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Guillain-Barré Syndrome

GBS

Guillain–Barré syndrome (GBS) is a rare postinfectious, immune-mediated disorder affecting the nervous system.

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Presentation

Guillain–Barré syndrome is a one-time episode in more than 95% of cases [1].

The generally accepted criteria for the diagnosis of Guillain–Barré syndrome include: progressive, ascending weakness of more than two extremities, areflexia and numbness and tingling of fingers and toes [2] [4] [6]. Other associated symptoms include mild sensory loss and elevated cerebrospinal fluid (CSF) protein with normal cell count [4]. Impairment of respiratory muscles happens in approximately 25% of patients and requires respiratory assist [7].

Symptoms of Guillain-Barré syndrome include [5]:

Neuro-muscular involvement

Autonomic system involvement 

The course of the disease is similar in most individuals with Guillain–Barré syndrome [3]. In 75% of cases there is a history of a preceding viral or bacterial infection. This is followed, within several weeks, by mild sensory symptoms such as numbness or tingling of hands and muscle weakness. The disorder progresses rapidly from this point, with profound neuro-muscular impairment, [4] respiratory dysfunction, and autonomic nervous system involvement.

The length of the acute stage may vary widely from 2 weeks to 6 or more months. Studies have shown that 98% of patients reached the peak of the disease by 4 weeks. The acute stage is followed by a plateau stage that lasts 1 to 2 weeks [5].

After this plateau phase, recovery occurs gradually but is often incomplete. Many patients still have symptoms for years after the disease. Some 12% report persistent fatigue ; 52% still have difficulty walking one year after onset; and 62% report continuing changes in their daily living as a result [6].

The mortality rate continues to be approximately 5%, despite modern medical care and intensive care. Tracheostomy and respiratory support is necessary in 10-30% of the patients. In addition, 3-10% of individuals with Guillain–Barré syndrome have relapses, and up to 20% have permanent disability [6].

Weakness
  • But weakness that increases over several days is also common. Muscle weakness or loss of muscle function (paralysis) affects both sides of the body. In most cases, the muscle weakness starts in the legs and spreads to the arms.[nlm.nih.gov]
  • Facial weakness, dysphasia or dysarthria may develop. In severe cases, muscle weakness may lead to respiratory failure. Pain : neuropathic pain may develop, particularly in the legs. Back pain may be another feature.[patient.info]
Fever
  • Until recent now, GBS was only reported in hemorrhagic fever patients in Europe and Asia, which termed as hemorrhagic fever with renal syndrome.[ncbi.nlm.nih.gov]
Limb Pain
  • A prodromal malaise with vomiting, headache, fever and limb pains is rapidly surmounted by a progressive and ascending paralysis. This can lead to respiratory dysfunction, and as such, the acute presentation can be a neurological emergency.[gpnotebook.co.uk]
  • , pain in both shoulders, subicterus, dark urine and levated serum values (total bilirubin (37 mol/L; range 0–20), LT (32.09 kat/L; range 0–0.8), AST (8.54 kat/L; range 0–0.65)). is cognition was normal, meningeal phenomena negative, and ranial nerves[scinapse.io]
  • Brunet, Upper limb pain in chronic demyelinating polyneuropathy: electrophysiological correlates, Acta Neurologica Scandinavica, 90, 4, (270-275), (2009). Ernst F. Hund, Hans-Peter Hartung, Allan H. Ropper and Daniel F.[oadoi.org]
  • An annual incidence of 0.5---2 cases per 100,000 individuals has been reported in the age range Clinical onset is characterized by limb pain, limb weakness, and in a few cases ataxia.[kundoc.com]
Acute Intermittent Porphyria
  • In South Africa (SA), a high incidence of variegate porphyria (VP) is seen as a result of a founder effect, but acute intermittent porphyria (AIP) is also encountered.[ncbi.nlm.nih.gov]
  • intermittent porphyria Functional paralysisMFS, BBE, and pharyngealcervicalbrachial weakness Brainstem stroke Myasthenia gravis Wernicke encephalopathy Botulism Brainstem encephalitis Diphtheria Tick paralysisParaparetic GBS Lumbosacral plexopathy Diabetic[vdocuments.net]
  • . · · · · · · · · Vasculitic neuropathy Diphtheric neuropathy Acute intermittent porphyria Critical illness neuropathy Lymphomatous neuropathy Heavy metal intoxication Post-rabies vaccine neuropathy Diabetic–uremic neuropathy with acute peritoneal dialysis[zdoc.site]
Recent Viral Illness
  • Ask about recent viral illnesses or immunizations. Perform thorough neurologic exam, including reflexes. Search for subtle weakness – make the child stand up from seated position without using hands.[pedemmorsels.com]
Dyspnea
  • Two patients with cerebral hemorrhagic stroke developed progressive flaccid quadriplegia and life-threatening dyspnea in acute stage. Combined with the cerebrospinal fluid and electromyogram results, they were diagnosed as having acute GBS.[ncbi.nlm.nih.gov]
  • A 13-year-old boy presented with acute dysphagia and dyspnea. He lived in a rural area and had a history of drinking potable deep-hole water. The patient was intubated because of increased respiratory distress.[scinapse.io]
  • Tachypnea may be a sign of ongoing dyspnea and progressive respiratory failure. Blood pressure lability is another common feature, with alterations between hypertension and hypotension. Temperature may be elevated or low.[emedicine.medscape.com]
Hoarseness
  • She did not have hoarseness butshe had mild dysarthria and experienced mild difficulty inswallowing.[docslide.us]
Vomiting
  • A 46-year man with type 2 diabetes and otitis media (OM) suffered with fever, headache, and vomiting for 6 days. The patient's neck stiffness was obvious and the Kernig and Brudzinski signs were produced.[ncbi.nlm.nih.gov]
  • R nausea, vomiting, and diarrhea 3 days ago (admits to eating discount sushi). Exam is remarkable for symetric 3/5 lower and upper extremity weakness, absent ankle and patellar reflexes and 1 biceps reflex.[step2.medbullets.com]
  • A prodromal malaise with vomiting, headache, fever and limb pains is rapidly surmounted by a progressive and ascending paralysis. This can lead to respiratory dysfunction, and as such, the acute presentation can be a neurological emergency.[gpnotebook.co.uk]
  • […] chills, vomiting, diarrhea, h/a, numbness in fingers & toes;28JUN vision was blurred & legs were weak;became paralyzed;dx GBS;no coordination[whale.to]
  • […] and signs are common usually milder than motor 15% of GBS patients have no sensory symptoms (pure motor) muscular or radicular pain commonly back pain, but not always may precede weakness in 1/3 cases Other features autonomic dysfunction diarrhoea, vomiting[lifeinthefastlane.com]
Nausea
  • The most common adverse effects of pazopanib include diarrhea, fatigue, and nausea, but neuropathic complication has not been documented.[ncbi.nlm.nih.gov]
  • R nausea, vomiting, and diarrhea 3 days ago (admits to eating discount sushi). Exam is remarkable for symetric 3/5 lower and upper extremity weakness, absent ankle and patellar reflexes and 1 biceps reflex.[step2.medbullets.com]
  • Infection with the bacteria Campylobacter jejuni, which causes gastroenteritis (including symptoms of nausea, vomiting and diarrhea), is one of the most common risk factors for GBS.[cdc.gov]
Abdominal Pain
  • Absence of abdominal pain does not exclude the possibility of porphyria, and attacks may be precipitated by antiretroviral and antituberculosis medication.[ncbi.nlm.nih.gov]
  • pain, ileus, orthostatic hypotension, urinary retention, bilateral tonic pupils, fluctuating heart rate and dysrhythmias, decreased sweating, salivation and lacrimation respiratory failure (affects 20-30% of cases) corneal ulceration (poor lid closure[lifeinthefastlane.com]
  • Differential diagnosis Acute myelopathy – back pain,sphincter disturbances Botulism –early loss of pupillaryactivity,descending paralysis Diphtheria –early oropharyngeal involvement Lyme disease polyradiculitis Porphyria –abdominal pain,seizure,psychosis[slideshare.net]
  • Most commonly reported ADRs include headaches, back or abdominal pain, nausea/vomiting, rhinitis, asthma, fever, chills and myalgias.[doi.org]
Fecal Incontinence
  • Even more confusing and mimicking a spinal cord lesion are the 5% of cases that experience bladder (urinary retention) and gastrointestinal (constipation, ileus, gastric distension, diarrhea, fecal incontinence) dysfunction.[ncbi.nlm.nih.gov]
Hypertension
  • Diabetes requiring insulin was significantly more common and hypertension less common with corticosteroids.[ncbi.nlm.nih.gov]
  • Development of hypertension requiring drug treatment.[doi.org]
Hypotension
  • He subsequently developed severe bradycardia and refractory hypotension, which initially responded to dopamine infusion. A temporary pacemaker wire was placed to stabilize the heart rate but hypotension persisted.[ncbi.nlm.nih.gov]
  • Complications of plasma exchange include the possibility of hypotension, abnormal clotting, and septicaemia. Intravenous immunoglobulin is believed to suppress autoantibodies. It has been shown to be a safe form of treatment.[symptoma.com]
  • GBS patients have no sensory symptoms (pure motor) muscular or radicular pain commonly back pain, but not always may precede weakness in 1/3 cases Other features autonomic dysfunction diarrhoea, vomiting, dizziness, abdominal pain, ileus, orthostatic hypotension[lifeinthefastlane.com]
Orthostatic Hypotension
  • She subsequently developed pandysautonomia that manifested as a tonically dilated pupil, gastrointestinal dysmotility, urinary retention, and profound orthostatic hypotension. Guillain-Barré syndrome was diagnosed on electromyography.[ncbi.nlm.nih.gov]
  • hypotension, urinary retention, bilateral tonic pupils, fluctuating heart rate and dysrhythmias, decreased sweating, salivation and lacrimation respiratory failure (affects 20-30% of cases) corneal ulceration (poor lid closure) CLASSIFICATION There are[lifeinthefastlane.com]
  • Sheps, Gastrointestinal motility disturbances in patients with orthostatic hypotension, Gastroenterology, 88, 6, (1852), (1985). Juan-R.[dx.doi.org]
  • Other features: ileus, urinary retention (1/4 cases), inappropriate ADH, altered sweating, mild orthostatic hypotension. f.Cranial nerve involvement is observed in 45-75% of patients with GBS. Cranial nerves III-VII and IX-XII may be affected.[physio-pedia.com]
Diplopia
  • CASE REPORT: A 76-year-old woman was initially presented with diplopia, ophthalmoplegia, and ataxia, but she later developed weakness of limbs, respiratory failure, deterioration of consciousness, and cognitive impairment.[ncbi.nlm.nih.gov]
  • His chief complaints at presentation were persistent diplopia of approximately 7 days' duration and a “wobbly” gait.[jaoa.org]
  • Diplopia was the main presenting symptom whereas ataxia was in two patients. Total external ophthalmoplegia was seen in three patients and partial in the rest. Half of the patient couldnt walk independently.[vdocuments.us]
  • Then, 12-15 days later, recovery typically begins. [11] Symptoms The most common symptoms in MFS are diplopia (65%), gait disturbance (32%), and dysesthesias (14%).[eyewiki.org]
Blepharoptosis
  • RESULTS: A 41-year-old man presented with aggressive speech difficulty, dysphagia, right blepharoptosis, and quadriplegia following coma. A diagnosis of glossopharyngeal, vagus, oculomotor, and peripheral nerve damage was made.[ncbi.nlm.nih.gov]
  • Pharyngeal Cervical Brachial Variant (PCB) It characteristically involves cervical, brachial and oropharyngeal muscles, hyporeflexia or areflexia in upper limbs and sometimes facial palsy, blepharoptosis, sensory disturbance, and preserved tendon jerk[vdocuments.us]
  • Less common symptoms include consciousness disturbance, blepharoptosis, limb weakness, bulbar dysfunction, dysphagia, photophobia, dizziness, blurry vision, headache, and facial weakness. [2] Signs MFS classically presents with three signs: ophthalmoplegia[eyewiki.org]
Muscle Weakness
  • Muscle weakness or loss of muscle function (paralysis) affects both sides of the body. In most cases, the muscle weakness starts in the legs and spreads to the arms. This is called ascending paralysis.[nlm.nih.gov]
  • We describe a 24-year-old previously healthy male presenting with behavioral symptoms including depression, anxiety, and amnesia, and autonomic dysfunction which preceded muscle weakness by two weeks.[ncbi.nlm.nih.gov]
  • This is followed, within several weeks, by mild sensory symptoms such as numbness or tingling of hands and muscle weakness.[symptoma.com]
Back Pain
  • After 6 months, she came back with acute onset of weakness in lower limbs, back pain, fever and urinary incontinence. Pinprick and light touch complete sensory loss was found beneath umbilicus.[ncbi.nlm.nih.gov]
  • GBS symptoms may include: lower limb numbness and tingling, symmetrical leg and arm weakness, severe back pain, muscle aching and cramping, shortness of breath and bieralfacial drooping (palsy).[muscle.ca]
  • Back pain may be another feature. Reflexes : these may be reduced or absent. Sensory symptoms : these can include paraesthesiae and sensory loss, starting in the lower extremities.[patient.info]
Myalgia
  • Suspected Zika virus disease was defined as a history of rash and 2 other Zika-related symptoms (fever, arthralgia, myalgia, or conjunctivitis).[ncbi.nlm.nih.gov]
  • Distal paresthesias 54% of cases [ Hicks, 2010 ] Neuropathic pain affects children prominently Myalgias 49% of cases [ Hicks, 2010 ] Hyoreflexia or areflexia Present in 94% of cases [ Hicks, 2010 ] Always check reflexes!![pedemmorsels.com]
  • In these patients, clinical manifestations were fever (96.6%), rash (93.3%), non-purulent conjunctivitis (88.8%), headache (85.4%), and myalgia (84.3%). No neurological manifestations were found among them.[frontiersin.org]
  • These were rigor in four, fever in two, and flu‐like symptoms, malaise with nausea and myalgia, hypotension and chest pain in one each. In the PE group, complications were attributed to PE in 8 of 121 participants.[doi.org]
Proximal Muscle Weakness
Muscular Atrophy
  • RESULTS: All six patients had different degrees of muscular atrophy at nadir and in two, respiratory muscles were involved. Five also had damaged cranial nerves and four of these had serum antibodies against gangliosides.[ncbi.nlm.nih.gov]
  • The peroneal muscular atrophy syndrome 8. The ataxic neuropathies 9. Sensory neuropathies 10. Neuropathies in metabolic and degenerative disorders 11.[scinapse.io]
  • The CSF findings, in combination with certain clinical features, allowed AIDP to be distinguished from anterior horn cell diseases such as poliomyelitis, spinal muscular atrophy and from other neuropathies.[emedicine.medscape.com]
  • Sarit Ravid, Leon Topper and Lydia Eviatar, Acute onset of infantile spinal muscular atrophy, Pediatric Neurology, 24, 5, (371), (2001). E. Sindern, J.M. Schröder, M. Krismann and J.P.[doi.org]
Guillain-Barré Syndrome
  • IgG Fc N-glycosylation in GuillainBarré syndrome treated with immunoglobulins. J. Proteome Res. 13, 1722–1730 (2014). 113. [No authors listed] Plasmapheresis and acute GuillainBarré syndrome. The GuillainBarré syndrome Study Group.[nature.com]
Flaccid Paralysis
  • Guillain-Barré syndrome (GBS) is the most common cause of acute flaccid paralysis in children.[ncbi.nlm.nih.gov]
Peripheral Neuropathy
  • Peripheral neuropathy in SLE should be given greater recognition, and rarer forms of presentation should be taken seriously in the differential diagnosis when the clinical picture is atypical.[ncbi.nlm.nih.gov]
  • Schmidt, The Inflammatory Demyelinating Neuropathies, Biopsy Diagnosis of Peripheral Neuropathy, 10.1007/978-3-319-07311-8_9, (161-196), (2014).[oadoi.org]
Paresthesia
  • CASE REPORT A 54-year-old man presented with lower left facial palsy and paresthesia of his extremities, following an upper respiratory tract infection.[ncbi.nlm.nih.gov]
Unable to Walk
  • However, about 25% of patients need artificial ventilation and 20% are still unable to walk unaided after 6 months.[ncbi.nlm.nih.gov]
  • Table 3 Patients unable to walk independently at six months after onset Figure 3 Milestones of recovery for Guillain-Barré syndrome patients unable to walk independently at six months after onset.[dx.doi.org]
Urinary Retention
  • She subsequently developed pandysautonomia that manifested as a tonically dilated pupil, gastrointestinal dysmotility, urinary retention, and profound orthostatic hypotension. Guillain-Barré syndrome was diagnosed on electromyography.[ncbi.nlm.nih.gov]
  • Urinary retention. Psychiatric problems - eg, depression, anxiety. Prognosis With modern intensive care support, the outcome is excellent for most patients.[patient.info]
  • retention Fisher variant opthalmoplegia, areflexia, with or without ataxia and/or weakness Evaluation Spirometry to ensure adequate respiratory function intubate early if necessary Lumbar puncture albumin cytologic disassociation elevated protein mostly[step2.medbullets.com]
  • A few patients (possibly with a variant form) have significant, life-threatening autonomic dysfunction causing BP fluctuations, inappropriate ADH secretion, cardiac arrhythmias, GI stasis, urinary retention, and pupillary changes.[msdmanuals.com]
Urinary Incontinence
  • After 6 months, she came back with acute onset of weakness in lower limbs, back pain, fever and urinary incontinence. Pinprick and light touch complete sensory loss was found beneath umbilicus.[ncbi.nlm.nih.gov]

Workup

There is no specific test for Guillain–Barré syndrome. Diagnosis is based on clinical symptomology and supported by nerve biopsy. Analysis of the cerebrospinal fluid is the only recommended laboratory study. Cerebrospinal fluid shows an elevated protein level and fewer mononuclear cells [5].

Routine blood tests may be helpful with monitoring the disorders progress. They may show an elevated sedimentation rate, hyponatraemia due to release of antidiuretic hormone, and mildly abnormal liver function tests [4] [5].

Laboratory studies suggested in Guillain–Barré syndrome include [5]:

  • Cerebrospinal fluid analysis
  • Antiganglioside antibodies
  • Stool culture for Campylobacter jejuni
  • Antibodies to Campylobacter jejuni, cytomegalovirus, Epstein Barr virus
  • Full blood count
  • Erythrocyte sedimentation rate
  • Liver function tests
  • Electrolytes

Other tests

  • Electrocardiograph
  • Autonomic function tests
  • Electrophysiology
  • Nerve biopsy

Magnetic Resonance Imaging is a non-specific test that may show enlargement of the anterior root indicative of inflammation which strongly suggests Guillain-Barré syndrome [5].

Slow Nerve Conduction Velocities
  • Nerve conduction analysis will reveal slow nerve conduction velocities due to the damage to the nerve. Lab work that screens for the following diseases should be performed to rule them out: mumps, rubella, cytomegalovirus, and myasthenia gravis.[emedicinehealth.com]
  • Initial electrodiagnostic testing detects slow nerve conduction velocities and evidence of segmental demyelination in two thirds of patients; however, normal results do not exclude the diagnosis and should not delay treatment.[msdmanuals.com]
  • Delayed distal latencies, slowed nerve conduction velocities, temporal dispersion of waveforms, conduction block, prolonged or absent F waves, and prolonged or absent H-reflexes are all findings that support demyelination.[emedicine.medscape.com]
Hypercapnia
  • Sleep hypercapnia and hypoxia, which worsens during sleep can be the result of a restrictive pulmonary function.[physio-pedia.com]

Treatment

There are currently only two therapies that have proven to be effective in treating Guillain–Barré syndrome: plasma exchange and intravenous immunoglobulin (IVIg) [1]. Guillain-Barré syndrome does not respond to corticosteroids and other immunosuppressive medications [1] [2]. Treatment with intravenous immunoglobulin and plasma exchange do not cure the disorder, but seem to reduce recovery time. Mortality rates and percentage of patients with residual disabled seem to be unchanged [2] [4] [6]. Both intravenous immunoglobulin and plasma exchange seem to be equally effective in treating Guillain–Barré syndrome [5].

Plasma exchange has been used in the treatment of Guillain–Barré syndrome since 1978 [5]. Studies have shown that plasma exchange, if begun in the first two weeks after the onset of symptoms, reduced hospital stay and length of assisted ventilation. Recommended therapy is two exchanges in mild cases and four in severe cases [5].

Plasma exchange is not risk free and may be associated with morbidity and mortality. Another limitation to this therapy is that it can only be administered in tertiary facilities with experienced personnel [6]. Complications of plasma exchange include the possibility of hypotension, abnormal clotting, and septicaemia [5].

Intravenous immunoglobulin is believed to suppress autoantibodies. It has been shown to be a safe form of treatment. When compared to plasma exchange it is equally effective, does not require a specialized medical environment for administration, is more convenient, and of comparable cost [5].

Studies have shown no significant difference between the effectiveness of plasma exchange and intravenous immunoglobulin, nor does there seem to be any advantage to using combined treatment [1] [2] [5]. As a result intravenous immunoglobulin treatment is the preferred therapy for Guillain–Barré syndrome [5].

Corticosteroid use in Guillain-Barré syndrome has not been shown to be effective [5]. However, some recent studies suggest the use of intravenous methylprednisolone in combination with intravenous immunoglobulin may be more beneficial [4] [5].

Current treatments, though helpful in decreasing the length of the acute phase of Guillain–Barré syndrome, do not decrease mortality or morbidity rates. It is evident, therefore that new treatment methods are needed for the disorder, aimed at decreasing or preventing disability from the disease [2] [5]. Current research is focused on doing this. Studies are looking at the use of nerve growth factors in the treatment of Guillain–Barré syndrome [4].

Prognosis

The prognosis and outcome of Guillain-Barré syndrome depend on multiple factors including: patient age, underlying disease, and availability of intensive medical care [5].

Between 3% and 12% of patients with Guillain–Barré syndrome die during the acute phase of the disease of complications usually from cardiac arrhythmias, pulmonary emboli, or respiratory failure [2] [4]. The mortality rate is highest in older patients. Approximately 25% of deaths occur during the first week and 50% in the first four weeks [5]. Ventilator assisted respiratory support is needed in 25% of patients with Guillain–Barré syndrome [2].

Recovery in cases of Guillain–Barré syndrome may take weeks to several months. Complete recovery may not occur for years or it may never be complete [2]. Studies have shown that 65% of patients have almost complete resolution of symptoms at the end of one year. The 35% figure of permanent disability has not changed despite the increased use of plasma exchange and intravenous immunoglobulin [4].

Other studies have shown 18% of patients recovering from Guillain–Barré syndrome are still unable to run  at one year, 9% unable to walk without help,  and 4% are bed ridden  or on a ventilator [5].

Recurrence is uncommon with Guillain–Barré syndrome, occurring in fewer than 5% of patients [1].

Etiology

About 75% of patients with Guillain–Barré syndrome have a history of symptoms of infection a few days to weeks before being diagnosed with the disease [4]. Associated organisms include Camyplobacter jejuni, cytomegalovirus, and Epstein-Barr virus. Evidence of Camyplobacter jejuni infection has been documented in about 30% of patients with Guillain -Barré syndrome [4]. Triggers for the disorder may also include immunizations. The outbreak of Guillain–Barré syndrome in the 1970’s was associated with national swine flu vaccination. Rabies vaccines have also been implicated. The discovery of antiganglioside antibodies in at least one third of patients seems to confirm this hypothesis. These antibodies cross-react with the infecting antigens. This may be a mechanism for the disease [4].

Guillain-Barré syndrome is now thought to include, not one, but several distinct subtypes [4]. All of the variants have the primary symptomatology of muscles weakness and nerve involvement, but have differences in clinical presentation.

Epidemiology

A disorder with “ascending paralysis” was first reported in 1859 [5]. The syndrome was first fully described in 1916 by French physicians, Guillain, Barré, and Strohl, during World War I. (4)(5) Two French soldiers presented with motor weakness, areflexia, and abnormalities in the cerebrospinal fluid. This clinical syndrome was named after Guillain and Barré [5].

The incidence of Guillain–Barré syndrome is 1 - 3:100,000 [4] [5]. In men it is 1.5 times higher than in women [1]. Guillain–Barré syndrome can occur at any age, [3] though studies show the incidence increases with age, with a small peak in young adults [6]. Approximately one-third of childhood cases occur before the age of 3 years [1].

Guillain–Barré syndrome includes multiple subtypes; the specific sub-type may vary by geographic location and/or population. The form of Guillain–Barré syndrome occurring in Europe and North America is the subtype that causes demyelination of the myelin sheath of peripheral neurons. An axonal form is common in China, Japan, India and Central America, but rarely seen in Europe and North America [2].

Sex distribution
Age distribution

Pathophysiology

Guillain–Barré syndrome is assumed to be an immune-mediated disease where a preceding infection triggers an immune response. This response then cross reacts with the patient’s nerves causing demyelination of nerve fibers or involvement of the axions [5]. The symptoms of Guillain–Barré syndrome are the result of diffuse inflammation of the myelin sheaths of the peripheral nerves and spinal roots [1] [2]. Studies have linked Guillain–Barré syndrome to Campylobacter jejuni, cytomegalovirus, and Epstein Barr virus [4].

Guillain–Barré syndrome is actually a group of subtypes resulting in disorders with similar symptoms and courses.  The two most common subtypes are: the demyelinating form of the disease accounting for about 75% of cases, and the subtype that attacks the axonal process and spares the myelin [4].

However, some features of the disorder are not characteristic of this type of pathology [1]. The major reason for rejecting the immune-mediated hypothesis is the lack of a response to immunosuppressive medications in Guillain–Barré syndrome and the fact that it is a single occurrence, acute rather than chronic, illness [1]. Thus the exact pathophysiology of Guillain–Barré syndrome is not fully understood.

Prevention

There is no clear means of preventing Guillian-Brre syndrome since the exact cause is not known. Prevention of the prodromal infections may decrease the incidence of the disease, however.

Summary

Guillain-Barré syndrome (GBS) is an acute autoimmune disease affecting peripheral nerves, central nervous system and the autonomic nervous system [1]. Guillain-Barré syndrome varies in severity and is thought to be triggered by a preceding infection [1]. Symptoms are a result of inflammation of the nerves [1] [2]. The disorder causes the rapid development of muscle weakness of the extremities, face, neck, and trunk. Muscles involved in swallowing and breathing are often affected [2]. The term Guillain-Barré syndrome actually refers a group of several disorders with similar etiology and pathology [1] [3].

The cause of Guillain–Barré syndrome is still not fully understood. The most common theory is that it is an autoimmune response triggered by an earlier bacterial or viral infection. The attack on the nervous system results when antibodies or T-cells mistake neural cells for the infecting microbes [2].

Guillain–Barré syndrome is a progressive disease, lasting from a few weeks to months. The initial, acute stage reaches a plateau within several weeks. The recovery stage lasts several more weeks or months and is often incomplete [2].

As many as 12% of patients with Guillain–Barré syndrome die of complications during the acute stage [2].

Patient Information

What is Guillain– Barré syndrome?

Guillain-Barré syndrome is an auto-immune disorder that causes progressive muscle weakness, sensory-motor losses, and possible life threatening respiratory and cardiac symptoms. It is thought to be the result of a dysfunction of the immune response following a mild viral or bacterial infection.

What are the symptoms?

The complications of Guillain-Barré syndrome are the result of damage to peripheral or cranial nerves. Symptoms include: weakness of muscles of extremities, numbness and tingling of fingers, facial drooping and difficulty swallowing, difficulty walking, pain, and respiratory distress. These complications include respiratory failure, cardiac arrhythmias, permanent motor disability, and possible death.

What causes Guillain–Barré syndrome?

Guillain-Barré syndrome is caused by an abnormal immune response, where nerve cells are mistaken for foreign viruses or bacteria. As a result nerve fibers are attacked by the body’s immune system.  Inflammation and damage to these nerves occurs causing the symptoms of the disease.

Who gets Guillain–Barré syndrome?

Anyone, at any age, is susceptible to Guillain- Barré syndrome.  In 80% of cases the disorder is preceded by a mild bacterial or viral infection.  This disease has also been associated with vaccination, swine flu and rabies in particular. The incidence of Guillain- Barré syndrome seems to increase with age.

How is it diagnosed?

There is no specific test for Guillain-Barré syndrome. Diagnosis is made by the clinical features of the disease: muscle weakness of extremities, facial palsy, pain, and numbness of fingers and toes.

How is Guillain–Barré syndrome treated?

There are two forms of treatment for Guillain- Barré syndrome: plasma exchange and intravenous immunoglobulin therapy.  Plasma exchange involves the washing of harmful substances from the blood.  immunoglobulin therapy is the infusion of human antibodies from donors into affected individuals.  Both of these treatments have been proven to shorten the course of the disease but do not seem to decrease its complications.

What are the complications of Guillain–Barré syndrome?

Complications of Guillain- Barré syndrome include:

References

Article

  1. Steiner I, Rosenberg G, and Wirguin I. Transient immunosuppression: a bridge between infection and the atypical autoimmunity of Guillain-Barré syndrome? British Society for Immunology, Clinical and Experimental Immunology, 2012, 162: 32-40.
  2. Hughes RAC, Pritchard J, Hadden RDM. Pharmacological treatment other than corticosteroids, intravenous immunoglobulin and plasma exchange for Guillain-Barré syndrome. Cochrane Database of Systematic Reviews 2013, Issue 2. 
  3.  Jacobs BC, Koga M, van Rijs W, Geleijns K, van Doorn PA, Willison HJ, et al. Subclass IgG to motor gangliosides related to infection and clinical course in Guillain-Barré syndrome. J Neuroimmunol. Feb 2008;194(1-2):181-90.
  4. Winer J B. Guillain Barré syndrome. Clin Pathol: Mol Pathol 2001;54:381–385.
  5. Seneviratne U. Guillain-Barré syndrome. Postgrad Med J. Dec 2000;76(902):774-82.
  6. Rajah A. The use of high-dose intravenous immunoglobulins in Guillain-Barrii syndrome. Anaesthesia, 1992, Volume 47, pages 220-222.
  7. Mullings KR, Alleva JT, Hudgins TH. Rehabilitation of Guillain-Barré syndrome. Dis Mon. May 2010;56(5):288-92.
  8. Jacobs BC, van Doorn PA, Schmitz PI, Tio-Gillen AP, Herbrink P, Visser LH, et al. Campylobacter jejuni infections and anti-GM1 antibodies in Guillain-Barré syndrome. Ann Neurol. Aug 1996;40(2):181-7.
  9. Bersano A, Carpo M, Allaria S, Franciotta D, Citterio A, Nobile-Orazio E. Long term disability and social status change after Guillain-Barré syndrome. J Neurol. Feb 2006;253(2):214-8.

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Last updated: 2019-07-11 20:43