Acute Flaccid Paralysis

ACUTE FLACCID PARALYSIS

• 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 DEF 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 Compylobacter 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

Differentiation from spinal cord syndrome Loss of arm reflexes Absence of sensory level Lack of spinal tenderness Normal bowel and bladder function

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

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 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,. meningismus, 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

Pleocytosis with raised protiens

EMG/NCS

Signs of denervation, Normal initially NCS shows delayed conduction

NCS normal, EMG denervation later

Course and prognosis

Recovery in majority Depends on within 12 months eitiology

Permanent disability in 1% cases