ller-Fisher syndrome
ophthalmoplegia, areflexia, ataxia associated with anti-GQ1b ganglioside antibody
• acute axonal motor neuropathy • polyneuritis cranialis
• acute onset of peripheral and cranial nerve dysfunction • immunologic reaction directed against myelin
components of peripheral nerves • focal segmental demyelination often combined with
Sensory symptoms
– paresthesias and numbness in the distal limbs (stocking and glove distribution)
– half have pain and an aching discomfort in the muscles, mainly in hips, thighs and back.
• CSF: protein-cell dissociation • Electrophysiological evidence: slowed conduction
velocity, prolonged distal latencies and prolonged or absent F responses
Autonomic dysfunction
– sinus tachycardia, sweating dysfunction, urinary retention and constipation
Laboratory Data
CSF
• normal pressure • increased protein but normal cell (characteristic protein-cell
Differentiated Diagnosis
• acute poliomyelitis • acute myelitis • myasthenia gravis • periodic paralysis
Treatment
• Supportive treatment • Specific treatment
dissociation) 2 weeks after symptom onset
EMG
• Nerve conduction studies (NCS): slowed conduction velocity, prolonged distal latencies and
prolonged or absent F responses
axonal wallerian degeneration
Clinical manifestations
incidence is 0.6~1.9 cases per 100,000 persons per year
weakness
• symmetrical weakness from distal to proximal • trunk, intercostal, neck and cranial muscles (Ⅶ, facial
Diagnosis
• respiratory or gastrointestinal infection precedes by 1-3 weeks
• progressive symmetrical flaccid weakness and areflexia, mild sensory involvement, cranial nerve involvement, autonomic dysfunction
diplegia) maybe affected later • some patients require mechanical ventilation because of
respiratory failure • flaccid paralysis
Clinical manifestations
1, intravenous immunoglobulin (IVIg) 0.4 g/kg daily for 5 consecutive days
2, plasmapheresis 3, corticosteroids
methylprednisolone 500-1000mg IVgtt, X 5 days dexamethasone 10-15mg IVgtt, X 5 days
Guillain-Barre Syndrome (GBS)
Characteristics
• acute inflammatory demyelinating polyneuropathy (AIDP)
• Respiratory or gastrointestinal infection precedes the neuropathic symptoms by 1-3 weeks in about 60% pts.