Guillain–Barré syndrome (GBS) is a rare postinfectious, immune-mediated disorder affecting the nervous system.
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
- Symmetrical weakness of extremities (> 90%)
- Neck muscle weakness
- Weakness of respiratory muscles (52%)
- Cranial nerve palsy with difficulty swallowing (53%)
- Areflexia
- Sensory nerve involvement (80%)
- Pain (90%)
- Parenthesis, numbness and tingling
- Loss vibratory, touch, and pain sensation
- Ataxia
Autonomic system involvement
- Tachycardia and bradycardia or other arrthythmias
- Hypertension
- Postural hypotension
- Hypersalivation
- Decreased gastric motility and constipation (20%)
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].
Entire Body System
- Weakness
Home British & World English Guillain–Barré syndrome Guillain–Barré syndrome noun mass noun Medicine An acute disorder of the peripheral nerves, often preceded by a respiratory infection, causing weakness and often paralysis of the limbs. [en.oxforddictionaries.com]
This causes weakness. The weakness seen in GBS usually comes on quickly and worsens over hours or days. Symptoms are usually equal on both sides of the body (called symmetric). [ninds.nih.gov]
Symptoms of Guillain-Barré syndrome include: Neuro-muscular involvement Symmetrical weakness of extremities (> 90%) Neck muscle weakness Weakness of respiratory muscles (52%) Cranial nerve palsy with difficulty swallowing (53%) Areflexia Sensory nerve [symptoma.com]
The legs may feel heavy, wooden and weak and the arms may feel limp. Gripping, turning or holding things may be difficult. Rising from a chair may become a problem. Most patients feel very weak, and are unable to get out of bed. [healthnavigator.org.nz]
- Pain
The pain descriptors were compared to the severity of disease and laboratory examination results.Around 34.5% of the patients reported pain during the acute phase of GBS. [ncbi.nlm.nih.gov]
Pain was rated on a scale from 0 (no pain) to 10 (maximum pain). [doi.org]
Intravenous immunoglobulin (IVIg) Plasmapheresis Pain medication Physical therapy Speech therapy if needed Ventilator, if needed Take safety measures to compensate for loss of sensation [peripheralneuropathycenter.uchicago.edu]
They also carry sensations such as pain from the body to the brain. [health.harvard.edu]
- 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.com]
pain, esp. paresthesias affecting distal extremities Advanced symptoms Ascending paralysis: Bilateral flaccid paralysis spreads from the lower to the upper limbs in a “stocking‑glove” distribution. [amboss.com]
[…] and partially in his ower limbs, 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 [scinapse.io]
Sensory Sign and Symptom Hypesthesia 3 11.1 Paresthesia 2 7.4 Hyperesthesia 1 3.7 Pain at: – Inferior Limb 2 7.4 – Shoulder Girdle 1 3.7 – Back 1 3.7 – Head 1 3.7 c. [biomedpharmajournal.org]
- Unable to Stand
[…] to stand 1 mo after onset 5 7 Days after cough, ageusia, and anosmia Facial weakness, flaccid areflexic paraplegia (days 2–3), and respiratory failure (day 4) Day 3: protein level, 40 mg/dl; white-cell count, 3 per mm3; CSF:serum albumin ratio, 1.2%; [nejm.org]
A 12 years old male patient admitted in Government hospital with the complaint of inability to walk and stand. His lower extremities become weak. He cannot stand up and he was unable to walk. [longdom.org]
Patients may be unable to stand or walk despite reasonable strength, especially when ophthalmoparesis or impaired proprioception is present. Respiratory muscle weakness with shortness of breath may be present. [emedicine.medscape.com]
Therefore, a valid approach in milder GBS cases, with no bulbar or respiratory affection, is to closely observe patients for progression in the first 2 weeks while reserving treatment for those who become non-ambulatory or unable to stand unaided (9). [frontiersin.org]
Respiratoric
- 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]
Gastrointestinal
- Dysphagia
Clinical dysphagia was observed in seven patients and six of these cases involved a lower cranial nerve while five patients without cranial nerve involvement had silent dysphagia according to the dysphagia limit test. [ncbi.nlm.nih.gov]
- Vomiting
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 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]
He gave a history of frequent watery diarrhea, vomiting, and low grade fever a day prior to onset of the weakness. [ajns.paans.org]
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.com]
- Nausea
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]
The most common adverse effects of pazopanib include diarrhea, fatigue, and nausea, but neuropathic complication has not been documented. [ncbi.nlm.nih.gov]
[…] dict.cnki.net In addition to diarrhea, fever, abdominal cramps, nausea, and vomiting, campylobacter infection can cause Guillain-Barre syndrome, the most common cause of paralysis in the United States. [dict.youdao.com]
Finally, symptomatic treatment is not exempt from adverse effects, and may be a cause of iatrogenic consequences (i.e., ill effects caused by the treatment itself), such as allergic reactions, stomach bleeding, central nervous system effects (nausea, [en.wikipedia.org]
- 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]
[…] 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, urinary retention, bilateral tonic pupils, fluctuating heart rate [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]
Enteritis from Campylobacter jejuni has been identified as an important etiology2, thus a history of fever, crampy abdominal pain with nausea/vomiting/watery-diarrhea or bloody diarrhea would be important to elicit from the patient. [emdocs.net]
pain, vomiting, arthralgia or myalgia, dizziness, headache, fatigue, fever, and pain.[15] Serious side effects of immunoglobulin infusions include chest discomfort or pain, myocardial infarction, tachycardia hyponatremia, hemolysis, hemolytic anemia, [en.wikipedia.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]
Cardiovascular
- 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]
[…] metal poisoning, toxin ingestion (botulism, ciguatoxin, tetrodotoxin), tick paralysis, multiple sclerosis, carcinomatous meningitis Treatment Plasmaphoresis best effect if initiated within first 2 wks roughly halves hospital course adverse effects: hypotension [step2.medbullets.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, paroxysmal hypertension, dysrhythmiasetc.), constipation etc. [youtube.com]
[…] 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, dizziness, abdominal pain, ileus, orthostatic [lifeinthefastlane.com]
Sheps, Gastrointestinal motility disturbances in patients with orthostatic hypotension, Gastroenterology, 88, 6, (1852), (1985). Juan-R. [oadoi.org]
Eyes
- 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]
Rarely patients present with cranial nerve involvement, for example, diplopia, slurred speech, or swallowing difficulties. Progressive bilateral ophthalmoplegia and ataxia is suggestive of Miller Fisher syndrome. [bjgp.org]
The patient did not present nystagmus or diplopia. There were no signs of meningeal irritation. The blood test revealed leucocytosis of 22600/μL, ESR 52mm/h, GOT 42U/L, GGT 51U/L, LDH 990U/L, ferritin 551ng/mL and TSH 9.69μU/mL. [elsevier.es]
- 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]
Musculoskeletal
- Muscle Weakness
Symptoms of Guillain-Barré syndrome include: Neuro-muscular involvement Symmetrical weakness of extremities (> 90%) Neck muscle weakness Weakness of respiratory muscles (52%) Cranial nerve palsy with difficulty swallowing (53%) Areflexia Sensory nerve [symptoma.com]
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. [mountsinai.org]
Symptoms usually start with numbness or tingling in the fingers and toes, and over a few days, muscle weakness in the legs and arms develops. This usually affects both sides of the body. [healthnavigator.org.nz]
Facial weakness or paralysis can affect one or both sides of the face. Weakness or paralysis of the facial muscles will cause difficulty with eye movements, speech, chewing and swallowing. [facialpalsy.org.uk]
- 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. Symptoms usually start with numbness or tingling in the fingers and toes. Over several days, muscle weakness in the legs and arms develops. After about 4 weeks, most people begin to get better. [uofmhealth.org]
They also tend to have reduced reflexes and lower back pain. The tingling occurs on both sides.” May Kim-Tenser is co-director of the Neurointensive Care Unit and an assistant professor of clinical neurology at the Keck School of Medicine. [news.usc.edu]
- 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]
The most frequent signs and symptoms are paresthesias, weakness, and myalgias [1]. [hindawi.com]
These include arthritis, arthralgia, myalgia, Guillain-Barre syndrome, polyneuritis, polyneuropathy, anaphylaxis, and death. 这些疾病包括关节炎,关节痛,肌痛,Guillain-Barre综合症,多神经炎,?,过敏性反应以及死亡。 6. [danci.911cha.com]
- Proximal Muscle Weakness
Proximal muscle weakness very frequent, especially initially, with subsequent distal arm and leg weakness. [physio-pedia.com]
muscle weakness of the lower extremities. [emedicine.medscape.com]
- 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.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]
Urogenital
- 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]
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]
Complications can arise from Guillain-Barré syndrome which include: respiratory failure, infection, blood clots, pressure ulcers (injuries), facial paralysis, aspiration, difficulty swallowing or speaking, urinary retention, autonomic dysfunction (orthostatic [youtube.com]
Urinary retention. Psychiatric problems - eg, depression, anxiety. Prognosis With modern intensive care support, the outcome is excellent for most patients. [patient.info]
[…] the Urinary Tract Urinary Tract Infection Ureteral Calculi Urinary Tract Obstruction Urinary Disorders Secondary to Other Disease Processes Urinary Incontinence Urinary Retention Neurogenic Bladder Urinary Diversions 5. [play.google.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]
[…] the Urinary Tract Urinary Tract Infection Ureteral Calculi Urinary Tract Obstruction Urinary Disorders Secondary to Other Disease Processes Urinary Incontinence Urinary Retention Neurogenic Bladder Urinary Diversions 5. [play.google.com]
Neurologic
- Guillain-Barré Syndrome
Origin 1916: named after Georges Guillain (1876–1961) and Jean Barré (1880–1967), two of those who first described the syndrome. Pronunciation Guillain–Barré syndrome /ˌɡɪjãˈbareɪ/ [en.oxforddictionaries.com]
Guillain-Barré facts *Guillain-Barré facts Medically Edited by: Melissa Conrad Stöppler, MD Guillain-Barré syndrome (GBS) occurs when the immune system attacks the peripheral nervous system. [medicinenet.com]
- Ataxia
Instead, these patients have either ophthalmoplegia or ataxia. [dx.doi.org]
Miller Fisher syndrome is an uncommon variant of GBS that can be diagnosed by tests for its characteristic triad of conditions: areflexia, ataxia, and ophthalmoplegia.2 In cases of MFS, ataxia is primarily noted during the patient's gait, typically in [jaoa.org]
Ataxia in MFS may be due to peripheral and/or central pathology. [eyewiki.org]
Patients who had cranial nerve involvement, ataxia and weakened or disappeared tendon reflex should be paid attention. Albuminocytologic dissociation of cerebrospinal fluid and electromyography were helpful to diagnosis. [ncbi.nlm.nih.gov]
- Flaccid Paralysis
Guillain-Barré syndrome (GBS) is the most common cause of acute flaccid paralysis in children. [ncbi.nlm.nih.gov]
- 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]
The chief complaint may center on complaints of pain or paresthesia/weakness3. The course can begin with fine paresthesia in the toes or fingertips that is followed within days by leg weakness2. [emdocs.net]
- 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]
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].
Serum
- Hypercapnia
Emergency Management For respiratory emergencies, guidelines for intubation include a VC less than 12-15 mg/kg, refractory hypoxemia, hypercapnia, or an inability to clear oral secretions. [pulmonologyadvisor.com]
Sleep hypercapnia and hypoxia, which worsens during sleep can be the result of a restrictive pulmonary function.[18][19] Treatment methods are: Night time saturation records with pulse oximeter and bilevel positive airway pressure (BiPAP) may be indicated [physio-pedia.com]
Blood gas demonstrated severe hypoxemia (pO2 43.8 mmHg) and hypercapnia associated with respiratory acidosis (pCO2 99.1 mmHg, pH 7.06, HCO3− 28.2 mmol/L). [bmcinfectdis.biomedcentral.com]
Other Test Results
- Slow Nerve Conduction Velocity
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. [merckmanuals.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].
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:
- Permanent nerve damage resulting in motor dysfunction or facial palsies
- Respiratory failure requiring mechanical ventilation
- Cardiac arrhythmias, cardiac arrest
References
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