Guillain barre syndrome

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  • American actor Andy Griffith developed Guillain-Barré syndrome in 1983. Griffith is seen here receiving an award at the White House in 2005
  • Evidence of recent C. jejuni infection was present in 30% of GBS patients compared to 8% of controls (15/50 vs. 3/40 P<0.005). Eight (47%) C. jejuni-positive patients reported symptoms of gastroenteritis 4-30 days (mean 13 days) prior to onset of GBS. Of the 15 patients with evidence of C. jejuni infection, 10 (67%) patients had axonal type of GBS. Axonal variety of GBS presented in a younger age group compared to acute inflammatory demyelinating polyradiculoneuropathy (AIDP) patients (mean age: axonal vs. AIDP: 30.11 + 13.73 vs. 40.2 + 18.77). C. jejuni-positive patients presented mainly in spring and winter and had a similar age and sex incidence as compared to the rest of the GBS patients
  • Weakness and sensory disturbance are the most common presenting symptoms. There is usually a progressive ascending motor weakness starting in the lower limbs ranging from difficulty walking to paralysis. The weakness may ascend to involve respiratory muscles and cause respiratory failure. Facial nerve palsies are common and there may be associated bulbar weakness and ophthalmoplegia.Sensory symptoms may include pain, numbness and paraesthesia. Pain commonly affects the lower back and may be severe. Numbness and paraesthesia starts distally and ascends in a similar fashion to the motor weakness in 80% of patients.
  • Urea and electrolytes are usually normal but may have evidence of the syndrome of inappropriate ADH secretion (SIADH) or renal dysfunction. ALT and gamma GT may be raised in 33% of patients. Creatine kinase may be raised.
  • Send serology for Campylobacter jejuni, Cytomegalovirus, Epstein-Barr virus, Herpes simplex virus, Mycoplasma pneumoniae. HIV antibodies should be considered. Stool cultures looking for evidence of gastrointestinal infections particularly Campylobacter jejuni.•.
  • MRI findings. Left: MRI on admission. T2 weighted image (lower) showed high intensity areas in the deep white matter in the bilateral frontal lobes, and in the cortex and subcortex in the right occipital lobe (arrows). T1 weighted image (upper) showed low intensity areas in the corresponding place. Middle: MRI one month after admission. T1 weighted image (upper) showed cavity formation in the deep white matter in the bilateral frontal lobes (arrowheads). Abnormal low intensity was recognised in the cortex and subcortex in the right occipital lobe (arrow). Fluid attenuated inversion recovery image (lower) showed high intensity areas around the cavity formations. Right: MRI one year after discharge. The size of cavity and the extent of abnormal high intensity areas were remarkably reduced.
  • Guillain barre syndrome

    1. 1. Journey To Health by Andy Griffith Manteo, North Carolina “Cindi had had frequent and major attacks of sore throat all of her life. So, shortly after our marriage we returned to Los Angeles and went to see a throat specialist, Dr. Robert Feder. He determined her sore throats were coming from her tonsils, and scheduled an operation. Early the next morning we drove over to Cedars-Sinai Medical Center and Dr. Feder took out her tonsils. While she was recuperating, I got a bad case of the flu. Not exactly a honeymoon of the rich and famous. My illness was strange. As I got better, the symptoms of influenza were replaced by pain--terrible, searing pain that ricocheted through my entire body. Cindi and I joked about our invalid status and settled in that Saturday to watch the Kentucky Derby on television. But after the race, when I stood up and took a few steps, I pitched headlong into a nightmare. I was overcome by pain so encompassing that I couldn't feel my feet. I had no control over them, and fell to the floor in agony.
    2. 2. We couldn't reach any of our doctors that weekend. Yet I was so desperate for relief from the pain that I took some of the codeine prescribed for Cindi for her throat. It barely made a difference. On Monday my doctor met us at a local hospital. There a roomful of doctors attempted to find out what was wrong. For four days they hadn't a clue. Finally they did a spinal tap. When the results came in, one mystery was solved. I had Guillain-Barre syndrome, a rare form of nerve inflammation. It's thought to be caused by an allergic reaction to a viral infection, such as the flu. The nerves become inflamed and begin to send erroneous and scrambled messages to the brain. In some people it causes little pain but extensive paralysis; in cases such as mine, it causes little paralysis but intense pain. There are no drugs or surgery to treat Guillain-Barre--so the doctor sent me home. "There is nothing we can do," he said. "You've got to ride it out. I'll prescribe some pain medication, but use as little as possible. Come back in a week."
    3. 3. Introduction Guillain-Barré syndrome (GBS) is an acute, frequently severe, and fulminant polyradiculoneuropathy that is autoimmune in nature. collection of clinical syndromes that manifests as an acute inflammatory polyradiculoneuropathy with resultant weakness and diminished reflexes.
    4. 4. 1 to 2 per 100,000 population. Males are at 1.5-fold higher risk for GBS than females, in western countries adults are more frequently affected than children. With poliomyelitis under control in developed countries, GBS is now the most important cause of acute flaccid paralysis
    5. 5. History The French physician Jean Landry first described the disorder in 1859. In 1916, Georges Guillain, Jean Alexandre Barré, and André Strohl diagnosed two soldiers with the illness and described the key diagnostic abnormality of increased spinal fluid protein production, but normal cell count.
    6. 6. Epidemiology Worldwide, the annual incidence is about 0.6–4 occurrences per 100,000 people. Men are one and a half times more likely to be affected than women. The incidence increases with age; there are approximately 1 cases per 100,000 people aged below 30 years and about 4 cases per 100,000 in those older than 75 years. The incidence of GBS during pregnancy is 1.7 cases per 100,000 of the population. Congenital and neonatal Guillain–Barré syndrome have also been reported
    7. 7. GBS is also known as acute idiopathic polyradiculoneuritis, acute idiopathic polyneuritis, French polio, Landry's ascending paralysis and Landry Guillain Barré syndrome.
    8. 8. Related anatomy and physiology
    9. 9. Pathophysiology Guillain-Barré is the result of a cell-mediated immune attack on peripheral nerve myelin proteins. The best-accepted theory is that an infectious organism contains an amino acid that mimics the peripheral nerve myelin protein. The immune system cannot distinguish between the two proteins and attacks and destroys peripheral nerve myelin. the ganglioside GM1b, is the most likely target of the immune attack. With the autoimmune attack there is an influx of macrophages and other immune-mediated agents that attack myelin, cause inflammation and destruction, and leave the axon unable to support nerve conduction.
    10. 10. Research Input Campylobacter jejuni infection in Guillain-Barré syndrome: A prospective case control study in a tertiary care hospital A Sharma1, V Lal1, M Modi1, C Vaishnavi2, S Prabhakar1 Background: This study was carried out to determine the relationship between C. jejuni infection and GBS in an Indian setting. Materials and Methods: This prospective study was carried out on a cohort of 50 patients with GBS who were treated in a tertiary care hospital in India. Based on electrophysiological findings the patients were divided into various subtypes. Serology for C. jejuni (Immunoglogulin G, IgG and Immunoglogulin, IgM) using ELISA was done both in patients and 40 age, sex and geographically matched controls.
    11. 11. Conclusions Preceding C. jejuni infection is common among GBS patients and is often associated with the axonal variety of GBS. Axonal variety of GBS generally presents in a younger age group as compared to AIDP.
    12. 12. SIGNS & SYMPTOMS Areflexic motor paralysis with or without sensory disturbance. Bulbar weakness Pain Deep tendon reflexes attenuate or disappear Cutaneous sensory deficits Bladder dysfunction
    13. 13. Subtypes • 1.Acute inflammatory demyelinating polyradiculoneuropathyAIDP • 2.Acute motor axonal neuropathyAMAN • 3.Acute motor and sensory axonal neuropathyAMSAN MFS 4.Miller fisher syndrome
    14. 14. 1.Acute inflammatory demyelinating polyradiculoneuropathy (AIDP) is the most common form and accounts for around 85– 90% of cases. The clinical features are of symmetrical ascending motor weakness with hypo- or areflexia. The underlying pathological process involves inflammation and destruction of the myelin sheaths surrounding peripheral nerve axons by activated macrophages. This leads to slowing and blockage of conduction within peripheral nerves causing muscle weakness. Severe cases may develop secondary axonal damage
    15. 15. Acute motor axonal neuropathy (AMAN) is more common in Japan and China, amongst young people and in the summer months. It has an association with precedent infection with Campylobacter jejuni. Clinical features are similar to AIDP but tendon reflexes may be preserved. Electrophysiological testing may distinguish from other variants as selective motor nerve and axonal involvement is demonstrated. In AMAN the pathological process involves binding of antibodies to ganglioside antigens on the axon cell membrane, macrophage invasion, inflammation and axonal damage.
    16. 16. Acute motor and sensory axonal neuropathy (AMSAN) is a variant of GBS in which both motor and sensory fibres are involved and which can be demonstrated on electrophysiological studies. It is more severe and associated with prolonged or even partial recovery. Clinical features are similar to AMAN but also involve sensory symptoms. The underlying pathological process is similar to that for AMAN (i.e. antibody mediated axonal damage).
    17. 17. Miller Fisher syndrome (MFS) presents with ataxia, areflexia and ophthalmoplegia. 25% of patients may develop limb weakness. Electrophysiological studies show primarily sensory conduction failure. Antiganglioside antibodies to GQ1b are found in 90% of patients and are associated with ophthalmoplegia . There have been limited pathological studies in MFS but demyelination of nerve roots has been demonstrated.
    18. 18. INVESTIGATIONS Serum biochemistry:evidence of SIADH or renal dysfunction Inflammatory markers :ESR is usually raised and CRP is sometimes
    19. 19. INVESTIGATIONS Antiganglioside antibodies : Anti-GM1 • It is positive in 25% of pts and is a w worse outcome Anti-GD1a • AMAN subtype of GBS Anti-GQ1b • Miller- Fisher syndrome
    20. 20. INVESTIGATIONS Infection screen : Campylobacter jejuni, Cytomegalovirus, Epstein-Barr virus, Herpes simplex virus, Mycoplasma pneumoniae. HIV antibodies
    21. 21. INVESTIGATIONS Radiological: A CT brain is indicated to exclude other causes of symptoms and evidence of raised intracranial pressure prior to performing a lumbar puncture. An MRI of the spine may show selective anterior spinal nerve root enhancement with gadolinium and will exclude cervical nerve impingement
    22. 22. INVESTIGATIONS
    23. 23. INVESTIGATIONS Lumbar puncture: Cell count and glucose are usually normal with a raised protein, although the latter may also be normal in first two weeks.
    24. 24. INVESTIGATIONS Nerve conduction studies: – Findings depend on subtype of GBS. – The majority show demyelinating pattern while – some patients may show evidence of axonal loss with little or no demyelination.
    25. 25. INVESTIGATIONS Respiratory function tests: – These may show reduced vital capacity, maximal inspiratory and expiratory pressures. – Arterial blood gases may indicate progressive respiratory failure.
    26. 26. DIFFERENTIAL DIAGNOSIS Neurological – Myasthenia gravis – Eaton-Lambert (myasthenic) syndrome – Multiple sclerosis – Transverse myelitis
    27. 27. DIFFERENTIAL DIAGNOSIS Metabolic – Hypokalaemic periodic paralysis – Hypermagnesaemia – Hypophosphataemia – Acute intermittent porphyria
    28. 28. DIFFERENTIAL DIAGNOSIS Infective – Post diphtheria neuropathy – Polio – Botulism – Tick paralysis
    29. 29. DIFFERENTIAL DIAGNOSIS Drugs / toxins – Heavy metal poisoning (e.g. lead) – Biological toxins (including snake and scorpion toxins) – Drugs (including stavudine, nitrofurantoin and aminoglycosides)
    30. 30. DIFFERENTIAL DIAGNOSIS Other – Acute polymyositis – Critical illness myopathy
    31. 31. MANAGEMENT Supportive Immunomodulatory
    32. 32. Supportive Airway and respiratory Cardiovascular Gastrointestinal Neurological Psychological Rehabilitation
    33. 33. Immunomodulatory Intravenous immunoglobulin – most effective if administered within two weeks of the onset of symptoms – Indications for IVIg include • muscle weakness and • respiratory depression
    34. 34. IVIG IVIg contains pooled donor IgG antibodies and may reduce the severity of autoimmune inflammation in GBS by blocking Fc receptors. This prevents the Fc portion of antibodies binding and thus interrupts antibody mediated cell destruction. Complement activation is also altered.
    35. 35. Plasma exchange The aim of plasma exchange is to remove antibodies associated with the underlying autoimmune response. passage of blood through an extracorporeal cell separator. The plasma fraction of the blood is removed then and replaced with FFP or human albumin solution. Anticoagulants are administered during the procedure.
    36. 36. Recommendations for the treatment of GBS: 1)plasma exchange (PE) is recommended for nonambulant adult patients with GBS who seek treatment within 4 weeks of the onset of neuropathic symptoms.PE should also be considered for ambulant patients examined within 2 weeks of the onset of neuropathic symptoms. 2) IV IG is recommended for nonambulant adult patients with GBS within 2 or possibly 4 weeks of the onset of neuropathic symptoms; 3) Corticosteroids are not recommended for the management of GBS. 4) sequential treatment with PE followed by IV IG or immunoabsorption followed IV IG is not recommended for patients with GBS ;and 5) PE and IV IG are treatment options for children with severe GBS
    37. 37. Recommendations For Rx 1)plasma exchange (PE) is recommended for nonambulant adult patients with GBS who seek treatment within 4 weeks of the onset of neuropathic symptoms.PE should also be considered for ambulant patients examined within 2 weeks of the onset of neuropathic symptoms. 2) IV IG is recommended for nonambulant adult patients with GBS within 2 or possibly 4 weeks of the onset of neuropathic symptoms; 3) Corticosteroids are not recommended for the management of GBS. 4) sequential treatment with PE followed by IV IG or immunoabsorption followed IV IG is not recommended for patients with GBS ;and 5) PE and IV IG are treatment options for children with severe GBS
    38. 38. PROGNOSIS Most patients with GBS recover fully but this may take many months of intensive therapy. 15% of patients suffer persistent disability. 10% are unable to walk unaided at one year. There may be a recurrence in 2–5% of cases. The mortality from GBS ranges from 2–12%.
    39. 39. Prognosis Common causes of death include venous thromboembolism, pneumonias, arrhythmias and complications related to dysautonomia. Markers of poor prognosis include – age > 40 years, – rapid onset of symptoms, – severe weakness(especially if mechanically ventilation is required or there is marked upper limb weakness), – association with precedent diarrhoeal illness or campylobacter infection, – evidence of axonal damage on electrophysiological studies and – lack of treatment with either plasma exchange or IVIg.
    40. 40. Nursing Management Assessment: Acute onset (hours to weeks) of progressive, usually ascending muscle weakness and fasciculation, possibly leading to paralysis (maximal weakness is reached within 2 weeks). Paresthesia and painful sensations. Possible hypoventilation due to chest muscle weakness. Difficulty with swallowing, chewing, speech, and gag, indicating fifth (trigeminal) and ninth (glossopharyngeal) cranial nerve movement. Reduce or absent deep tendon reflexes, position and vibratory perception.
    41. 41. Assessment: Autonomic dysfunction with orthostatic hypotension and tachycardia. Check for previous history of viral illness or surgical procedure Urinary incontinence Check for facial nerve paralysis. Inspect the patient’s face at rest and during conversation.Assess for any problems during talking,swallowing,and chewing. Assess for any change in the vital signs
    42. 42. Maintaining respiratory function incentive spirometry and chest physiotherapy Mechanical ventilation Close monitoring Suctioning
    43. 43. Enhancing physical mobility paralyzed extremities are supported in functional positions, and passive range-of-motion exercises are performed at least twice daily Range-of-motion exercises, altering positioning, anticoagulation, thigh-high elastic compression stockings or sequential compression boots, and adequate hydration
    44. 44. Providing adequate nutrition IV fluids and parenteral nutrition monitors for the return of bowel sounds gastrostomy tube assesses the return of the gag reflex and bowel sounds before resuming oral nutrition.
    45. 45. Improving communication picture cards or an eye blink system Collaboration with the speech therapist
    46. 46. Decreasing fear and anxiety participate in physical care providing information about the condition, emphasizing a positive appraisal of coping resources, teaching relaxation exercises and distraction techniques Encouraging visitors, engaging visitors or volunteers to read to the patient, listening to music or books on tape, and watching television
    47. 47. Monitoring and managing potential complications Thorough assessment of respiratory function at regular intervals cardiac dysrhythmias, which necessitate ECG monitoring, transient hypertension, orthostatic hypotension, DVT, pulmonary embolism, urinary retention, and other threats to any immobilized and paralyzed patient. These require monitoring and attention to prevent them and prompt treatment if indicated.

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