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Guillain-Barré syndrome.....My Understanding..
 

Guillain-Barré syndrome.....My Understanding..

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  • Bradley’s neurology in clinical practice

Guillain-Barré syndrome.....My Understanding.. Guillain-Barré syndrome.....My Understanding.. Presentation Transcript

  •  Guillain-Barré syndrome (GBS) is an acute, frequently severe, and fulminant polyradiculoneuropathy that is autoimmune in nature. Males are at slightly higher risk for GBS than females, and in Western countries adults are more frequently affected than children.
  • Subtypes
  • Antecedent events Approximately 70% of cases of GBS occur 1–3 weeks after an acute infectious process, usually respiratory or gastrointestinal. Viruses e.g human herpes virus infection, CMV, Epstein- Barr virus and Mycoplasma pneumoniae; Infection or reinfection with Campylobacter jejuni , recent immunizations. The swine influenza vaccine, administered widely in the United States in 1976, is the most notable example. Older-type rabies vaccine, prepared in nervous system tissue, the mechanism is presumably immunization against neural antigens.
  •  GBS occurs more frequently in patients with lymphoma (including Hodgkins disease), HIV-seropositive individuals, Patients with systemic lupus erythematosus. C. jejuni has been implicated in summer outbreaks of AMAN among children and young adults exposed to chickens in rural China.
  •  Panel A shows the immunopathogenesis of acute inflammatory demyelinating polyneuropathy. Autoantigens unequivocally identified, autoantibodies bind to myelin antigens and activate complement. Formation of membrane- attack complex (MAC) on the outer surface of Schwann cells and the initiation of vesicular degeneration. Macrophages subsequently invade myelin and act as scavengers to remove myelin debris.
  •  Panel B shows the immunopathogenesis of acute motor axonal neuropathy. Myelinated axons are divided into four functional regions: the nodes of Ranvier, paranodes, juxtaparanodes, and internodes. Gangliosides GM1 and GD1a are strongly expressed at the nodes of Ranvier, where the voltage-gated sodium (Nav) channels are localized. Contactin associated protein (Caspr) and voltage- gated potassium (Kv) channels are respectively present at the paranodes and juxtaparanodes.
  •  IgG anti-GM1 or anti-GD1a autoantibodies bind to the nodal axolemma, leading to MAC formation. Results in the disappearance of Nav clusters and the detachment of paranodal myelin, which can lead to nerve-conduction failure and muscle weakness. Axonal degeneration may follow at a later stage. Macrophages subsequently invade from the nodes into the periaxonal space, scavenging the injured axons.
  • Neuropathies
  • Gangliosides
  •  Gangliosides, which are composed of a ceramide attached to one or more sugars (hexoses) and contain sialic acid (N-acetylneuraminic acid) are important components of the peripheral nerves. Four gangliosides GM1, GD1a, GT1a, and GQ1b — differ with regard to the number and position of their sialic acids, where M, D, T, and Q represent mono-, di-, tri-, and quadri-sialosyl groups.
  •  IgG autoantibodies to GM1 and GD1a are associated with acute motor axonal neuropathy and its subtypes; Subtypes More extensive acute motor-sensory axonal neuropathy Less extensive acute motor conduction-block neuropathy, not with acute inflammatory demyelinating polyneuropathy. Motor and sensory nerves express similar quantities of GM1 and GD1a, but their expression within various tissues may differ. This explains the preferential motor axon injury seen in acute motor axonal neuropathy
  •  IgG autoantibodies to GQ1b, which cross-react with GT1a, are strongly associated with the Miller Fisher syndrome. Patients with pharyngeal–cervical–brachial weakness More likely to have IgG anti-GT1a antibodies, which may cross-react with GQ1b, Less likely to have IgG anti-GD1a antibodies, which suggests a link to the axonal Guillain–Barré syndrome Glossopharyngeal and vagus nerves strongly express GT1a and GQ1b, possibly accounting for dysphagia in this subtype.
  •  Guillain-Barré syndrome (GBS), Miller Fisher syndrome (FS) and Bickerstaff brainstem encephalitis represent a spectrum of acute post-infectious immune-mediated diseases. Miller Fisher syndrome (MFS) is characterized by an acute onset of ataxia, areflexia without weakness and ophthalmoplegia. Miller Fisher syndrome (MFS) can be broadly categorized in to two part, More extensive form, Bickerstaff brainstem encephalitis (BBE) characterized by Miller Fisher syndrome with impairment of consciousness
  •  Incomplete form Acute ophthalmoparesis without ataxia Acute onset ataxia neuropathy without ophthalmoplegia Anti-GQ1b antibody syndrome includes Miller Fisher syndrome, Acute ophthalmoparesis, without ataxia Acute ataxic neuropathy, without ophthalmoplegia Bickerstaff’s brain-stem encephalitis Pharyngeal–cervical–brachial weakness.
  •  Anti-GQ1b IgG antibodies are found in >90% of patients with MFS and titers of IgG are highest early in the course. GQ1b is strongly expressed in the oculomotor, trochlear, and abducens nerves, and muscle spindles in the limbs. EO motor nerves are enriched in GQ1b gangliosides in comparison to limb nerves. Pharyngeal–cervical–brachial weakness categorized as localized form of acute motor axonal neuropathy or an extensive form of the Miller Fisher syndrome. Half of patients of pharyngeal–cervical–brachial weakness have IgG anti-GT1a antibodies, which often cross-react with GQ1b.
  • Clinical symptoms Rapidly evolving hypo to areflexic motor paralysis with or without sensory disturbance. Although hyporeflexia or areflexia is a hallmark of the GBS, 10% of patients have normal or brisk reflexes during the course of illness. Ascending paralysis, noticed as rubbery legs. The legs are usually more affected than the arms. Facial diparesis is present in 50% of affected individuals.
  •  The lower cranial nerves frequently involved, causing bulbar weakness. Mistaken for brainstem ischemia. Bladder dysfunction may occur in severe cases but is usually transient. Autonomic involvement is common Loss of vasomotor control with wide fluctuation in blood pressure, postural hypotension, and cardiac dysrhythmias.
  •  Fever and constitutional symptoms are absent Deep tendon reflexes attenuate or disappear within the first few days of onset Functions subserved by large sensory fibers, such as deep tendon reflexes and proprioception, are affected.
  • Diagnostic criteria
  • GBS disability score 0, A healthy state; 1, Minor symptoms and capable of running; 2, Able to walk 10 meters or more without assistance but unable to run; 3, Able to walk 10 meters across an open space with help; 4, Bedridden or chair bound; 5, Requiring assisted ventilation for at least part of the day; 6, Dead.
  • Criteria for mechanical ventilationin absence of clinical respiratorydistress Major Hypercarbia (partial pressure > 48 mm hg) Hypoxemia (partial pressure <56 mm hg) Vital capacity less than 15ml/kg body weight Minor Inefficient cough Impaired swallowing Atelectasis1 major and 2 minor criteria needed
  • Brain 1996, 119,2053-2061The prognosis and main prognostic indicators of GBS The percentage of patients with respiratoty failure seemed to increase with age (<35 years,7%; 35-54 years, 13%; 55+ years, 18%) Mean age to clinical recovery was 157 days in patients aged 35-54 years, and 253 days in patients 55 years and older. Patients in whom gastroenteritis preceded the onset of symptoms had the longest interval to clinical recovery.
  • DIFFERENTIAL DIAGNOSIS 1.ACUTE POLYNEUROPATHIES Hepatic porphyria Critical illness polyneuropathy Diptheria Vasculitis Toxins - arsenic,thallium,organophosphorus,lead,neurotoxic fish
  •  DISORDER OF NEUROMUSCULAR JUNCTION Botulism Myaesthenia gravis Tick paralysis
  • POLYRADICULOPATHIES Inflammatory or neoplastic meningoradiculopthy Lyme radiculitis Cytomegalovirus lumbosacral radiculomyelopathy
  •  CNS DISORDERS Transverse myelitis Basilar artery thrombosis Rabies
  • MYOPATHEIS Hypokalemia Hypophosphatemia Rhabdomyolysis Polymyositis Critical care neuropathy
  •  ANTERIOR HORN SYNDROME Poliomyelitis West nile and enterovirus poliomyelitis
  • Paralytic Polio GBSFever occurs just Fever Occurs 2-3 weeksbefore onset of before onset ofparalysis. paralysisAsymmetrical SymmetricalDescending AscendingCSF-Normal Protein CSF-IncreasedWBC 20- 300 proteins WBC < 10
  • GBS vs BOTULINISM Bilateral symmetrical & descending flaccid paralysis occurs after 12-36 hr of ingestion Oculobulbar weakness is an early feature of botulism. Absence indicates an alternative diagnosis.(BMJ /best practice) Nausea, vomiting, constipation, diplopia, ophthalmoplegia, ptosis, blurring of vision, dysphagia, dysarthria, urinary retention. There is No fever. Consciousness is not impaired Deep Tendon reflexes are absent. There is No sensory loss. CSF examination is normal.
  • Hypokalaemic periodic paralysis Symptoms typically begin in the first or second decade, attacks of flaccid paralysis usually occurring on awakening in the night or in the early morning. Weakness may be focal or generalized, usually sparing facial and respiratory muscles, and lasting for hours (occasionally days) with gradual resolution.
  • THALLIUM POISONING Ascending type of painful sensorimotor neuropathy with abdominal pain, nausea and vomiting(DTR can be present or reduced)Development of skin lesions on 10-15 th day Hair loss between 15th to 20th day
  • GBS VS PORPHYRIA Abdominal pain, peripheral neuropathy, and changes in mental status are the classic triad of an acute attack. Severe abdominal pain is the most commonly reported presenting symptom during acute attacks.
  • GBS VS CRITICAL ILLNESSPOLYNEUROPATHY The neuromuscular syndrome of acute limb and respiratory weakness that commonly accompanies patients with multi-organ failure and sepsis constitutes critical illness polyneuropathy. Vijayan J, Alexander M. Critical illness neuropathy. Indian J Crit Care Med 2005;9:32-4
  • Vasculitic neuropathy systemic vasculitis/nonsystemic vasculitic neuropathy mononeuritis multiplex or asymmetric sensorimotor neuropathy. Symmetric neuropathy is rare Asymmetric or multifocal painful sensorimotor neuropathy is the most common presentation systemic symptoms (e.g.,unexplained weight loss, fevers, rash); multiorgan involvement (e.g., joints, skin, kidney, respiratory tract).
  •  INVESTIGATION – ELECTRODIAGNOSTIC STUDIES(NCS) CSF EXAMINATION
  • NERVE CONDUCTIONSTUDIES
  •  CMAP amplitude NCV distal motor latencies (DML) F-wave latency H reflex Abnormal temporal dispersion Conduction block
  • CMAP The CMAP is the sum of all the action potentials occurring individually in the contracting muscle fibers. Motor nerve conduction is evaluated by recording the compound muscle action potential (CMAP) associated with a mechanical contraction of a given muscle (twitch), in response to electrical stimulation of the motor nerve fibers supplying that muscle.
  • CMAP Amplitude and Latency
  •  Why is it necessary to use two stimulation sites? The time between the shock and the appearance of the CMAP (the latency) comprises three components: (1) time for action potentials to travel down the nerve, (2) time to cross the neuromuscular junction, (3) time for muscle action potentials to disperse throughout the muscle. Only the first component is relevant to calculating nerve conduction velocity. Including components (2) and (3) would introduce a small systematic error, but these are constants, and can be removed by subtracting the distal stimulation site latency from the proximal site latency.
  • NERVE CONDUCTION VELOCITY
  • In a typical F wave study, a strong electrical stimulus(supramaximal stimulation) is applied to the skin surface above thedistal portion of a nerve so that the impulse travels both distally(towards the muscle fiber) and proximally (back to the motorneurons of the spinal cord) as shown in figure.
  •  When the orthodromic stimulus reaches the muscle fiber, it elicits a strong M wave indicative of muscle contraction. When the antidromic stimulus reaches the motor neuron cell bodies, a small portion of the motor neurons backfire and orthodromic wave travels back down the nerve towards the muscle. This reflected stimulus evokes small proportion of the muscle fibers causing a small,second CMAP called the F wave. The name F wave is derived since test was done the first time in the intrinsic muscles of foot by Magladery and McDougal in 1950. The afferent and efferent for F wave s are alpha motor neurons.
  •  Why are F waves useful? F waves allow testing of proximal segments of nerves that would otherwise be inaccessible to routine nerve conduction studies. F waves test long lengths of nerves whereas motor studies test shorter segments. Therefore F wave abnormalities can be a sensitive indicator of peripheral nerve pathology, particularly if sited proximally. The F wave ratio which compares the conduction in the proximal half of the total pathway with the distal may be used to determine the site of conduction slowing—for example, to distinguish a root lesion from a patient with a distal generalised neuropathy
  • H REFLEX
  •  Hoffmann reflex (H-reflex) and muscle response (M-wave) pathways. When a short-duration, low-intensity electric stimulus is delivered, action potentials are elicited selectively in sensory Ia afferents due to their large axon diameter . These action potentials travel to the spinal cord, where they give rise to excitatory postsynaptic potentials, in turn eliciting action potentials, which travel down the alpha motor neuron (αMN) axons toward the muscle (response 3). Subsequently, the volley of efferent action potentials is recorded in the muscle as an H-reflex. Gradually increasing the stimulus intensity causes action potentials to occur in the thinner axons of the αMNs (response 1), traveling directly toward the muscle and recorded as the M-wave
  • Conduction blocks Conduction block: compound musle action potential amplitudes drop by more the 40% on proximal stimulation compared to distal stimulation. Highly suggestive of acquired demyelinating neuropathy.
  • Temporal dispersion (TD) Decrement-a reduction of the compound muscle action potential (CMAP) on proximal versus distal stimulation Abnormal decrement can also be the result of increased temporal dispersion (TD), which is an increase in the difference between the conduction times along the different axons within a nerve.
  • Difference between demyelinationand axonal degeneration Demyelinating Neuropathy slow conduction velocity, prolonged sensory and motor latencies, generally preserved amplitueds Axonal Neuropathy reduced sensory and motor amplitudes, normal latencies and velocities, fibrillations and positive sharp waves
  • Electrodiagnostic Medicine Criteria forPeripheral Nerve Demyelination Conduction velocity reduced in 2 or more nerves 1. If CMAP amplitude is > 80% of lower limit of normal (LLN), the NCV must be < 80% of LLN. 2. If CMAP amplitude < 80% of LLN, the NCV must be < 70% of LLN
  • CMAP conduction block or abnormal temporal dispersion in1 or more nerves1. Regions to examine:• Peroneal nerve between fibular head and ankle• Median nerve between wrist and elbow• Ulnar nerve between wrist and below elbow2. Partial conduction block criteria• CMAP duration difference between the above noted proximaland distal sites of stimulation must be < 15%; and A > 20% drop inCMAP negative spike duration, or baselineto- peak amplitude.3. Abnormal temporal dispersion and possible conduction block• CMAP duration difference between the above proximal anddistal sites of stimulation is > 15%; and• > 20% drop in CMAP negative spike duration or baseline-topeakamplitude.
  •  Prolonged distal motor latencies (DML) in 2 or more nerves 1. If CMAP amplitude is > 80% of LLN, the DML must be > 125% of the upper limit of normal (ULN). 2. If the CMAP is < 80% of LLN, the DML must be > 150% of ULN. Prolonged minimum F-wave latency or absent F- wave 1. F-waves performed in 2 or more nerves (10–15 trials) 2. If the CMAP amplitude is > 80% of LLN, the F-wave latency must be > 120% of ULN. 3. If CMAP amplitude is < 80% of LLN, the F-wave latency must be > 150% of ULN.
  • Salient features of NCS in GBS The most common EDX abnormalities seen in the first 2 weeks of illness are absent H reflex and absent,delayed or impersitent F waves,finding that are common in polyneuropathies but not specific for demyelinating type. Reduced CMAP amplitude or SNAPs in upper extremity combined with normal Sural SNAPs (sural sparing) are changes highly specific for diagnosis of AIDP and occurs in 50% of patient in first 2 weeks of illness Sural sparing combined with abnormal F waves has very high sensitivity but occurs in only a third of patients during first 2 weeks of illness.
  •  Conduction block of motor axons-recognised as decrease of greater than 50% in CMAP amplitude from distal to proximal stimulation in the absence of temporal dispersion. Conduction block is highly specific for demyelination but occurs in only 15-30% of early GBS.
  • CSF EXAMINATION Classic finding is elevated CSF protein with normal cell count (albumino cytological dissociation). Occurs in up to 90% at week 1 after symptom onset. CSF protein is usually normal within the first 2 to 3 days but then begins to rise very quickly, reaching a peak at 4 to 6 weeks and then persisting at a variably elevated level for many weeks.
  •  CSF protein level varies from 0.45 to 3.0 g/L (45-300 mg/dL), but levels as high as 10 g/L (1000 mg/dL) can be seen. Around 59% of patients with Bickerstaff brainstem encephalitis (BBE) have elevated protein in CSF
  •  Around 10% will not have a protein elevation and this includes patients with the Miller-Fisher variant. Cell counts are typically <5 cells/mm^3. However, in 10% of patients, lymphocytosis <50 cells/mm^3 may be present early on but quickly normalises over a few days.
  •  HIV testing is done in high-risk person or presence of CSF lymphocytic pleocytosis (>100 cell/mm^3)
  •  Monitoring of cardiac and pulmonary dysfunction Electrocardiography, blood pressure, pulse oximetry for oxyhemoglobin saturation, vital capacity, and swallowing should be regularly monitored in patients who have severe disease, with checks every 2–4 hr if the disease is progressing and every 6–12 hr if it is stable. Insertion of a temporary cardiac pacemaker, use of a mechanical ventilator,and placement of a nasogastric tube should be performed on the basis of the monitoring results. Guillain–Barré Syndrome. Nobuhiro Yuki, M.D., Ph.D., and Hans-Peter Hartung, M.D.. N Engl J Med 2012; 366:2294-2304
  •  Prevention of pulmonary embolism Prophylactic use of subcutaneous heparin and compression stockings is recommended for adult patients who cannot walk.
  •  Immunotherapy Intravenous immune globulin or plasma exchange should be administered in patients who are not able to walk unaided. In patients whose status deteriorates after initial improvement or stabilization, retreatment with either form of immunotherapy can be considered. However, plasma exchange should not be performed in patients already treated with immune globulin because it would wash out the immune globulin still present in the blood. Also, immune globulin should not be used in patients already treated with plasma exchange because this sequence of treatments is not significantly better than plasma exchange alone
  • Treatment A multidisciplinary consensus group has recommended subcutaneous heparin and graduated stockings to prevent deep venous thrombosis and pulmonary emboli in pt admitted in ICU. Hughes RA, Wijdicks EF, Benson E, Cornblath DR, Hahn AF,Meythaler JM, et al, Multidisciplinary Consensus Group. Supportive care for patients with Guillain-Barré syndrome. Arch Neurol 2005;62:1194-8.
  •  Recommendation: ACP recommends against the use of mechanical prophylaxis with graduated compression stockings for prevention of venous thromboembolism (Grade: strong recommendation,moderate-quality evidence). Recommendation: ACP recommends pharmacologic prophylaxis with heparin or a related drug for venous thromboembolism in medical (including stroke) patients unless the assessed risk for bleeding outweighs the likely benefits (Grade: strong recommendation, moderate- quality evidence). Venous Thromboembolism Prophylaxis in Hospitalized Patients: A Clinical Practice Guideline From the American College of Physicians. Ann Intern Med. 2011;155:625-632.
  •  The use of enoxaparin plus elastic stockings with graduated compression, as compared with elastic stockings with graduated compression alone, was not associated with a reduction in the rate of death from any cause among hospitalized, acutely ill medical patients. Kakkar AK, Cimminiell C, Goldhaber SZ, Parakh R, Wang C, Bergmann JF; LIFENOX Investigators. Low-molecular-weight heparin and mortality in acutely ill medical . patients. N Engl J Med 2011;365:2463-72
  • PAIN MANAGEMENT Pain management is not easy, but gabapentin and carbamazepine may help. Narcotic analgesics may occasionally be needed Hughes RA, Wijdicks EF, Benson E, Cornblath DR, Hahn AF, Meythaler JM, et al, Multidisciplinary Consensus Group. Supportive care for patients with Guillain-Barré syndrome. Arch Neurol 2005;62:1194-8.
  • Mechanical ventilation in GBS The major criteria are hypercarbia (partial pressure of arterial carbon dioxide, >6.4 kPa [48 mm Hg]), hypoxemia (<7.5 kPa [56 mm Hg]) {partial pressure of arterial oxygen while the patient is breathing ambient air} vital capacity less than 15 ml per kilogram of body weight Burakgazi AZ, Höke A. Respiratory muscle weakness in peripheral neuropathies. J Peripher Nerv Syst 2010;15:307-13.
  •  Minor criteria are Inefficient cough Impaired swallowing Atelectasis Even in the absence of clinical respiratory distress, mechanical ventilation may be required in patients with at least one major criterion or two minor criteria.
  •  Rapid disease progression, Bulbar dysfunction, Bilateral facial weakness, Dysautonomia. Respiratory failure included vital capacity of less than 20 ml/kg, Maximal inspiratory pressure less than 30 cm H2O, Maximal expiratory pressure less than 40 cm H2O Reduction of more than 30% in vital capacity, maximal inspiratory pressure, or maximal expiratory pressure. No clinical features predicted the pattern of respiratory decline; however, serial measurements of pulmonary function tests allowed detection of those at risk for respiratory failure. Lawn ND, Fletcher DD, Henderson RD, Wolter TD, Wijdicks EM. Anticipating Mechanical Ventilation in Guillain- Barré Syndrome. Arch Neurol. 2001;58(6):893-898
  • Independent predictors of mechanical ventilationPaul BS, Bhatia R, Prasad K, Padma MV, Tripathi M, Singh MB. Clinical predictorsof mechanical ventilation in Guillain-Barré syndrome. Neurol India 2012;60:150-3
  • PLASMA EXCHANGE Cochrane review has shown that plasma exchange is better than supportive treatment .
  •  In five randomised but unblinded clinical trials of 623 patients, plasma exchange reduced the proportion of patients needing ventilation from 27% to 14% (relative risk 0.53, 95% confidence interval 0.39 to 0.74, P=0.001). Similarly, the time taken to recover walking with an aid was significantly shortened in two trials (30 v 44 days, P<0.01).
  •  Therapeutic plasma exchange has been recommended for moderate to severe weakness(defined as ability to walk only with support or worse). French cooperative group on plasma exchange(1997)-Even mildly affected patients benefit from two exchanges. Four exchanges were optimal for moderate to severe cases and six exchanges did not have any additional benefit. The recommended schedule entails a series of 4-5 exchanges(40-50 ml/kg) with a continuous flow machine on alternate days, using saline and albumin as replacement fluid.
  • PLASMA EXCHANGE Complications – Hematoma formation at puncture sites Pneumothorax after insertion of central line Catheter related septicemia Contraindications Septicemia Active bleeding Severe cardiovascular instability
  • IV IMMUNOGLOBULIN Although intravenous immunoglobulin has not been tested against supportive treatment alone, a Cochrane analysis of three trials indicated that such treatment was equivalent to plasma exchange.
  •  Two of these trials were combined in a meta-analysis of 398 patients, and change of disability , time to walk unaided, and proportion of patients unable to walk at one year were not significantly different between the two groups. Since these trials, intravenous immunoglobulin has become the standard treatment for the syndrome because it can be given rapidly and has fewer side effects than plasma exchange
  •  The standard regimen of 0.4 g/kg body weight each day for five consecutive days is well tolerated. This dose is set empiricaly based on clinical experience in patients with idiopathic thrombocytopenic purpura The combination of PE followed by IVIG was not significantly better than PE or IVIG alone Six daily infusion of 0.4g/kg were reported to be superior to three daily infusions in patients.(raphael et al., 2001)
  • Monitor bulbar function, BP, fvc q2-6 hr FVC <12-15 Asses clinical severity ml/kg Walks Walks with support or Mechanical unassisted bedbound ventilation Noprogression Symptoms <14 days Conservative management Plasma IVIG exchange Bradley’s neurology in clinical practice
  •  In vast majority of the patients with GBS treatment shoul be initiated as soon as possible. Each day counts:<2 weeks after the first motor symptoms. If the patient has reached plateau stage then treatment probably is no longer indicated, unless the patient has severe motor weakness and one cannot exclude the possibility that an immunological attack is going on. (motor weak ness rapidly progresses initially but cease by 4 weeks. Nadir attained by 2 weeks in 50%,3 weeks in 80% and 90% by 4 weeks) Harrison’s principles of internal medicine.
  • Side effects of iv ig Minor side effects Headache,myalgia,arthralgia,flulike symptom, Fever,vasomotor reaction Serious complication Anaphylaxis in lga defecient individuals Aseptic meningitis Congestive heart failure Thrombotic complication Transient renal failure
  • When plasma exchange is preferred over IVIG Hyperviscosity Congestive heart failure Chronic renal failure Congenital IGA defeciency
  • Corticosteroids for Guillain-Barrésyndrome Corticosteroids should not be used in the treatment of GBS Hughes RAC, van der Meché FGA. Corticosteroids for Guillain-Barré syndrome. The Cochrane Database of Systematic Reviews 2000,
  • PROGNOSIS OF GBS
  • Guillain-Barre´ SyndromeDisability Score0. HEALTHY STATE1. MINOR SYMPTOMS AND CAPABLE OF RUNNING2. ABLE TO WALK 10 M OR MORE WITHOUT ASSISTANCE BUT UNABLE TO RUN3. ABLE TO WALK 10 M ACROSS AN OPEN SPACE WITH HELP4. BEDRIDDEN OR CHAIRBOUND5. REQUIRING ASSISTED VENTILATION FOR AT LEAST PART OF THE DAY6. DEADHughes RA, Newsom-Davis JM, Perkin GD, Pierce JM. Controlled trial prednisolone in acutepolyneuropathy. Lancet 1978;2:750–753
  • The Erasmus GBS outcome scoreAge of onset >60 1 41-60 0.5 <40 0Diarrhea Absent 0 present 1GBS disability score 0 or 1 1(at 2 weeks after entry) 2 2 3 3 4 4 5 5EGOS 1-7
  •  If the EGOS is 3, the data suggest there is a <5% chance the patient won’t be walking independently at 6 months; if EGOS is 4, the chance is 7%; if EGOS is 5, the chance is 25%; if EGOS is 6, the chance is 55%; and if EGOS is 7, the chance of not walking independently at 6 months is 85%
  • Characteristics of included studies of intravenous immunoglobulin
  • Characteristics of included studies of plasma exchange
  • Characteristics of included studies ofcorticosteroids
  • Jean Baptiste Octave Landry de Thézillat