GBS UPDATE.pptx

Gbs

1. FROM PEDIATRICS WARD 23/07/2015 E.C 1 HAWASSA UNIVERSTY COMPREHESIVE SPECIALIZED HOSPITAL

2. Guillain-Barré syndrome (GBS) GROUP MEMBERS 1.NITSUKAL GULILAT 2. REDIET ASSEFA 3. MESFIN ABREHAM 4. MIHRET BIRATU 5. MISGANA TONJA 6. MEBRAT G/ yESUS 2 SEMINARY PRESENTATION ON:

3. Presentation outline Definition Ethology/cause Pathophysiology Subtypes of GBS Risk factor Clinical presentation Sign and symptoms Diagnosis Treatment Complication Prognosis Clinical summary Reference 3

4. Guillain-Barré syndrome (GBS) A collection of clinical syndromes that manifests as an acute inflammatory polyradiculoneuropathy with resultant weakness and diminished reflexes. GBS is a rare disorder in which your body's immune system attacks your nerves. Weakness and tingling in your hands and feet are usually the first symptoms. These sensations can quickly spread, eventually paralyzing your whole body. In its most severe form GBS is a medical emergency. Most people with the condition must be hospitalized to receive treatment. Sensory, autonomic, and brainstem abnormalities may also be seen. GBS is the most common cause of acute motor paralysis in children. 4

5. Ethology/cause The exact cause of Guillain-Barre syndrome is unknown. Increasing data indicate that it is an autoimmune disease , often triggered: 5 By Vaccination against the: Flu Rabies Meningitis B y a preceding viral or bacterial infection with organisms such as: Campylobacter jejuni Cytomegalovirus Epstein-Barr virus Mycoplasma pneumoniae . COVID-19, Zika virus Two-thirds of patients report symptoms of an infection in the six weeks preceding. These include a COVID-19, respiratory or a gastrointestinal infection or Zika virus.

6. Pathophysiology Two pathophysiological forms have been described: Demyelinating form of GBS Axonal forms of GBS Demyelinating form of GBS : Segmental demyelination of peripheral nerves is due to immune mediated involving both humoral and cell- mediated immune mechanisms Axonal forms of GBS axonal degeneration may occur without demyelination or inflammation. 6

7. Pathophysiology (CONT’D) The mechanism of disease possibly involves an abnormal T-cell response precipitated by an infection which activate CD4 + helper-inducer T cells. There is strong evidence that the cause is autoimmune. The immune system produces an immune response to an infection which cross-reacts with the nerves. It usually reacts with and damages the outer coating sheath of the nerve fibers, called myelin. In more severely affected people, this damage also affects the central conducting core of the nerve, called the axon. In some people the axon is itself the main target of the autoimmune response. 7

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10. Types of GBS The clinical spectrum of GBS, which includes individual variation and variable severity of presentation, comprises the following: Acute inflammatory demyelinating polyradiculoneuropathy ( AIDP ) Acute motor axonal neuropathy ( AMAN ) Acute motor and sensory axonal neuropathy ( AMSAN ) Miller-Fisher syndrome ( MFS ) Polyneuritis cranialis 10

11. 1.Acute inflammatory demyelinating polyradiculoneuropathy ( AIDP ) Accounts for 80-90% of GBS cases ( Europe and North America) Characterized by an immune-mediated attack on myelin with infiltration of lymphocytes and macrophages with segmental stripping of myelin . Motor and sensory fibres are usually affected simultaneously , producing corresponding deficits. Electrophysiology shows: Slow nerve conduction velocity Prolonged F waves. 11

12. 2.Acute motor axonal neuropathy ( AMAN ) Most commonly seen in China and Japan ( 50-60% of cases), as apposed to Western countries ( 10-20% of cases). In this form, axonal degeneration occurs by immune attack within 1-2 weeks after infection. Specific antibodies to axonal membranes of motor fibres attack the nodes of Ranvier. This, in turn, activates complement and intrusion of macrophages into periaxonal space, resulting in destruction of axons . 12

13. 2.Acute motor axonal neuropathy ( AMAN ) {CONT’D} C jejuni is the most common preceding infection, and antiganglioside antibodies are usually found in this type. Electrophysiology shows: Reduction in muscle action potentials with relatively preserved motor nerve conduction velocity Normal sensory nerve action potentials and F waves 13

14. 3.Acute motor and sensory axonal neuropathy ( AMSAN ) This type is rare and resembles AMAN except sensory nerves are also affected . This type is associated with a severe course and poor prognosis . 14

15. 4.Miller-Fisher syndrome ( MFS ) The involvement of Cranial nerves. is very distinct in this form of GBS. Ocular motor nerves ( oculomotor , trochlear, and abducens ) are affected and produce a triad of o phthalmoplegia , a taxia, and a reflexia . Electrophysiology is normal. The characteristic autoantibodies are against gangliosides GQ1b and GT1a. GQ1b plays a key role in the pathogenesis of MFS. 15

16. 5. Polyneuritis cranialis This is an acute onset of Multiple Cranial nerves. Elevated cerebrospinal fluid protein Slowed nerve conduction velocity Uncomplicated recovery. 16

17. Risk factors undercooked poultry, common cause for campylobacter infection. Influenza virus, Cytomegalovirus, Epstein-Barr virus, Zika virus, COVID-19 virus Males than females Risk increases as age increases Hepatitis A, B, C and E HIV, the virus that causes AIDS Mycoplasma pneumonia Surgery Trauma COVID-19 Johnson & Johnson and AstraZeneca vaccine 17

18. Clinical Presentation Guillain-Barre syndrome often begins with tingling and weakness starting in your feet and legs and spreading to your upper body and arms. Some people notice the first symptoms in the arms or face. As Guillain-Barre syndrome progresses, muscle weakness can turn into paralysis. Symptoms can progress over hours, days, or weeks. 18

19. Signs and symptoms of GBS may include : A pins and needles sensation in your fingers, toes, ankles or wrists Weakness in your legs that spreads to your upper body Unsteady walking or inability to walk or climb stairs Difficulty with facial movements, including speaking, chewing or swallowing Double vision or inability to move the eyes Severe pain that may feel achy, shooting or cramplike and may be worse at night Difficulty with bladder control or bowel function Rapid heart rate Low or high blood pressure Difficulty breathing 19

20. Diagnosis Guillain-Barre syndrome can be difficult to diagnose in its earliest stages. Its signs and symptoms are similar to those of other neurological disorders and may vary from person to person. Physical Examination and medical history is our major diagnosis criteria. Physical Examination Examine hands, feet or limb to check for numbness Recent history of GBS secondary to food poisoning or flu Check for reflexes Ask for duration and severity of symptoms 20

21. Diagnosis (CONT’D) The diagnosis of GBS is typically based on the presence of : Progressive ascending weakness Areflexia Findings on : Lumbar puncture Electrodiagnostic studies MRI (occasionally) Can give support for the diagnosis. Abnormalities on these studies do not develop until days to weeks after onset of symptoms. 21

22. Lumbar Puncture Typically , the LP findings are suggestive of demyelination (i.e., increased protein >45 mg/ dL within 3 weeks of onset) without evidence of active infection (lack of CSF pleocytosis ), The CSF findings may be normal within the first 48 hours of symptoms Occasionally the protein may not rise for a week. Usually by 10 days of symptoms, elevated CSF protein findings will be most prominent. Most patients have fewer than 10 leukocytes per milliliter, but occasionally a mild elevation (i.e., 10-50 cells/mL) is seen. Greater than 50 mononuclear cells/mL of CSF makes the diagnosis of GBS doubtful . 22

23. Electrodiagnostic Studies Within the first week of the onset of symptoms, electrodiagnostic studies in at least two limbs reveal the following: A d ispersed, i mpersistent , p rolonged, or a bsent O F response ( 88%) Increased distal latencies ( 75%) Conduction block ( 58% ) or temporal dispersion of compound muscle action potential ( CMAP ) Reduced conduction velocity ( 50% ) of motor and sensory nerves Criteria for axonal forms include: Lack of neurophysio l ogic evi d ence of demyelination Loss of amplitude of CMAP or sensory nerve action potentials to at least less than 80% of lower limit of normal values for age 23

24. Electrodiagnostic Studies Electromyography.   Thin-needle electrodes are inserted into the muscles to study. The electrodes measure nerve activity in the muscles. Nerve conduction studies.   Electrodes are taped to the skin above nerves. A small shock is passed through the nerve to measure the speed of nerve signals. 24

25. MRI Nearly 2 weeks after presentation of symptoms, lumbosacral MRI can show enhancement of the nerve roots with gadolinium. This imaging study has been described to be 83% sensitive for acute GBS, with nerve root enhancement present in 95% of typical cases 25

26. differential diagnosis: Spinal cord lesions may be considered in the differential diagnosis: Transverse myelitis Vascular malformations Epidural abscess Cord infarctions Tumors Cord compression Enteroviral infections of the anterior horn cells Lumbosacral disk syndromes Poliomyelitis Trauma Hopkins syndrome 26 Spinal cord lesions may be considered in the differential diagnosis: Transverse myelitis Vascular malformations Epidural abscess Cord infarctions Tumors Cord compression Enteroviral infections of the anterior horn cells Lumbosacral disk syndromes Poliomyelitis Trauma Hopkins syndrome

27. Peripheral neuropathies from the following may produce a GBS-like picture: Vincristine Glue sniffing Heavy metals poisoning Organophosphate pesticides HIV infection Diphtheria Lyme disease Inborn errors of metabolism Leigh disease Tangier disease Porphyria 27

28. Treatment In pediatrics, the most effective form of therapy is generally considered to be: intravenous immunoglobulin (IVIG) Plasmapheresis Patients in early stages of this acute disease should be admitted to the hospital for observation because the ascending paralysis can rapidly involve respiratory muscles and cause respiratory failure and autonomic instability Steroids are not effective for weakness but may help with pain. Supportive care, such as respiratory support, prevention of pressure sores, nutritional support, pain management, prevention of deep vein thrombosis, and treatment of secondary bacterial infections is important. 28

29. Intravenous immunoglobulin (IVIG) Severe or rapidly progressive muscle weakness is treated with intravenous immunoglobulin (IVIG); common protocols include: IVIG 0.4 g/kg/day for 5 consecutive days or 1g/kg/day for 2 days. IVIG is a treatment made from donated blood that contains healthy antibodies. These are given to help stop the harmful antibodies damaging your nerves. 29

30. Plasmapheresis A plasma exchange, also called plasmapheresis , is sometimes used As an alternative of IVIG. This involves being attached to a machine that removes blood from a vein and filters out the harmful antibodies that are attacking your nerves before returning the blood to your body Most people need treatment over the course of around 5 days Plasma exchange shortens the disease course and hospital stay, and reduces mortality risk and incidence of permanent paralysis. However, it may cause hypotension due to large fluid shifts, and IV access may be difficult or cause complications. Plasma exchange removes any previously administered IVIG, negating its benefits, and so should never be done during or soon after use of IVIG. 30

31. Other treatments While in hospital, you'll be closely monitored to check for any problems with your lungs, heart or other body functions. You'll also be given treatment to relieve your symptoms and reduce the risk of further problems. This may include: a breathing machine (ventilator) if you're having difficulty breathing a feeding tube if you have swallowing problem. painkillers if you're in pain being gently moved around on a regular basis to avoid  bed sores  and keep your joints healthy a thin tube called a catheter in your urethra if you have difficulty peeing laxatives if you have constipation. 31

32. Complications of GBS The most common serious complications are: Weakness of the respiratory muscles Autonomic instability 32 Other important potential complications include: Pneumonia Ileus Adult respiratory distress syndrome constipation Septicemia gastritis Pressure sores dysesthesias Pulmonary embolus Nephropathy

33. Prognosis In general, the outcome of GBS is more favourable in children than in adults the recovery period is long , often weeks to months Rarely , it can be fatal in 5-10% of patients with respiratory failure and cardiac arrhythmia Recurrence of GBS occurs in approximately 5% of cases Overall mortality rate in childhood GBS is estimated to be less than 5% Deaths are usually caused by respiratory failure , often in association with : Cardiac arrhythmias Dysautonomia 33

34. Clinical Summary Features required for diagnosis are: Progressive weakness of more than one extremity Hyporeflexia or areflexia Elevated cerebrospinal fluid protein (>45 mg/ dL ) after 1 week following onset of symptoms Slow conduction velocity or prolonged F wave on electrophysiology testing. 34

35. Features that rule out the diagnosis include: A current history of hexacarbon abuse Abnormal porphyria metabolism Recent diphtheria infection Evidence of polio, botulism, toxic neuropathy, tic paralysis, or organophosphate poisoning. 35 Clinical Summary (Cont.)

36. REFERENCE Nelson textbook of pediatrics. MSD MANUAL. https://www.mayoclinic.org/diseases-conditions/guillain-barre-syndrome/symptoms-causes/syc-20362793 36

37. 37 THANK YOU