ACUTE FLACCID PARALYSIS Dr Shreedhar Paudel May, 2009

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

INVOLVEMENT
Anterior horn cells
Poliomyelitis Acute transverse myelitis

Nerve fibres
Postinfectious polyneuropathy (GBS syndrome) Toxins----diphtheria, porphyria

INVOLVEMENT
Neuromuscular junction
Tick toxin Botulinum toxin

Metabolic causes
Periodic paralysis

Muscular disease
Myositis

GUILLIAN BARRE SYNDROME

DEFINITION It is an idiopathic, acquired, postinfectious
polyneuropathy

EPIDEMIOLOGY
Annual incidence is 0.4 to 1.7/ 100000 Male > female Any age however rare before one year

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

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

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

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

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

CLINICAL VARIANTS
Polyneuritis cranialis
Cranial nerve involvement

Miller fisher syndrome

Ophthalmoplegia, ataxia, areflexia

Chronic progressive GBS

Symptoms persisting more than 6 weeks

Chronic relapsing GBS

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

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

MANAGEMENT
SPECIFIC
Steroids-----2mg/kg for 2 weeks Plasmapheresis-----to remove antibodies I/V immunoglobulins-----400mg/kg for 5 days or 1gm/kg for 2 days

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

D/D of acute flaccid paralysis

GB Syndrome Eitiology Delayed hypersesitivity. antibody mediated

Spinal cord syndrome Trasverse myelitis,spinal cord abscess,TB

poliomyelitis Poliovirus type I,II,III

History

GI or URTI, 5 to 14days preceding symptoms

Rapid progression Unimmunized, of symptoms 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

Signs

Symmetric flaccid weakness, sensations intact, gradually ascending

Symmetric areflexia,sensory loss below the level of lesion., pain, bowel and bladder dysfunction
Pleocytosis with raised protiens

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, Normal initially NCS shows delayed conduction Recovery in majority Depends on within 12 months eitiology

NCS normal, EMG denervation later Permanent disability in 1% cases

Course and prognosis