conduction studies) are common tests ordered in the diagnosis of GBS.
   
Because the immune mechanisms play a role in pathogenesis, plasma exchange or intravenous immunoglobulins over a course of treatment lasting five days may improve the outcome, preventing the need for a ventilator to be used. 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. Although [[corticosteroid]]s may be used in treatment, they are no longer considered the drug of first choice in modern practice because they may occasionally worsen symptoms.
+
===Testing===
  +
In [[cerebrospinal fluid]], characteristic findings include albumino-cytological dissociation. As opposed to infectious causes, this is an elevated protein level (100–1000 mg/dL), without an accompanying increased cell count (absence of [[pleocytosis]]). A sustained increased white blood cell count may indicate an alternative diagnosis such as infection.
   
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 the patients 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. There would also be a doctor, nurse and perhaps a speech trainer involved, depending on the needs of the patient. This team contribute with their knowledge to guide the patient towards his goal, and it is important that all goals set by the separate team members are relevant for the patient's own priorities.
+
[[Electromyography]] (EMG) and [[nerve conduction study|nerve conduction studies]] (NCS) may show prolonged distal latencies, conduction slowing, conduction block, and temporal dispersion of compound action potential in demyelinating cases. F waves and H-reflexes may be prolonged or absent. Needle EMG is frequently normal in acute cases. Reduced, neuropathic recruitment in weak muscles can be seen. Fibrillations will be seen on needle EMG if there is some axonal injury after 3 to 4 weeks. In primary axonal damage, the findings include reduced amplitude of the action potentials without conduction slowing.
   
After rehabilitation the patient should be able to function in his own home and attend necessary training as needed.
+
===Criteria===
  +
Features required for diagnosis are progressive weakness in legs and often arms and [[areflexia]] (the absence of deep tendon reflexes).<ref name="van Doorn">{{cite journal |author=van Doorn PA, Ruts L, Jacobs BC |title=Clinical features, pathogenesis, and treatment of Guillain-Barré syndrome |journal=Lancet Neurol |volume=7 |issue=10 |pages=939–50 |year=2008 |month=October |pmid=18848313 |doi=10.1016/S1474-4422(08)70215-1}}</ref> <ref name="Lerner">{{cite book | author=Lerner AJ | title=Diagnostic Criteria in Neurology (Current Clinical Neurology)| location=Totowa, NJ| publisher=Humana Press|page=186|isbn=1-61737-594-2}}</ref>
   
== Prognosis ==
+
Features that strongly support diagnosis are progression of symptoms over days to 4 weeks, relative symmetry of symptoms, mild sensory symptoms or signs, cranial nerve involvement (especially bilateral weakness of facial muscles), autonomic dysfunction, pain (often present), high concentration of protein in CSF, and typical electrodiagnostic features <ref name="van Doorn"/><ref name="Lerner"/>
Approximately 80% of patients have a complete recovery and about 5-10% recover with severe disability. 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 when a ventilator is needed in the worse cases.
 
   
== History ==
+
Features that should raise doubt about the diagnosis include severe pulmonary dysfunction with limited limb weakness at onset, severe sensory signs with limited weakness at onset, bladder or bowel dysfunction at onset, fever at onset, sharp sensory level, slow progression with limited weakness without respiratory involvement (subacute inflammatory demyelinating polyneuropathy or CIDP is more likely), marked persistent asymmetry of weakness, persistent bladder or bowel dysfunction, increased number of mononuclear cells in CSF (>50×10<sup>6</sup>/L), and polymorphonuclear cells in CSF.<ref name="van Doorn"/><ref name="Lerner"/>
The disease was first described by the French physician Jean Landry in [[1859]]. In [[1916]], Georges Guillain, Jean Alexandre Barré and Andre Strohl discovered the key diagnostic abnormality of increased spinal fluid protein production, but normal cell count.
 
   
A peer-reviewed study published in [[2003]],<ref>Goldman, AS ''et al'', [http://www.rsmpress.co.uk/jmb_2003_v11_p232-240.pdf ''What was the cause of Franklin Delano Roosevelt's paralytic illness?'']. J Med Biogr. 11: 232-240 (2003)</ref> concluded that Franklin Delano Roosevelt's 1921 paralytic illness was probably Guillain-Barré syndrome, not polio as previously assumed. The [[Bayesian analysis]] in the study found that six of eight posterior probabilities favored a diagnosis of Guillain-Barré syndrome over poliomyelitis.
+
==Treatment==
  +
Supportive care is the cornerstone of successful management in the acute patient. Of greatest concern is respiratory failure due to paralysis of the diaphragm, the muscle most important for breathing. [[Intubation]] may be needed when there is evidence of impending failure of the muscles of breathing&nbsp;– when the [[vital capacity]] (VC) is less than 20 ml/kg, the [[negative inspiratory force]] (NIF) is less negative (i.e., closer to zero) than -25 cmH<sub>2</sub>O, more than 30% decrease in either VC or NIF within 24 hours, rapid progression of disorder, or autonomic instability.
   
==See also==
+
Subsequent treatment consists of attempting to reduce the body's attack on the nervous system, either by [[plasmapheresis]], filtering antibodies out of the blood stream, or by administering [[intravenous immunoglobulins]] (IVIg), to neutralize harmful antibodies and inflammation causing disease. These two treatments are equally effective and a combination of the two is not significantly better than either alone. [[Glucocorticoids]] have not been found to be effective in GBS. <ref>Merck Manual [Online]. [http://www.merck.com/mmpe/sec16/ch223/ch223h.html#sec16-ch223-ch223h-1578 Peripheral Neuropathy, Treatment]. Retrieved 8-22-2009.</ref><ref name ="Hughes">{{cite journal |author=Hughes RA, Wijdicks EF, Barohn R, ''et al.'' |title=Practice parameter: immunotherapy for Guillain-Barré syndrome: report of the Quality Standards Subcommittee of the American Academy of Neurology |journal=Neurology |volume=61 |issue=6 |pages=736–40 |year=2003 |month=September |pmid=14504313 |doi= |url=http://www.neurology.org/content/61/6/736.full.html}}</ref> Treatment is usually begun as soon as the diagnosis is made. [[Plasmapheresis]] hastens recovery when used within 4 weeks of the onset of symptoms. <ref name=Hughes/> IVIg has equivalent efficacy to plasmapheresis when started within 2 weeks of the onset of symptoms, and has fewer complications. <ref name=Hughes/>
* [[Zimelidine]]
+
IVIg is usually used first because of its ease of administration and safety profile. 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.
   
==References==
+
Following the acute phase, treatment often consists of rehabilitation with the help of a multidisciplinary team to focus on improving [[activities of daily living]] (ADLs). Occupational therapists may offer equipment (such as wheelchair and special cutlery) to help the patient achieve ADL independence. Physiotherapists assist to correct functional movement, avoiding harmful compensations that might have a negative effect in the long run. There is also some evidence supporting [[Physical therapy|physiotherapy]] in helping patients with Guillain–Barré syndrome regain strength, endurance, and gait quality,<ref>Davidson, I., Wilson, C., Walton, T., & Brissenden, S. (2009). Physiotherapy and guillain-barre syndrome: Results of a national survey. Physiotherapy, 95(3), 157-163.{{cite doi|10.1016/j.physio.2009.04.001}}</ref> as well as helping them prevent [[contractures]], [[bedsores]], and cardiopulmonary difficulties.<ref>Karavatas, S. G. (2005). The role of neurodevelopmental sequencing in the physical therapy management of a geriatric patient with guillain-barre syndrome. Topics in Geriatric Rehabilitation, 21(2), 133-135. </ref> Speech and language therapists help regain speaking and swallowing ability, especially if the patient was intubated or received a [[tracheostomy]].
   
  +
==Prognosis==
  +
Recovery usually starts after the fourth week from the onset of the disorder. 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. Despite all improvements in treatment and supportive care, the death rate 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 four to six weeks, and in some cases up to one 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).
   
<div class="references-small">
+
Poor prognostic factors include age over 40 years, history of preceding diarrheal illness, requiring ventilator support, high anti-GM1 [[titre]], and poor upper-limb muscle strength.
<references />
 
</div>
 
   
== External links ==
+
==Epidemiology==
* [http://www.gbsfi.com/ Guillain-Barré syndrome forum and information]
 
* [http://www.gbs.org.uk Guillain-Barré Syndrome Support Group]
 
   
[[Category:Eponymous diseases]]
+
Worldwide<ref>http://www.smj.org.sa/DetailArticle.asp?ArticleId=812</ref>, the annual incidence is about 0.6–4 occurrences per 100,000 people. Men are one and a half times more likely to be affected than women. The incidence increases with age; there are approximately 1 cases per 100,000 people aged below 30 years and about 4 cases per 100,000 in those older than 75 years.<ref name="Pithadia">{{Cite journal| author = Pithadia AB, Kakadia N. | title = Guillain-Barré syndrome (GBS). | language = | journal = Pharmacol Rep | volume = 62| issue = 2 | pages = 220–32 | year = 2010 | month = March-April | pmid = 20508277 | doi = | url = http://www.if-pan.krakow.pl/pjp/pdf/2010/2_220.pdf | issn = }}</ref>
  +
The incidence of GBS during pregnancy is 1.7 cases per 100,000 of the population.<ref>{{Cite journal| doi = 10.1046/j.1365-2044.2000.01367.x| last = Brooks | first = H | coauthors = Christian AS; May AE | pmid = 10947755 | journal = Anaesthesia | year = 2000 | volume = 55 | issue = 9 | pages = 894–8 | title = Pregnancy, anaesthesia and Guillain-Barré syndrome }}</ref> Congenital and neonatal Guillain–Barré syndrome have also been reported.<ref>{{Cite book| last = Iannello | first = S | title = Guillain–Barré syndrome: Pathological, clinical and therapeutical aspects | publisher = [[Nova Publishers]] | year = 2004 | isbn = 1-59454-170-1 | url = http://books.google.com/?id=uvwX_ZGJlFoC&printsec=frontcover#v=onepage&q=&f=false}}</ref>The mean annual incidence of Guillain-Barré syndrome was 0.60 (95% confidence intervals 0.48 to 0.73) per 100,000 children under 15 years of age. There is no correlatable relationship between oral polio vaccine and GBS<ref>http://www.ncbi.nlm.nih.gov/pubmed/8301426</ref>.
  +
  +
==History==
  +
The [[France|French]] physician [[Jean Landry (physician)|Jean Landry]] first described the disorder in 1859. In 1916, [[Georges Guillain]], [[Jean Alexandre Barré]], and [[André Strohl]] diagnosed two soldiers with the illness and described the key diagnostic abnormality of increased spinal-fluid protein production, but normal cell count.<ref>{{WhoNamedIt2|synd|1766|Guillain-Barré-Strohl syndrome}} and {{WhoNamedIt|synd|1508|Miller Fisher's syndrome}}</ref>
  +
  +
GBS is also known as ''acute idiopathic polyradiculoneuritis'', ''acute idiopathic polyneuritis'', ''French polio'', ''Landry's ascending paralysis'' and ''Landry–Guillain–Barré syndrome''.
  +
  +
Canadian neurologist [[Miller Fisher|C. Miller Fisher]] described the variant that bears his name in 1956.<ref>{{cite journal | author=Fisher CM | title=An unusual variant of acute idiopathic polyneuritis (syndrome of ophthalmolplegia, ataxia and areflexia) | journal=N. Engl. J. Med. | year=1956 | volume=255 | issue=2 | pages=57–65 | pmid=13334797 | doi=10.1056/NEJM195607122550201}}</ref>
  +
  +
  +
  +
==References==
  +
{{Reflist|2}}
  +
  +
==External links==
  +
* {{DMOZ|Health/Conditions_and_Diseases/Neurological_Disorders/Demyelinating_Diseases/Guillain-Barre_Syndrome/}}
  +
  +
{{PNS diseases of the nervous system}}
  +
{{Autoimmune diseases}}
  +
  +
{{DEFAULTSORT:Guillain-Barre Syndrome}}
 
[[Category:Autoimmune diseases]]
 
[[Category:Autoimmune diseases]]
[[Category:Neurology]]
+
[[Category:Syndromes]]
  +
[[Category:Neurological disorders]]
   
 
<!--
 
<!--
[[de:Polyradikulitis]]
+
[[ar:متلازمة غيلان باريه]]
  +
[[az:Guillain Barre sindromu]]
  +
[[bg:Синдром на Гилен-Баре]]
  +
[[ca:Síndrome de Guillain-Barré]]
  +
[[cs:Guillain-Barrého syndrom]]
  +
[[da:Guillain-Barrés syndrom]]
  +
[[de:Guillain-Barré-Syndrom]]
 
[[es:Síndrome de Guillain-Barré]]
 
[[es:Síndrome de Guillain-Barré]]
  +
[[fa:نشانگان گیلن باره]]
 
[[fr:Syndrome de Guillain-Barré]]
 
[[fr:Syndrome de Guillain-Barré]]
  +
[[ko:길랭-바레 증후군]]
  +
[[hr:Guillain-Barréov sindrom]]
  +
[[id:Sindrom Guillain–Barré]]
  +
[[ia:Syndrome de Guillain-Barré]]
  +
[[it:Sindrome di Guillain-Barré]]
  +
[[he:תסמונת גיאן-בארה]]
 
[[lb:Guillain-Barré-Syndrom]]
 
[[lb:Guillain-Barré-Syndrom]]
 
[[ms:Sindrom Guillain-Barré]]
 
[[ms:Sindrom Guillain-Barré]]
 
[[nl:Syndroom van Guillain-Barré]]
 
[[nl:Syndroom van Guillain-Barré]]
 
[[ja:ギラン・バレー症候群]]
 
[[ja:ギラン・バレー症候群]]
[[pl:Zespół Guillaina-Bárrego]]
+
[[pms:Sìndrom ëd Guillain-Barré]]
  +
[[pl:Zespół Guillaina-Barrégo]]
 
[[pt:Síndrome de Guillain-Barré]]
 
[[pt:Síndrome de Guillain-Barré]]
  +
[[ru:Синдром Гийена — Барре]]
  +
[[sl:Guillain-Barréjev sindrom]]
  +
[[sr:Гијен-Бареов синдром]]
  +
[[sh:Guillain-Barréov sindrom]]
  +
[[fi:Guillain-Barrén oireyhtymä]]
  +
[[sv:Guillain-Barrés syndrom]]
  +
[[th:กลุ่มอาการกิลแลง-บาร์เร]]
 
[[tr:Guillain-Barré sendromu]]
 
[[tr:Guillain-Barré sendromu]]
[[zh:格林-巴利综合征]]
+
[[uk:Синдром Гієна-Барре]]
  +
[[zh-yue:吉巴氏綜合症]]
  +
[[zh:格林-巴利综合征]]
  +
 
-->
 
-->
 
{{enWP|Guillain-Barré syndrome}}
 
{{enWP|Guillain-Barré syndrome}}

Latest revision as of 13:19, October 3, 2012

Assessment | Biopsychology | Comparative | Cognitive | Developmental | Language | Individual differences | Personality | Philosophy | Social |
Methods | Statistics | Clinical | Educational | Industrial | Professional items | World psychology |

Clinical: Approaches · Group therapy · Techniques · Types of problem · Areas of specialism · Taxonomies · Therapeutic issues · Modes of delivery · Model translation project · Personal experiences ·


Guillain–Barré syndrome
Classification and external resources
ICD-10 G610
ICD-9 357.0
OMIM 139393
DiseasesDB 5465
MedlinePlus 000684
eMedicine emerg/222 neuro/7 pmr/48 neuro/598
MeSH D020275

Guillain–Barré syndrome (GBS) (Template:IPA-fr, English pronunciation: /ˈɡlænˈbɑr/), sometimes Landry's paralysis or Guillain–Barré–Strohl syndrome, is an acute polyneuropathy, a disorder affecting the peripheral nervous system. Ascending paralysis, weakness beginning in the feet and hands and migrating towards the trunk, is the most typical symptom, and some subtypes cause change in sensation or pain as well as dysfunction of the autonomic nervous system. It can cause life-threatening complications, in particular if the respiratory muscles are affected or if there is autonomic nervous system involvement. The disease is usually triggered by an infection.

The diagnosis is usually made by nerve conduction studies and with studies of the cerebrospinal fluid. With prompt treatment by intravenous immunoglobulins or plasmapheresis, together with supportive care, the majority will recover completely. Guillain–Barré syndrome is rare, at 1–2 cases per 100,000 people annually, but is the most common cause of acute non-trauma-related paralysis in the world. The syndrome is named after the French physicians Georges Guillain and Jean Alexandre Barré, who described it in 1916.

ClassificationEdit

Six different subtypes of Guillain–Barré syndrome exist:[citation needed]

  • 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. Accounting for approximately 5% of GBS cases, it manifests as a descending paralysis, proceeding in the reverse order of the more common form of GBS.[1] It usually affects the eye muscles first and presents with the triad of ophthalmoplegia, ataxia, and areflexia. The ataxia predominantly affects the gait and trunk, with the limbs relatively spared. Anti-GQ1b antibodies are present in 90% of cases.
  • Acute motor axonal neuropathy (AMAN),[2] also known as Chinese paralytic syndrome, attacks motor nodes of Ranvier and is prevalent in China and Mexico. It is probably 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[3] 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 probably due to an auto-immune response directed against the axoplasm of peripheral nerves. Recovery is slow and often incomplete.[4]
  • Acute panautonomic neuropathy is the most rare variant of GBS, sometimes accompanied by encephalopathy. It is associated with a high mortality rate, owing to cardiovascular involvement, and associated dysrhythmias. Frequently occurring symptoms include impaired sweating, lack of tear formation, photophobia, dryness of nasal and oral mucosa, itching and peeling of skin, nausea, dysphagia, and constipation unrelieved by laxatives or alternating with diarrhea. Initial nonspecific symptoms of lethargy, fatigue, headache, and decreased initiative are followed by autonomic symptoms including orthostatic lightheadedness, blurring of vision, abdominal pain, diarrhea, dryness of eyes, and disturbed micturition. The most common symptoms at onset are related to orthostatic intolerance, as well as gastrointestinal and sudomotor dysfunction (Suarez et al. 1994). Parasympathetic impairment (abdominal pain, vomiting, constipation, ileus, urinary retention, dilated unreactive pupils; loss of accommodation) may also be observed.
  • Bickerstaff's brainstem encephalitis (BBE) is a further variant of Guillain–Barré syndrome. It is characterized by acute onset of ophthalmoplegia, ataxia, disturbance of consciousness, hyperreflexia or Babinski's sign. The course of the disease can be monophasic or remitting-relapsing. Large, irregular hyperintense lesions located mainly in the brainstem, especially in the pons, midbrain and medulla, are described in the literature. Despite severe initial presentation, BBE usually has a good prognosis. Magnetic resonance imaging (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.

Signs and symptomsEdit

The disorder is characterized by symmetrical weakness that usually 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 (drooling, or difficulty swallowing and/or maintaining an open airway) and respiratory difficulties. Most patients require hospitalization and about 30% require ventilatory assistance for treatment of Type II respiratory failure.[5] Facial weakness is also common. Eye movement abnormalities are not commonly seen in ascending GBS, but are a prominent feature in the Miller-Fisher variant.

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 may be treated with standard analgesics. Bladder dysfunction may occur in severe cases but is usually transient.

In severe cases of GBS, loss of autonomic function is common, manifesting as wide fluctuations in blood pressure, orthostatic hypotension (a fall in blood pressure on standing, leading to an increased risk of collapse), and cardiac arrhythmias.

Acute paralysis in Guillain–Barré syndrome may be related to sodium channel blocking factor in the cerebrospinal fluid (CSF). Significant issues involving intravenous salt and water administration may occur unpredictably in this patient group, resulting in SIADH, a cause of low sodium levels in the blood.

CauseEdit

Neuron
Structure of a typical neuron

All forms of Guillain–Barré syndrome are autoimmune diseases, due to an immune response to foreign antigens (such as infectious agents) that is mistargeted at host nerve tissues instead, a phenomenon called molecular mimicry.[6] The targets of such immune attack are thought to be gangliosides, compounds naturally present in large quantities in human peripheral nerve tissues. The most common antecedent infection is the bacterium Campylobacter jejuni,[7][8] followed by cytomegalovirus (CMV).[9] However, 60% of cases do not have a known cause. Some cases may be triggered by the influenza virus, or by an immune reaction to the influenza virus.[10] There was increased incidence of Guillain-Barré syndrome following influenza immunization during the 1976-1977 swine flu pandemic;[11] however, epidemiological studies since then have demonstrated either an extremely small increased risk following immunization (under 1 additional case per million vaccinations) or no increased risk.[12][13]

The end result of this autoimmune attack on the peripheral nerves is damage to the myelin, the fatty insulating layer of the nerve, and a nerve conduction block, leading to muscle paralysis that may be accompanied by sensory or autonomic disturbances.

In mild cases, nerve axon (the long slender conducting portion of a nerve) function remains intact and recovery can be rapid if remyelination occurs. In severe cases, axonal damage occurs, and recovery depends on the regeneration of this important tissue. Approximately 80% of patients have myelin loss; in the remaining 20%, the pathological hallmark is axon loss.

Guillain–Barré, unlike disorders such as multiple sclerosis (MS) and Lou Gehrig's disease (ALS), is a peripheral nerve disorder and does not in general cause nerve damage to the brain or spinal cord.

DiagnosisEdit

The diagnosis of GBS usually depends on findings such as rapid development of muscle paralysis, areflexia, absence of fever, and a likely inciting event. Cerebrospinal fluid analysis (through a lumbar spinal puncture) and electrodiagnostic tests of nerves and muscles (such as nerve conduction studies) are common tests ordered in the diagnosis of GBS.

TestingEdit

In cerebrospinal fluid, characteristic findings include albumino-cytological dissociation. As opposed to infectious causes, this is an elevated protein level (100–1000 mg/dL), without an accompanying increased cell count (absence of pleocytosis). A sustained increased white blood cell count may indicate an alternative diagnosis such as infection.

Electromyography (EMG) and nerve conduction studies (NCS) may show prolonged distal latencies, conduction slowing, conduction block, and temporal dispersion of compound action potential in demyelinating cases. F waves and H-reflexes may be prolonged or absent. Needle EMG is frequently normal in acute cases. Reduced, neuropathic recruitment in weak muscles can be seen. Fibrillations will be seen on needle EMG if there is some axonal injury after 3 to 4 weeks. In primary axonal damage, the findings include reduced amplitude of the action potentials without conduction slowing.

CriteriaEdit

Features required for diagnosis are progressive weakness in legs and often arms and areflexia (the absence of deep tendon reflexes).[14] [15]

Features that strongly support diagnosis are progression of symptoms over days to 4 weeks, relative symmetry of symptoms, mild sensory symptoms or signs, cranial nerve involvement (especially bilateral weakness of facial muscles), autonomic dysfunction, pain (often present), high concentration of protein in CSF, and typical electrodiagnostic features [14][15]

Features that should raise doubt about the diagnosis include severe pulmonary dysfunction with limited limb weakness at onset, severe sensory signs with limited weakness at onset, bladder or bowel dysfunction at onset, fever at onset, sharp sensory level, slow progression with limited weakness without respiratory involvement (subacute inflammatory demyelinating polyneuropathy or CIDP is more likely), marked persistent asymmetry of weakness, persistent bladder or bowel dysfunction, increased number of mononuclear cells in CSF (>50×106/L), and polymorphonuclear cells in CSF.[14][15]

TreatmentEdit

Supportive care is the cornerstone of successful management in the acute patient. Of greatest concern is respiratory failure due to paralysis of the diaphragm, the muscle most important for breathing. Intubation may be needed when there is evidence of impending failure of the muscles of breathing – when the vital capacity (VC) is less than 20 ml/kg, the negative inspiratory force (NIF) is less negative (i.e., closer to zero) than -25 cmH2O, more than 30% decrease in either VC or NIF within 24 hours, rapid progression of disorder, or autonomic instability.

Subsequent treatment consists of attempting to reduce the body's attack on the nervous system, either by plasmapheresis, filtering antibodies out of the blood stream, or by administering intravenous immunoglobulins (IVIg), to neutralize harmful antibodies and inflammation causing disease. These two treatments are equally effective and a combination of the two is not significantly better than either alone. Glucocorticoids have not been found to be effective in GBS. [16][17] Treatment is usually begun as soon as the diagnosis is made. Plasmapheresis hastens recovery when used within 4 weeks of the onset of symptoms. [17] IVIg has equivalent efficacy to plasmapheresis when started within 2 weeks of the onset of symptoms, and has fewer complications. [17] IVIg is usually used first because of its ease of administration and safety profile. 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.

Following the acute phase, treatment often consists of rehabilitation with the help of a multidisciplinary team to focus on improving activities of daily living (ADLs). Occupational therapists may offer equipment (such as wheelchair and special cutlery) to help the patient achieve ADL independence. Physiotherapists assist to correct functional movement, avoiding harmful compensations that might have a negative effect in the long run. There is also some evidence supporting physiotherapy in helping patients with Guillain–Barré syndrome regain strength, endurance, and gait quality,[18] as well as helping them prevent contractures, bedsores, and cardiopulmonary difficulties.[19] Speech and language therapists help regain speaking and swallowing ability, especially if the patient was intubated or received a tracheostomy.

PrognosisEdit

Recovery usually starts after the fourth week from the onset of the disorder. 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. Despite all improvements in treatment and supportive care, the death rate 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 four to six weeks, and in some cases up to one 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).

Poor prognostic factors include age over 40 years, history of preceding diarrheal illness, requiring ventilator support, high anti-GM1 titre, and poor upper-limb muscle strength.

EpidemiologyEdit

Worldwide[20], the annual incidence is about 0.6–4 occurrences per 100,000 people. Men are one and a half times more likely to be affected than women. The incidence increases with age; there are approximately 1 cases per 100,000 people aged below 30 years and about 4 cases per 100,000 in those older than 75 years.[21] The incidence of GBS during pregnancy is 1.7 cases per 100,000 of the population.[22] Congenital and neonatal Guillain–Barré syndrome have also been reported.[23]The mean annual incidence of Guillain-Barré syndrome was 0.60 (95% confidence intervals 0.48 to 0.73) per 100,000 children under 15 years of age. There is no correlatable relationship between oral polio vaccine and GBS[24].

HistoryEdit

The French physician Jean Landry first described the disorder in 1859. In 1916, Georges Guillain, Jean Alexandre Barré, and André Strohl diagnosed two soldiers with the illness and described the key diagnostic abnormality of increased spinal-fluid protein production, but normal cell count.[25]

GBS is also known as acute idiopathic polyradiculoneuritis, acute idiopathic polyneuritis, French polio, Landry's ascending paralysis and Landry–Guillain–Barré syndrome.

Canadian neurologist C. Miller Fisher described the variant that bears his name in 1956.[26]


ReferencesEdit

  1. Davids, H Guillain-Barre Syndrome. Medscape Reference. URL accessed on 3 Jan 2012.
  2. McKhann GM, Cornblath DR, Ho T, et al (1991). Clinical and electrophysiological aspects of acute paralytic disease of children and young adults in northern China. Lancet 338 (8767): 593–7.
  3. Ho TW, Mishu B, Li CY, et al (1995). Guillain-Barré syndrome in northern China. Relationship to Campylobacter jejuni infection and anti-glycolipid antibodies. Brain 118 (3): 597–605.
  4. Griffin JW, Li CY, Ho TW, et al (1995). Guillain–Barré syndrome in northern China. The spectrum of neuropathological changes in clinically defined cases. Brain 118 (3): 577–95.
  5. Burt, Christiana C., Arrowsmith, Joseph E. (1 November 2009). Respiratory failure. Surgery (Oxford) 27 (11): 475–479.
  6. CW, Ang, Jacobs BC, Laman JD (1). Guillain-Barré syndrome: a true case of molecular mimicry.. Trends in Immunoology 25 (2): 61–66.
  7. Yuki N (June 2008). [Campylobacter genes responsible for the development and determinant of clinical features of Guillain-Barré syndrome]. Nippon Rinsho. Japanese Journal of Clinical Medicine 66 (6): 1205–10.
  8. Kuwabara S. et al. (2004-08-10). Does Campylobacter jejuni infection elicit "demyelinating" Guillain-Barré syndrome?. Neurology 63 (3): 529–33.
  9. Orlikowski D (April 2011). Guillain–Barré Syndrome following Primary Cytomegalovirus Infection: A Prospective Cohort Study. Clin Infect Dis. 52 (7): 837–844.
  10. Sivadon-Tardy V. et al. (Jan. 1 2009). Guillain-Barré syndrome and influenza virus infection. Clinical Infectious Diseases 48 (1): 48–56.
  11. Haber P et al. (Nov 24, 2004). Guillain-Barré syndrome following influenza vaccination. JAMA 292 (20): 2478–81.
  12. Lehmann HC P et al. (Sept 2010). Guillain-Barré syndrome following influenza vaccination. Lancet Infect Dis 10 (9): 643–51.
  13. Liang et al (February 2011). Safety of Influenza A (H1N1) Vaccine in Postmarketing Surveillance in China. New England Journal of Medicine 364: 638–647.
  14. 14.0 14.1 14.2 van Doorn PA, Ruts L, Jacobs BC (October 2008). Clinical features, pathogenesis, and treatment of Guillain-Barré syndrome. Lancet Neurol 7 (10): 939–50.
  15. 15.0 15.1 15.2 Lerner AJ. Diagnostic Criteria in Neurology (Current Clinical Neurology), Totowa, NJ: Humana Press.
  16. Merck Manual [Online]. Peripheral Neuropathy, Treatment. Retrieved 8-22-2009.
  17. 17.0 17.1 17.2 Hughes RA, Wijdicks EF, Barohn R, et al. (September 2003). Practice parameter: immunotherapy for Guillain-Barré syndrome: report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology 61 (6): 736–40.
  18. Davidson, I., Wilson, C., Walton, T., & Brissenden, S. (2009). Physiotherapy and guillain-barre syndrome: Results of a national survey. Physiotherapy, 95(3), 157-163.DOI:10.1016/j.physio.2009.04.001
    This citation will be automatically completed in the next few minutes. You can jump the queue or expand by hand
  19. Karavatas, S. G. (2005). The role of neurodevelopmental sequencing in the physical therapy management of a geriatric patient with guillain-barre syndrome. Topics in Geriatric Rehabilitation, 21(2), 133-135.
  20. http://www.smj.org.sa/DetailArticle.asp?ArticleId=812
  21. Pithadia AB, Kakadia N. (March-April 2010). Guillain-Barré syndrome (GBS).. Pharmacol Rep 62 (2): 220–32.
  22. Brooks, H, Christian AS; May AE (2000). Pregnancy, anaesthesia and Guillain-Barré syndrome. Anaesthesia 55 (9): 894–8.
  23. Iannello, S (2004). Guillain–Barré syndrome: Pathological, clinical and therapeutical aspects, Nova Publishers.
  24. http://www.ncbi.nlm.nih.gov/pubmed/8301426
  25. Template:WhoNamedIt2 and Who Named It synd/1508
  26. Fisher CM (1956). An unusual variant of acute idiopathic polyneuritis (syndrome of ophthalmolplegia, ataxia and areflexia). N. Engl. J. Med. 255 (2): 57–65.

External linksEdit


Template:Autoimmune diseases

This page uses Creative Commons Licensed content from Wikipedia (view authors).

Around Wikia's network

Random Wiki