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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].

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]
  • His vital signs were stable. 5 days later, his condition aggravated and mechanical ventilation was necessitated owing to severe dyspnea.[ncbi.nlm.nih.gov]
  • The major components of the chief complaints of GBS patients were weakness, numbness, pain, cranial nerve involvement, dyspnea, ataxia and autonomic dysfunction.[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]
Weakness
  • We report a case of an adolescent girl who presented with peripheral ascending weakness, preceded by Campylobacter jejuni infection. After treatment with intravenous immunoglobulin, the peripheral weakness improved.[ncbi.nlm.nih.gov]
  • Onset is characterized by lower back, buttock, or leg pain, followed by development of symmetric flaccid limb weakness in the absence of sensory disturbance.[ncbi.nlm.nih.gov]
  • Guillain-Barré syndrome is a rare autoimmune condition characterized by ascending motor weakness of the extremities that can ascend to the diaphragm, causing substantial morbidity and mortality.[ncbi.nlm.nih.gov]
  • After IV immunoglobulin and corticosteroid therapy, she began to recover consciousness and from the weakness from day 18.[ncbi.nlm.nih.gov]
  • The patient had persistent weakness and progressed to areflexia, at which point urgent lumbar puncture supported a diagnosis of GBS.[ncbi.nlm.nih.gov]
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]
  • Neurological complications of dengue fever are rare but have been reported in the literature. A 60-year-old Sri Lankan man presented with a history of fever, arthralgia, and generalized malaise of 2 days duration.[ncbi.nlm.nih.gov]
  • Chikungunya virus (CHIKV) is a mosquito-borne alphavirus which presents with symptoms of fever, rash, arthralgia, and occasional neurologic disease.[ncbi.nlm.nih.gov]
  • There were also case reports of dengue fever preceding GBS.[ncbi.nlm.nih.gov]
  • Various forms of neurological manifestations are reported in dengue fever. We describe here three cases of concomitant Guillain-Barré syndrome and dengue virus (DENV) infection during the largest DENV-1 outbreak in New Caledonia.[ncbi.nlm.nih.gov]
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]
Cerebral Palsy
  • “Microcephaly can occur in isolation, but other birth defects and problems may also result, including seizures, cerebral palsy, hearing loss and developmental disability.[news.usc.edu]
  • palsy (over 75 per cent of cases are not diagnosed at birth but after six months) or other such names. [2009] - City resident gets Guillain-Barré after H1N1 shot [2009 Nov] Va. teen suffers rare illness after swine flu shot Jordan McFarland, 14, was[whale.to]
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]
  • 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]
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 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]
  • 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]
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]
  • Antibiotics can sometimes cause side effects in both you and your baby, including nausea and diarrhoea for you (RCOG 2017) . Your doctor will be able to explain any side effects before giving you antibiotics.[babycenter.com.au]
  • Patients may develop fever, myalgia, headache, nausea, and vomiting, but these “influenza-like” symptoms are self-limiting. 1 Patients also may develop aseptic meningitis, neutropenia, and hypertension.[aafp.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]
  • 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]
Hypertension
  • Subsequently, the patient developed severe constipation and hypertension with tachycardia. Nerve conduction studies revealed demyelinating polyneuropathy. Serum anti-GQ1b IgG antibody was detected.[ncbi.nlm.nih.gov]
  • Risk factors associated with hyponatremia in multivariable analysis included advanced age, deficiency anemia, alcohol abuse, hypertension, and intravenous immunoglobulin (all P 0.0001).[ncbi.nlm.nih.gov]
  • Interleukin (IL)-17 levels are elevated in the CSF and plasma in GBS patients, and elevated IL-17 in the CSF of patients with idiopathic intracranial hypertension has been reported.[ncbi.nlm.nih.gov]
  • Diabetes requiring insulin was more common and hypertension less common with corticosteroids based on high quality evidence. Read the full abstract...[cochrane.org]
  • Sustained hypertension managed by angiotensin-converting enzyme inhibitor or beta blocking agent. Use short acting intravenous medication for labile hypertension requiring immediate therapy. iii.[physio-pedia.com]
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]
  • Shortly after uncomplicated intubation she developed hypotension and a profound tachycardia. The electrocardiogram showed sinus tachycardia with nonspecific ST-T segment changes.[ncbi.nlm.nih.gov]
  • 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]
  • 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]
  • 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]
  • Pandysautonomia The clinical features are hypertension, orthostatic hypotension, vomiting, diarrhoea or constipation, paralytic ileus, sweating disturbances and cardiac arrhythmias.CSF examination shows ACD in CSF.[vdocuments.us]
  • Unexplained autonomic nervous system involvement may cause sinus tachycardia or bradycardia, hypertension, orthostatic hypotension, and loss of bladder and bowel sphincter control.[zdoc.site]
Diplopia
  • 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]
  • Common complaints include: Facial Palsy Diplopia Dysarthria Dysphagia Ophthalmoplegia Pupillary disturbances . Facial and oropharyngeal weakness usually appears after the trunk and limbs are affected.[physio-pedia.com]
  • 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]
  • Common complaints include the following: Facial droop (may mimic Bell palsy) Diplopias Dysarthria Dysphagia Ophthalmoplegia Pupillary disturbances Facial and oropharyngeal weakness usually appears after the trunk and limbs are affected.[emedicine.medscape.com]
Blepharoptosis
  • 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]
Muscle Weakness
  • 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 characterised by muscle weakness, which occurs because of damage to the peripheral nervous system.[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]
  • We report on a 39-year-old woman who was intubated because of progressive respiratory failure due to muscle weakness and mucous plugging because of Guillain- Barré syndrome.[ncbi.nlm.nih.gov]
  • After 8 months of rehabilitative therapy, she was able to swallow food without difficulty, but she still cannot walk without assistance, showed cognitive impairment, and the tracheostomy remained in place because of persistent respiratory muscle weakness[ncbi.nlm.nih.gov]
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]
  • 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]
  • It rendered Miss Bailey paralysed, unable to walk, talk or breathe The next morning when she woke up, she unknowingly dismissed more signs of the condition, blaming a 'really bad hangover' for her back pain and numb legs.[dailymail.co.uk]
Myalgia
  • A higher proportion of case-patients (83%) compared to controls (21%) reported an antecedent illness (OR 18.1, CI 6.9-47.5), most commonly characterized by rash, headache, fever, and myalgias, within a median of 8 days prior to GBS onset.[ncbi.nlm.nih.gov]
  • 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]
  • Patients may develop fever, myalgia, headache, nausea, and vomiting, but these “influenza-like” symptoms are self-limiting. 1 Patients also may develop aseptic meningitis, neutropenia, and hypertension.[aafp.org]
Proximal Muscle Weakness
Muscular Atrophy
  • 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.[scinapse.io]
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]
  • Dysautonomia (hypotension, hypertension, arrhythmias, and urinary retention) occur in about 70 percent of patients.[journalofethics.ama-assn.org]
  • 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 axonal and demyelinating[lifeinthefastlane.com]
  • 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]
  • More than one-half of patients experience severe pain, and about two-thirds have autonomic symptoms, such as cardiac arrhythmias, blood pressure instability, or urinary retention. Advancing symptoms may compromise respiration and vital functions.[aafp.org]
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]
Guillain-Barré Syndrome
  • […] of Guillain-Barré syndrome.[ncbi.nlm.nih.gov]
  • Our aim was to assess the correlation between serum bilirubin levels and Guillain-Barré syndrome (GBS).[ncbi.nlm.nih.gov]
  • Double-blind trial of intravenous methylprednisolone in Guillain-Barré syndrome. Guillain-Barré Syndrome Steroid Trial Group. Lancet . 1993 Mar 6. 341(8845):586-90. [Medline] . Hughes RA, Pritchard J, Hadden RD.[emedicine.medscape.com]
  • Here, we report a rare case of ulcerative colitis with Guillain-Barré Syndrome. We described a patient with Guillain-Barré syndrome during the remission period of ulcerative colitis.[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]
  • Lower extremity weakness with upper and lower extremity paresthesias developed progressively 11 months post transplantation, coinciding with immune control of CMV.[ncbi.nlm.nih.gov]
  • The patient was treated with intravenous immunoglobulin and experienced near-complete neurologic recovery, reporting ongoing mild paresthesia up to 2 months later.[ncbi.nlm.nih.gov]
  • A 55year old woman in New York City presented in May 2016 with progressive weakness, ataxia, paresthesia, and areflexia, shortly after returning from the Dominican Republic. Lumbar puncture revealed cytoalbuminological dissociation.[ncbi.nlm.nih.gov]
  • Reported herein is a case of nivolumab-induced chronic inflammatory demyelinating polyradiculoneuropathy, in which an 85-year-old woman with stage IV melanoma developed grade 1 paresthesia 2 weeks after the initial dose of nivolumab was administered.[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]
  • Guillain-Barré syndrome (GBS) is an immune-mediated peripheral neuropathy. The goal of this research was the identification of biomarkers associated with recovery from GBS.[ncbi.nlm.nih.gov]
  • Guillain-Barré syndrome (GBS), an immune-mediated demyelinating peripheral neuropathy, is characterized by acute weakness of the extremities and areflexia or hyporeflexia.[ncbi.nlm.nih.gov]
  • Though GBS is typically considered a peripheral neuropathy, evidence for CNS involvement exists; GBS should be considered within the differential diagnosis, and neurological features should be monitored, in a patient with new onset unclear psychiatric[ncbi.nlm.nih.gov]
  • TNF has a proinflammatory action, and various immunoregulatory actions that, together, seem to promote the development of peripheral neuropathies syndromes in the organism.[ncbi.nlm.nih.gov]
Flaccid Paralysis
  • An 82-year-old woman presented with acute ascending flaccid paralysis and acute respiratory failure.[ncbi.nlm.nih.gov]
  • BACKGROUND Guillain-Barré syndrome (GBS) is an acute inflammatory demyelinating polyneuropathy that is usually associated with preceding respiratory or gastrointestinal infection and has the hallmark manifestation of ascending flaccid paralysis.[ncbi.nlm.nih.gov]
  • Despite being the most common cause of acute flaccid paralysis in children Guillain-Barré syndrome has a low incidence under 18 years old, and is even rarer under the age of 2.[ncbi.nlm.nih.gov]
  • In regions where dengue is hyperendemic, screening for dengue illness may be important in patients presenting with acute flaccid paralysis.[ncbi.nlm.nih.gov]
  • Guillain-Barré syndrome (GBS) is an autoimmune polyneuropathy causing acute flaccid paralysis and it is known to improve with plasmapheresis.[ncbi.nlm.nih.gov]
Cranial Nerve Involvement
  • Furthermore, cranial nerve involvement in patients with GBS likely increased the incidence of oropharyngeal dysphagia, and subclinical dysphagia may be present in this population as well.[ncbi.nlm.nih.gov]
  • The major components of the chief complaints of GBS patients were weakness, numbness, pain, cranial nerve involvement, dyspnea, ataxia and autonomic dysfunction.[ncbi.nlm.nih.gov]
  • Blink reflex (BR) studies were performed in 57 patients and were abnormal in 80% of those with clinical facial weakness and in 17 of 52 patients (33%) with no clinical cranial nerve signs, suggesting subclinical cranial nerve involvement.[ncbi.nlm.nih.gov]
  • Cranial Nerve Involvement Bulbar palsies, Facial weakness, Optic nerve palsies [ Teksam, 2015 ] 46% of cases [ Hicks, 2010 ] Autonomic dysfunction Blood pressure instability, sinus tachycardia Abnormal sweating, pupillary abnormalities Common and under-recognized[pedemmorsels.com]
  • Albuminocytologic dissociation, nerve conduction findings, disease progression over days to four weeks, symmetry, mild sensory abnormalities, cranial nerve involvement, autonomic dysfunction, and recovery that starts in two to four weeks all support a[journalofethics.ama-assn.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].

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]
Hypercapnia
  • Sleep hypercapnia and hypoxia, which worsens during sleep can be the result of 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:

  • Permanent nerve damage resulting in motor dysfunction or facial palsies
  • Respiratory failure requiring mechanical ventilation
  • Cardiac arrhythmias, cardiac arrest

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: 2018-06-21 23:40