Case presentation 4 14


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Case presentation 4 14

  1. 1. Guillain-Barré syndrome <ul><li>Epidemiology </li></ul><ul><li>Most common cause of acute flaccid paralysis in Western countries </li></ul><ul><li>Overall incidence 1-2/100,000; a little higher in elderly population </li></ul><ul><li>All age groups can be affected </li></ul><ul><li>Bimodal peak, small peak in young adults and larger peak in elderly; rare in infancy </li></ul><ul><li>75% have an antecedent ‘event’ 1-4 weeks before onset of weakness. respiratory (68%), GI (22%), resp and GI (10%), surgery (2%), vaccination or pregnancy </li></ul><ul><li>Associated organisms: CMV, EBV, VZV, HIV, C. jejuni , M. pneumoniae , Shigella </li></ul>
  2. 2. Guillain-Barré basics <ul><li>peripheral neuropathy that causes acute neuromuscular failure </li></ul><ul><li>5-10% of patients die from respiratory failure, pulmonary emboli, or infection </li></ul><ul><li>Structural similarities between a triggering infectious organism and peripheral nerve tissue are important in its pathogenesis </li></ul><ul><li>Treatment consists of rapid administration of intravenous immunoglobulin or plasma exchange, which shortens the time to recovery </li></ul><ul><li>Peak symptoms usually 2-4 weeks after onset. </li></ul><ul><li>20-30% have residual symptoms at 1 yr </li></ul>
  3. 3. Copyright ©2008 BMJ Publishing Group Ltd. Winer, J. B BMJ 2008;337:a671 Fig 1 Structural similarities between ganglioside GM1 in nerve cell membranes and a Campylobacter jejuni lipopolysaccharide. Adapted, with permission, from a review by Ang27
  4. 4. Diagnostic criteria for ‘typical’ GBS <ul><li>Required features: </li></ul><ul><li>Progressive weakness in both arms and legs </li></ul><ul><li>Areflexia (or hyporeflexia) “typical GBS” </li></ul><ul><li>Features supportive of diagnosis </li></ul><ul><li>Progression of symptoms over days to 4 weeks </li></ul><ul><li>Relatively symmetric </li></ul><ul><li>Mild sensory signs or symptoms </li></ul><ul><li>CN involvement, especially bilateral facial weakness </li></ul><ul><li>Recovery begins 2-4 weeks after progression ceases </li></ul><ul><li>Autonomic dysfunction </li></ul><ul><li>Absence of fever at onset </li></ul><ul><li>Typical CSF and EMG/NCS features </li></ul>
  5. 5. CSF <ul><li>Typical profile: elevated protein (may take 1-2 wks. to develop) with normal to mild increase (<10) lymphocytes ( albumino-cytologic dissociation ) </li></ul><ul><li>May be normal early on and 10% may have normal profile throughout course of disease </li></ul>
  6. 6. Recurrent Guillain-Barre Syndrome <ul><li>Guillain Barre: 0.75 to 2 per 100 000 patients </li></ul><ul><li>Recurrence: 2-5%? </li></ul><ul><li>Varying presentations: sometimes pure sensory </li></ul>
  7. 8. Guillain-Barre and influenza vaccine <ul><li>1976: outbreak of swine flu among military personnel at Fort Dix, NJ </li></ul><ul><li>nationwide National Influenza Immunization Program activated: 45 million vaccinated in 10 weeks </li></ul><ul><li>Unusual amount of side effects, including GBS reported </li></ul><ul><li>vaccine stopped, CDC director eventually fired, public distrust </li></ul><ul><li>Q: Is GBS more common after influenza, or influenza vaccine administration? </li></ul>
  8. 9. Take home points (4) <ul><li>1. Guillain-Barré syndrome should be considered in any patient developing rapidly progressive limb weakness </li></ul><ul><li>2. Absent reflexes are a “red flag” for Guillain-Barré syndrome in patients with rapidly progressive weakness </li></ul>
  9. 10. Take home points, cont. <ul><li>3. Patients with suspected Guillain-Barré syndrome should be referred to hospital as an emergency </li></ul><ul><li>4. A history of weakness preceded by respiratory or gastrointestinal tract infection suggests Guillain-Barré syndrome </li></ul>
  10. 11. sources <ul><li>Winer, J. Guillain-Barre Syndrome. BMJ 2008;337:a671, doi:10.1136/bmj.a671 </li></ul><ul><li>Kuitwaard, K. Recurrent Guillain–Barré syndrome. J Neurol Neurosurg Psychiatry 2009;80:56-59 doi:10.1136/jnnp.2008.156463 </li></ul><ul><li>Lewis, M. Lou Gehrig, Rawhide , and 1938. Neurology 2007;68:615-618 </li></ul><ul><li>Poncelet AN et al. An algorithm for the evaluation of peripheral neuropathy Am Fam Physician. (1998) </li></ul>