Guillain-Barré Syndrome

Introduction In 1859 Landry published a report on 10 patients with an ascending paralysis 1916 French physicians (Guillain, Barré, and Strohl) they described 2 French soldiers with motor weakness, areflexia, and albuminocytological dissociation in the CSF Later named Guillain-Barré syndrome (GBS)

Description An acute, predominantly motor neuropathy of uncertain cause that is the most common cause of acquired generalized paralysis in humans Usually ascending, symetric weakness Paresthesias are more variable

Epidemiology Incidence / Prevalence Race Age Sex Annual incidence 1-2 / 100.000 Race All race affected Age All ages : double peak distribution (15-35 y) and (50-75) y Sex Male : Female = 1,5 : 1

Etiology Precise cause is unclear but follows infection in most cases. Infections produce immune reaction resulting in humoral & cellular responses that attack unknown mielin components and result in macrophage induced demyelination Sporadic disease : some association with HLA types Mirip aoutoimun yang dipicu oleh infeksi, misalnya strptokokous  GNC ec strptokok atau RHD ec strptokok

Risk Factors Infection C. jejuni infection is the most common precipitating infection occuring in 30-40% cases.  GE that precedes weakness by 7 -14 days Other infection includes infuenza viruses, EBV, CMV, HIV, Coxsackie virus, herpes simplex, hepatitis A, mycoplasma pneumonia Hematologic malignancy, hyperthyroidism, collagen vascular diseases, immunization (swine flu), pregnancy.

Signs and Symptoms Begin with numbness and tingling in fingers, toes or trunk that may last 7 – 10 days Symmetric weakness follows usually starting in legs and going to arms (ascending pattern). Facial 50% Extent of progression variable  30% ventilator assistance

Signs and Symptoms Tendon reflexes typically absent or depressed Sensory loss variable Dull, aching, burning pain involving low back or lower extremities occur in 90% cases Autonomic involements (BP, bowel & bladder, pupilary changes, cardiac arrytmias)

Variants of clinical features Fisher syndrome (5%) Opthalmoplegia, ataxia, arefleksia, often without weakness Pharygeal-cervical-brachial weakness (3%) Weakness in the pharynx, face, neck fleksors and arms Paraparetic weakness (~2%) Only leg weakness and areflexia with variable sensory loss

Variants of clinical features Pure motor variant (~3%) Have no sensory symptoms Axonal variant Rapid progressive weakness & sensory loss with early respiratory insufficiency (predominantly have had preceding C. jejuni infection)

Variants of clinical features Pure sensory variant (<1%) Ataxic, aferlexic sensory neuropathy with little or no motor involvement, tremor and autonomic features Acute pandysautonomic varian (rare) Presents with GI distrubances, orthostatis, urinary retention, fatique, impotence, diminished sweating and salvation.

Laboratory Procedures Spinal fluid analysis Elevated protein without leucocytosis (<10 cells/mm3) Anti-GM1 antibody  serologic testing for C. jejuni Anti-GQ1b antibodies  confirming Fisher syndrome Do not change the therapi but may indicate worst prognosis if positive

Management Prehospital Care ABCs, IV, oxygen, and assisted ventilation may be indicated. Monitor for cardiac arrhythmias. Transport expeditiously

Management Emergency Department Care ABCs, IV, oxygen, and assisted ventilation may be indicated. Intubation  respiratory failure. (FVC is less than 15 mL/kg) Patients who have, or are suspected of having, GBS should be monitored closely for changes in blood pressure, heart rate, and other arrhythmias. Treatment rarely is needed for tachycardia. Atropine  for symptomatic bradycardia. Hypotension of dysautonomia usually responds to intravenous fluids and supine positioning. Temporary pacing  with 2nd degree and 3rd degree heart block. Emergency Department Care ABCs, IV, oxygen, and assisted ventilation may be indicated. Intubation should be performed on patients who develop any degree of respiratory failure. Clinical indicators of the need for intubation include hypoxia, rapidly declining respiratory function, poor or weak cough, and suspected aspiration. Typically, intubation is indicated when the FVC is less than 15 mL/kg.24 Patients who have, or are suspected of having, GBS should be monitored closely for changes in blood pressure, heart rate, and other arrhythmias. Treatment rarely is needed for tachycardia. Atropine is recommended for symptomatic bradycardia. Because of the lability of dysautonomia, hypertension is best treated with short-acting agents, such as a short-acting beta-blocker or nitroprusside. Hypotension of dysautonomia usually responds to intravenous fluids and supine positioning. Temporary pacing may be required for patients with second-degree and third-degree heart block.

Management Only plasma exchange (PE) therapy and intravenous immune serum globulin (IVIG) have proven effective Plasma exchange (200 – 250 cc/kg BW) total exchange volume divided into 4-6 x over 2 – 3 weeks Intravenous immune globulin (IVIG) Dose 0.4 g/kg/d IV for 5 d Randomized trials in severe disease show that IVIG started within 4 weeks from onset hastens recovery as much as plasma exchange.26,27,28,29  Combining plasma exchange and IVIG neither improved outcomes nor shortened the duration of illness.30  IVIG has also been proven safe and effective in the treatment of pediatric GBS.30,31  Additionally, IVIG is the preferential treatment in hemodynamically unstable patients and in those unable to ambulate independently.32,30  

Management Enoxaparin Enoxaparin for prophylaxis of venous thromboembolism. Average duration of treatment is 7-14 d. <2 months: 0.75 mg/kg/dose bid SC >2 months to 18 years: 0.5 mg/kg/dose bid SC Randomized trials in severe disease show that IVIG started within 4 weeks from onset hastens recovery as much as plasma exchange.26,27,28,29  Combining plasma exchange and IVIG neither improved outcomes nor shortened the duration of illness.30  IVIG has also been proven safe and effective in the treatment of pediatric GBS.30,31  Additionally, IVIG is the preferential treatment in hemodynamically unstable patients and in those unable to ambulate independently.32,30  

Complications Persistent paralysis Respiratory failure, mechanical ventilation Hypotension or hypertension Thromboembolism, pneumonia, skin breakdown Cardiac arrhythmia Ileus Aspiration Urinary retention Psychiatric problems such as depression and anxiety

Prognosis Poor prognosis Rapid progression of symptoms, Advanced age, Prolonged ventilation (>1 mo) A longer-lasting increase in IgM anti-GM1 predicts slow recovery.

Prognosis Neurologic sequelae Reported incidence of permanent neurologic sequelae ranges from 10-40%. The worst-case scenario is tetraplegia within 24 hours with incomplete recovery after 18 months or longer. The best-case scenario is mild difficulty walking, with recovery within weeks. The usual scenario is peak weakness in 10-14 days with recovery in weeks to months. Average time on a ventilator (without treatment) is 50 days.

Prognosis Mortality Most is due to severe autonomic instability or from the complications of prolonged intubation and paralysis.39,40,41,23 Mortality rates range from 5-10%.

Conclusion GBS is an acute inflammatory demyelinating polyneuropathy characterized by progressive symmetric ascending muscle weakness, paralysis, and hyporeflexia with or without sensory or autonomic symptoms GBS is believed to result from autoimmune humoral- and cell-mediated responses to a recent infection or any of a long list of medical problems.

Conclusion Symptoms could be motor dysfunction, sensory dysfunction, and/or autonomic dysfunction Treatment are ABCs, IV, oxygen, and assisted ventilation, plasma exchange, IVIG.

Conclusion Poor prognosis is associated with rapid progression of symptoms, advanced age, prolonged ventilation (>1 month) Mortality rates range from 5-10%

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