- Post infectious polyneuropathic syndrome mainly affecting motor but may also affect sensory nerves.
- Affect all ages.
- It is a demyelinating neuropathy
- Usually post viral infection (10 days).
- GI (Campylobacter jejuni) and respiratory (Mycoplasma pneumoniae) symptoms of original infection.
- Landry ascending paralysis: paralysis beginning from lower extremities and progressing upwards to trunk, upper limbs and bulbar muscles (face, neck, deglutition).
- 91% symmetric paralysis.
- Progress of paralysis is gradual (days or weeks)
- Myalgia in initial stages > weakness > flaccid tetraplegia
- Sometimes paresthesis
- Sometimes symptoms of viral meningitis or meniningoencephalitis.
- Miller-Fisher Syndrome: is a variant of GBS. It is a descending paralysis with paralysis beginning external opthalmoplegia, ataxia, areflexia, sometimes papilledema, without significant motor or sensory deficit in limbs.
- Tendon reflexes are lost and are usually the last to return.
- If the autonomic NS is affected, BP, cardiac rate, postural hypotension, bradycardia and occasional asystole can occur. Temporary pacemaker may be required.
- CSF: *essential; CSF protein is elevated > 2x, glucose is normal, no pleocytosis (leucocytes in CSF) (<10/ul)
- Motor nerve conduction: greatly reduced conduction velocity in motor nerves and slow in sensory nerves.
- EMG shows acute denervation of muscles.
- Creatine Phospokinase (CPK/CK): mildly elevated
- Antiganglioside antibodies GM! and GD1 are elevated in serum
- Muscle biopsy (not required): denervation atrophy in chronic GBS.
- Sural nerve biopsy (nerve below the calf): shows segmental demyelination, focal inflammation, Wallerian degeneration (axon degeneration observed when a nerve is cut)
- Stool cultures are rarely positive as GBS is post infectious.
- Immediate admission for observation because of risk of respiratory muscle paralysis.
- Immunoglobulin I.V.: Octagam 0.4g/kg/day for 3-7 days; caution in renal insufficiency
- Plasmapheresis (blood serum replacement), steroids (methylprednisolone I.V. 30ug/kg) and/or immunosuppressants if IVIG is ineffective.
- IVIG + Interferon is effective in some patients
- Supportive therapy (artificial ventilation and prevention of decibitus)
- Treatment of bacterial infection
- Spontaneous full recovery in 2-3 weeks usually
- Tendon reflexes are last to return
- Recovery from paralysis in reverse direction of onset
- Sequelae determined by: cranial nerve involvement, intubation, maximum disability at presentation
- Relapse present in 7% of children
