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 Nerve fibres Poliomyelitis Acute transverse myelitis Nerve fibres Postinfectious polyneuropathy (GBS syndrome) Toxins----diphtheria, porphyria

INVOLVEMENT Neuromuscular junction Metabolic causes Muscular disease 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 Miller fisher syndrome 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 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

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