2. • is an acute polyneuropathy, a disorder
affecting the peripheral nervous system.
• Ascending paralysis, weakness beginning
in the feet and hands and migrating
towards the trunk
• also can cause cranial nerve dysfunction
3. Epidemiology
• Worldwide, the annual incidence is about
0.6–1.9 occurrences per 100,000 people
• Men are one and a half times more likely
to be affected than women
• the incidence increasees in patients with
Hodgkin disease,pregnancy or general
surgery
4. Etiology
• autoimmune response
-The targets of such immune attack are
thought to be gangliosides, compounds
naturally present in large quantities in
human peripheral nerve tissues
- infections by bacterium Campylobacter
jejun, cytomegalovirus, influenza virus,
6. Variants
• acute motor axonal neuropathy
- motor axonal degeneration with little or
no demyelination or imflammation
- may follow infections with
Campylobacter jejuni or parenteral
injection of gangliosides
• the Miller-Fisher syndrome
- characterized by gait ataxia, areflexia
and ophthalmoparesis; papillary
abnormalities are sometimes present
7. • acute motor and sensory axonal
neuropathy or neuronopathy
• acute autonomic neuropathy or
pandysautonomia
8. Diagnostic criteria
• features required for diagnosis
- progressive motor weakness of more
than 1 limb [ from minimum weakness to
+/- ataxia to paralysis
- areflexia [usually universal but distal
areflexia with difinite hyporeflexia of
biceps and knee jerks sufficess if other
features consistent]
9. Clinical features
• progression motor weakness peaks at
2wks in 50% , 3wks 80% , 4wks > 90%
• relative symmetry
• mild sensory symptoms/signs eg mild
paresthesias in hands or feets
• cranial nerve involvment ; facial
weakness , oropharyngeal muscles may
be affected
• recovery usually 2-4wks after progression
stops , may be delayed for months
10. • autonomic dysfunction [ may flactuate]
tachycardia, arrythmias, postural hypotension ,
HTN, vasomotor symptoms
• afebrile at onset of neuritic symptoms
• fever,
• severe sensory loss with pain
• progression >4wks
• cessation of progression without recovery
• sphincter dysfunction eg; bladder paralysis
11. CSF findings
• protein > 55mg/dl
• cells <10 mononuclear leukocytes/ml
• variants
- no protein rise
- 11-50 monocytes/ml
12. differential diagnosis
• acute polyneuropathy
• acute polyneuropathy after administration
of penicillin
• infections affecting the anterior horn
ganglion cells
• myasthenia gravis
• acute myopathies
• albumincytologic dissociation of the CSF
due to another disease
• Acquired immunodeficiency syndrome
13. Treatment
The patient needs to be in a unit where
intensive respiratory care is available.
Respiratory assistance should be
instituted at the first sign of dyspnea
(arterial PO2< 70 mmHg) or markedly
reduced vital capacity (<12 to 15 mL/kg)
• early plasaphaeresis hastens recovery
and reduces the residual deficit
• immunoglobulins may be helpful in severe
cases
14. Prognosis
• recovery may not be complete for several
months
• 35% of untreated patients have residual
weakness and atrophy
• reccurance after maximum recovery
occours in 2%
