1. ACUTE FLACCID PARALYSIS Dr Shreedhar Paudel May, 2009

2. DEFINITION
Sudden onset of weakness or paralysis over a period of 15 days in a patient less 15 years of age

3. INVOLVEMENT Anterior horn cells Nerve fibres Poliomyelitis
Acute transverse myelitis
Nerve fibres
Postinfectious polyneuropathy (GBS syndrome)
Toxins----diphtheria, porphyria

4. INVOLVEMENT Neuromuscular junction Metabolic causes Muscular disease
Tick toxin
Botulinum toxin
Metabolic causes
Periodic paralysis
Muscular disease
Myositis

5. GUILLIAN BARRE SYNDROME
DEFINITION
It is an idiopathic, acquired, postinfectious polyneuropathy
EPIDEMIOLOGY
Annual incidence is 0.4 to 1.7/
Male > female
Any age however rare before one year

6. PATHOPHYSIOLOGY Immune mediated disease
Follows a viral respiratory tract infection or a gastrointestinal infection
Bacteria share antigenic sites with axons & peripheral nerve myelin sheaths or both
Inflammation and demyelination with infiltration of macrophages which penetrate the basement membrane of schwann cells exposing the axons and causing focal conduction block

7. PATHOPHYSIOLOGY
Inflammation causes leakage of proteins into the CSF causing raised CSF proteins without pleocytosis
Can involve the peripheral nerves, cranial nerves, dorsal roots, dorsal root ganglia & sympathetic chain

8. EITIOLOGY Mycoplasma Hepatitis B CMV EBV Measles Mumps Echovirus
Cocksakie virus
Influenza virus
Campylobacter jejuni

9. CLINICAL FEATURES Appear within 2 wks after onset of viral infections
C/O numbness or parasthesias in the hand and feet followed by a heavy weak feeling in the legs
Followed by inability to walk
The weakness is symmetrical beginning in the legs and ascending to involve the arms, trunk, throat and face
Progression can occur rapidly in hours or days or more slowly over weeks

10. SIGNS Marked flaccidity ---- hypotonia Complete areflexia
Minimal signs of sensory loss
Progress to bulbar signs and respiratory insufficiency
Cranial nerve involvement
Autonomic dysfunction --- hypotension, hypertension, orthostatic hypotension, tachycardia, urinary retention or incontinence, stool retention, episodes of abnormal sweating, flushing, vasoconstriction
Intact sensations

11. CLINICAL VARIANTS Polyneuritis cranialis Miller fisher syndrome
Cranial nerve involvement
Miller fisher syndrome
Ophthalmoplegia, ataxia, areflexia
Chronic progressive GBS
Symptoms persisting more than 6 weeks
Chronic relapsing GBS

12. DIAGNOSIS Clinical Nerve conduction studies
slowing of nerve conduction
Electromyogram
extensive fibrillation showing denervation
CSF
increased proteins with normal leukocytes during 2nd week of illness

13. MANAGEMENT SUPPORTIVE Close monitoring of vital signs Nursing care
Repeated spirometries
Bowel and bladder care
Tube feeding
Care for bed sores
Ventilatory support if required

14. MANAGEMENT SPECIFIC Steroids-----2mg/kg for 2 weeks
Plasmapheresis-----to remove antibodies
I/V immunoglobulins mg/kg for 5 days or 1gm/kg for 2 days

15. PROGNOSIS Mortality 3% Recovery 1 to 6 months, may take 12 months
Delayed recovery may be followed by permanenent neurological sequelea

16. D/D of acute flaccid paralysis

17. GB Syndrome
Spinal cord syndrome
poliomyelitis
Eitiology
Delayed hypersesitivity.
antibody mediated
Trasverse myelitis,spinal cord abscess,TB
Poliovirus type I,II,III
History
GI or URTI, 5 to 14days preceding symptoms
Rapid progression of symptoms
Unimmunized, URTI or GI infection
Symptoms
Symmetric weakness in LL gradually ascending with parasthesias. normal bowel & bladder function
Back pain. Sensory loss below the level of lesion, sphincter problems
Fever,. meningism, muscle tenderness, asymmetric weakness

18. Signs
Symmetric flaccid weakness, sensations intact, gradually ascending
Symmetric areflexia,sensory loss below the level of lesion., pain, bowel and bladder dysfunction
Assymmetric flaccid weakness, sensations intact, muscle wasting
CSF findings
Normal cell count with raised protiens 2nd week of illness
Pleocytosis with raised protiens
EMG/NCS
Signs of denervation, NCS shows delayed conduction
Normal initially
NCS normal, EMG denervation later
Course and prognosis
Recovery in majority within 12 months
Depends on eitiology
Permanent disability in 1% cases