Guillain Barre syndrome

Question 1

GBS

Answer 1

-acute inflammatory demyelinating polyradiculoneuropathy

• most rapid cause of rapidly evolving motor paresis/paralysis and sensory deficits
• affects the nerve roots and peripheral nerves leading to motor neuropathy and flaccid paralysis

Question 2

GBS etiology

Answer 2

• 90% of patients will have had an illness during the past 30 days
• 2/3 report an acute infection within 2 months of the onset of GB

Question 3

guillain Barre: pathology

Answer 3

• lesions occur throughout the PNS from the spinal nerve roots to the distal termination of both motor an dsensory fibers
• there is both a demyelinating and axonal form
• the amt of sensory or motor involvement can depend on the initiating agent
• spinal roots and peripheral nerves infiltrated with macrophages and T-lymphocytes. by products further damage axon

Question 4

GBS diagnosis

Answer 4

• largely clinical- motor weakness- progressive signs and symptoms that progress rapidly in more than one extremity loss of DTRs
• weakness ceases to progress in 4 weeks
• progression distal to proximal. ranging from distal weakness to complete quadriplegia.

Question 5

GBS labs

Answer 5

• done after symptoms have been present for 1 week.
• lumbar puncture allows assessment of CSF.
• typical findings show evidence of demyelination w/o evidence of active infection

Question 6

GBS electrodiagnostic studies

Answer 6

• nerve conduction usually abnormal with slowed NCV with demyelination
• fibrillation potentials with axonal degeneration

Question 7

GBS clinical manifestation “Acute”

Answer 7

• “classic types”
• time of onset to maximal impairment is 4 weeks
• recurrent form is present in 10% of the cases, often difficult to distinguish from the chronic forn

Question 8

GBS clinical manifestation “Chronic”

Answer 8

• CIDP
• chronic demyelinating
• polyradiculoneuropathy. progresses over a period of months instead of weeks

Question 9

GBS sensory symptoms

Answer 9

• distal hyperthesias, parethesias, numbness, decreased vibratory sense and position sense. sensory distributions will have more of a stocking- and- glove pattern vs. dermatomals

Question 10

miller fisher syndrome

Answer 10

• characterized by an acute onset of:
• extraocular muscle paralysis
• sluggish pupillary reflexes
• peripheral sensory ataxia
• loss of DTR’s
• relatively spared motor function in the trunk and extremities
• facial weakness and sensory involvement of the UE may also occur

Question 11

GBS prognosis

Answer 11

• usually begins 2-4 weeks after plateau of disease
• recovery is variable, taking months to years
• many patients can make a full recovery, up to 67%
• 50% of patients may show minor neurological deficits absent tendon reflexes
• 80% will become ambulatory in 6 mo
• after 2 years 8% have not recovered

Question 12

GBS initial management

Answer 12

• monitor breathing and other body functions
• mechanical ventilation as needed
• careful blood gas monitoring. PO2 monitoring for respiratory failure. watch for confusion and/or disorientation

-aimed at controlling the response. plasmapharesis. intravenous immunoglobulin

Question 13

plasmapheresis

Answer 13

• removes the antibodies and other potentially injurious factors from the blood stream and returns the red blood cells.
• this has been shown to decreased imapirments associated with GBS
• typical tx is 4-6 echanges of 500ml per tx over the period of a week

Question 14

IVIg

Answer 14

• High dose of IV administration of immunoglobulin
• intent is to help block the damaging antibodies that may contribute to GBS and decrease inflammation
• .4/g/kg/day for 5 days