afp pakar dr norazni
TRANSCRIPT
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Acute flaccid paralysis (AFP) is a clinical syndromecharacterized by rapid onset of weakness, including(less frequently) weakness of the muscles of
respiration and swallowing, progressing to maximumseverity within several days to weeks.
The term "flaccid" indicates the absence of spasticity
or other signs of disordered central nervous systemmotor tracts such as hyperreflexia, clonus, orextensor plantar responses
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AFP is a complex clinical syndrome with a broad arrayof potential etiologies.
Accurate diagnosis of the cause of AFP has profoundimplications for therapy and prognosis.
If untreated, AFP may not only persist but also lead todeath due to failure of respiratory muscles.
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AFP, a syndrome that encompasses all cases ofparalytic poliomyelitis, also is of great public healthimportance because of its use in surveillance for
poliomyelitis in the context of the global polioeradication initiative.
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Each case of AFP is a clinical emergency and requiresimmediate examination.
For all cases, a detailed clinical description of thesymptoms should be obtained, including fever,myalgia, distribution, timing, and progression ofparalysis.
The symptoms of paralysis may include gait
disturbance, weakness, or troubled coordination inone or several extremities
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Comprehensive neurologic examination, includingassessment of muscle strength and tone, deep tendonreflexes, cranial nerve function, and sensation
Look for meningismus, ataxia, or autonomic nervoussystem abnormalities (bowel and bladder dysfunction,sphincter tonus, neurogenic reflex bladder)
Fasciculation is often cited as a sign of anterior horn
cell damage, but it may also be present indemyelinating neuropathies.
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Electrophysiologic studies are very important fordetermining the diagnosis and prognosis of lowermotor neuron disease
Nerve conduction velocity and electromyographicstudies are used to differentiate demyelinatingneuropathies from axonal neuropathies
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Clinical Pathways for AFP
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Guillain-Barre syndrome: the commonestcause of acute flaccid paralysis (AFP) inhealthy children
0.9-1.5 per 100,000 population
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Acquired, monophasic
Immune mediated disease
No known genetic factor
Two thirds of cases follow a respiratoryor gastrointestinal infection
Campylobacter infection is the most common,but other organisms include CMV, EBV, HSV,enteroviruses, …
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The main lesions are acute inflammatorydemyelinating polyradiculoneuropathy, withacute axonal degeneration in some cases,particularly those following campylobacterinfection
A variety of autoantibodies to gangliosides
have been identified especially with axonalforms of the disease
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Two to four weeks after a benign febrileillness
Common presentations are paresthesiasin the fingers and toes, pain is a commonpresentation in children (79%),particularly low back pain
Symmetrical weakness in the lower
extremities, that ascends over hours todays to involve the arms, and in severecases respiratory muscles
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Cranial nerves are affected in 30% of thecases, most commonly the facial nerve withbilateral facial weakness
More than 90% of patients reach the nadir oftheir function within 2-4 weeks
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Symmetrical weakness with diminished or absentreflexes
Vibration and position sensation are affected in 40%
of cases 50% of patients have evidence of autonomic
dysfunction
Cardiac dysrythmias
Orthostatic hypotension, hypertension
Paralytic ileus
Bladder dysfunction
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Cerebrospinal fluid:
After the first week of symptoms, CSF typically reveals normalpressure, normal cell count, and elevated protein
Electrophysiologic studies:
Most specific and sensitive tests for diagnosis
Evidence of evolving multifocal demyelination
Normal studies after 10 days of illness make the diagnosis ofGBS unlikely
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Expectant with mild cases
Immune modulatory therapy for rapidly progressivecases, most effective the first 10 days
Plasmapharesis
IVIG
Steroids are not effective and not indicated
Critical care monitoring
Most common cause of death is autonomic dysfunction
Second most common cause of death is respirotoryfailure
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Risk factors for respiratory failure in GBS
Cranial nerve involvement
Short time from preceding respiratory illness
Rapid progression over less than 7 days
Elevated CSF protein in the first week
Severe weakness
Unable to lift elbows above the bed
Unable to lift head above the bed
Unable to stand
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Children have a shorter clinical course than adults
Severity of the illness does not correlate with long termoutcome, 85% of children have excellent recovery
50% are ambulatory by 6 months, 70% walk within ayear of onset of the disease
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Acute demyelinating disorder of the spinal cord thatevolves over days usually but may have a hyperacutepresentation
May be associated with demyelination in other partsof the central nervous system
Commonly preceded by a viral infection orimmunization
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Commonly presents with an ascending weakness
Initially reflexes may be depressed or absent becauseof spinal shock or involvement of the nerve roots
Must be considered in cases of weakness withoutbulbar involvement
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Mean age of onset is 9 years
Symptoms progress rapidly, peaking within 2days
Usually level of myelitis is thoracic
Sensory level, asymmetrical leg weakness, andearly bladder involvement. Back pain is commonat the onset
Tendon reflexes may be decreased or increased
Recovery usually begins after a week of onset
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Diagnosis: MRI of the spineusually shows swelling of thecord, but at times is normal.Exclusion of acute cordcompression is essential
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High doses of IV steroids(methylprednisolone) followed by taperingdoses of prednisone
Prognosis: 50% make a full recovery, 40%recover incompletely, and 10% do notrecover
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Poliovirus, cosxackievirus, and theechovirus group are RNA viruses thatinhabit the GI tract of humans
They are neurotropic, and produceparalytic disease by destroying the motorneurons of the brainstem and spinal cord
Poliovirus causes the most severe
paralysis, coxsackie and echoviruses aremore likely to cause an asepticmeningitis
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Nonpolio enteroviruses hav been associatedwith polio-like paralytic disease, frequentlyaccompanied by other clinical syndromes,
such as aseptic meningitis, hand-foot-and-mouth disease, and acute hemorrhagicconjunctivitis.
Among all known nonpolio enteroviruses,
enterovirus 71 has been most stronglyimplicated in outbreaks of central nervoussystem disease and AFP.
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Known since ancient Egyptiantimes
English physician MichaelUnderwood in 1789, refers to
polio as "a debility of thelower extremities
Poor sanitation contributed toincreased natural immunities
among human population
Outbreaks started to occur in20th century
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Outbreaks reached pandemicproportions in Europe, North America,Australia, and New Zealand during thefirst half of the 20th century.
In US, 1952 polio epidemic becamethe worst outbreak in the nation'shistory.
Of nearly 58,000 cases reported thatyear 3,145 died and 21,269 were leftwith mild to disabling paralysis.
Intensive care medicine has its originin the fight against polio
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Epidemics usually occur in the spring and summer
Usually a brief illness characterized by fever, malaiseand GI symptoms precedes the paralytic illness
After the febrile illness, there is a brief period ofapparent well being, after which the fever recurs, withheadache, vomiting and meningeal irritation
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Pain in the limbs and spine is followed rapidly by limbweakness
Pattern of limb weakness is variable, but is generallyasymmetric
Weakness, diminished reflexes and muscle atrophy are seen
Paralysis
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Bulbar polio may occur with or without spinal polioand is life threatening
Affected children have prolonged periods of apnea and
require mechanical ventilation
Extraoccular muscles are spared
Paralytic polio is rarely seen after the introduction ofthe polio vaccine
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Diagnosis:
Clinical suspicion
CSF leukocytosis is seen the acute phase, elevatedprotein may also be seen
CBC shows leukocytosis
Virus recovery from stool is essential
Obtain stool, blood and throat samples for viral serology,demonstrating a four fold rise in IgG is helpful but notalways easy. Positive IgM antibodies is diagnostic
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Treatment: mainly supportive
Mechanical ventilation may be needed in bulbarinvolvement
Pain management for paresthesias
Physical therapy
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The early 20th century epidemics—which leftthousands of children and adults paralyzed—providedthe impetus for a "Great Race" towards the
development of a vaccine. Developed in the 1950s, polio vaccines are credited
with reducing the global number of polio cases peryear from many hundreds of thousands to around athousand
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1950 – Koprowsky intoduced live attenuated vaccine
1952 – Salk developed The Salk vaccine, orinactivated poliovirus vaccine (IPV), based on
poliovirus grown in a type of monkey kidney tissueculture(Vero cell line), which is chemically inactivatedwith formalin.
After two doses of IPV (given by injection), 90% or
more of individuals develop protective antibody to allthree serotypes of poliovirus, and at least 99% areimmune to poliovirus following three doses
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Albert Sabin developed oral polio vaccine (OPV
The attenuated poliovirus in the Sabin vaccine replicates veryefficiently in the gut, but the vaccine strain is unable toreplicate efficiently within nervous system tissue.
A single dose of Sabin's oral polio vaccine producesimmunity to all three poliovirus serotypes in approximately50% of recipients. Three doses of live-attenuated OPVproduce protective antibody to all three poliovirus types inmore than 95% of recipients.
Human trials of Sabin's vaccine began in 1957, and in 1958it was selected, in competition with the live vaccines ofKoprowski and other researchers, by the US NationalInstitutes of Health and licensed in 1962.
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Because OPV is inexpensive, easy toadminister, and produces excellent immunityin the intestine (which helps prevent infectionwith wild virus in areas where it is endemic),it has been the vaccine of choice for
controlling poliomyelitis in many countriesworldwide.
]1 case per 750,000 vaccine recipients theattenuated virus in OPV showed risk to revertinto a paralytic form.
Most industrialized countries have switchedto IPV, which cannot revert, either as the solevaccine against poliomyelitis or incombination with oral polio vaccine
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Collecting stools for enterovirus in children with AFP is animportant part of the Global Polio Eradication Initiative (GPEI)
For Malaysia to remain a polio-free country we need to provethat none of our cases of AFP are caused by poliovirusinfection.
The target ―background rate‖ for AFP in the GPEI is 2 per100,000 children below 15 years old.
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GBS – 30.2%
CNS infections – 16.2%
Transverse myelitis – 10.6%
Causes of AFP in Malaysia(1997-2001 Surveillance)
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WHO FP
CL SSIFIC TION
PROTOCOL
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Age-standardised disability-adjustedlife year (DALY) ratesfrom Poliomyelitis by country (per
100,000 inhabitants) - 2002
Polio-free1994
Polio-free 2002
Polio-free 2000
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