Download - Acute flaccid paralysis: make it easy
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ACUTE FLACCID PARALYSIS AFP
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DEFINITION
• sudden onset of weakness or paralysis
over a period of 15 days in a patient aged less than 15 years age.
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Involvement
• AHC:
1- acute poliomyelitis
2- acute transverse myelitis
• Peripheral Nerves:
1- roots: GBS (post-infectious)
2- toxins: Diphteria, porphyria
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Involvement
• N-M junction:
botulinum toxin
tick toxin
• Metabolic:
Periodic paralysis
• Muscular:
myositis
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Case Presentation
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Climbing Paralysis
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Guillain Barre Syndrome GBS
• The most common cause of acute flaccid paralysis (AFP) among infants.
• Age : any including newborn • Sex : any ( male > female)
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Guillain-Barre’ Syndrome
• Post-infectious polyneuropathy; ascending polyneuropathic paralysis
• An acute, rapidly progressing and potentially fatal form of polyneuritis
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Pathophysiology
Autoimmune disorder (T cell sensitization)
cause of demyelination Due to attack of the myelin sheath of
nerves by:• antibodies (Ig M, Ig G)• white blood cells (macrophages)• Complement activation on the outer
surface of myelinated fibers Because (POST-) Virus/Bacteria share antigenic sites with
axons & peripheral nerve sheath or both
MYELIN SHEATH
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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 & sympathatic chain
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Preceding Events : (1-3 WEEKS)
• Respiratory infections :
1- Viral: CMV, EBV, Varicella virus , influenza virus
2- Bacterial: Mycoplasma pneumoniae, H influenza
• Gastrointestinal infections : Campylobacter-jejuni (Bloody GE)
• Vaccinations• Post surgery
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Aetiology
• Mycoplasma•Hepatitis B•CMV•EBV•Measles•Mumps•Echovirus•Cocksakie virus•Influenza virus
• Varicella virus•Compylobacter jejuni
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classification of GBS (Clinical, Pathological & neurophysiological)
• 1-Classic type (mixed): Acute inflammatory demyelinating polyradiculo-neuropathy (AIDP)
• 2- Pure motor GBS*• 3- Pure sensory GBS • 4- Pure pandysautonomia • 5-- Miller-Fisher syndrome ( hypotonia, ophthalmoplegia,
ataxia)
NB., Pure Motor GBS* Usually Post Campylobacter-.jejuni infection
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C/P:
• 1- IP ( afrebrile ) : 1-3 weeks • 2- CP: motor , sensory , autonomic• 3- Serious Association
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Guillain-Barre’ Syndrome
• Affects the peripheral nervous system•
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Symptoms and Signs (Typical GBS)
Motor : 1- Symmetric acute progressive ascending weakness <4 wks, starting in LL
2- Areflexia or hyporeflexia 3- Atonia or hypotonia
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Symptoms and Signs (Typical GBS)
Sensation : 1- C/O pain as hyperthesia or cramps 2- O/E loss of pain sensation (hypothesia) in feet/hands 3- Both C/O, and O/E
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Autonomic :
1- BP: orthostatic hypotension, labile hypertension 2- bradycardia, arrythmia 3- atonic bladder, 4- constipation 5- flashing and/or sweating 6- alteration of temperature
• Symptoms and Signs (Typical GBS)
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Characteristic “3A”triad:
• ascending weakness : a- bilateral symmetrical weakness b- usually start in LL, then UL c-then, might be affected : i- cranial nerves (Brain stem) :
including glosssopharyngeal and vagus nerves ( difficulty of swallowing even of fluid and water) and III, IV, VI cranial nerves ( eye muscles in Miller Fisher variety), VII Facial nerve ( unilateral or bilateral), and then respiratory muscles
ii- respiratory muscles iii- phrenic nerves ( diaphragm )• areflexia ( Hallmark)• atonia ( hypotonia)
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Serious Association:
• respiratory failure: • diaphragmatic weakness (Phrenic nerves.)• respiratory muscles weakness• oropharyngeal weakness: impaired swallowing of
secretions & aspiration• cardiac arrest• aspiration pneumonia
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CLINICAL VARIANTS
1–Polyneuritis cranialis
Cranial nerve involvement
2–Miller fisher syndrome
Ophthalmoplegia, ataxia, areflexia
3–Chronic progressive GBS
Symptoms persisting more than 6 weeks
4- Chronic relapsing GB
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Differentiation from spinal cord syndrome
• Loss of arm reflexes
• Absence of sensory level
• Lack of spinal tenderness
• Normal bowel and bladder function
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Investigations
Early
Nerve Conduction Velocity (NCV) abnormality
AFTER 1ST WEEK • Late : CSF study : albuminocytogenic dissociation
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1- NCV/EMG:
• i- Early :Delayed or absent F waves or H reflexes
• ii- slow or block of Nerve conduction velocity
• iii- normal EMG/ extensive fibrillation showing denervation
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2- CSF: Albuminocytologic dissociation (Froin Syndrome)
• i- Increased CSF protein with normal cells
• ii- might be normal CSF during 1st week
• ii- usually +ve after 2 weeks of onset
Differential Diagnosis of cytoalbuminous dissociation
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DD
• 1- GBS• 2- poliomyelitis• 3- Diphteria polyneuritis • 4- spinal cord compression• 5- transverse myelitis• 6- infratentorial tumor• 7- venous sinus thrombosis• 8- lead poison• 9- botulism
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Investigations (OTHERS)
• Antibody study : Ig M autoantibodies to GM1 and GM2 gangliosides or Spinal MRI or normal CPK
• Anti-GQ1b antibodies are typically found in patients with the Miller Fisher syndrome (Acta Pediatr 2011)
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Outcome of GBS patients
• Regressive : 90 % of patients making a good recovery, after 2-3 weeks of onset, Recovery, usually beginning 2 to 4 weeks after progression stops starting from the last muscles affected till lower limb ( descending pattern )
• Chronic Relapsing: Less than 5% of patients• Mortality: 3-% die from complications as respiratory
failure especially in infants
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Treatment
Hospitalization • 1- General care • 2-- Specific treatment • 3- complication treatment
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Treatment
• Hospitalization : i- Must be treated in a hospital, never at home
ii- because of a risk of sudden onset of cardiac or respiratory arrest
iii- any hospital ? No, it must be a hospital have a pediatrics ICU
Hospitalization is continued until the child's condition has clearly stabilized.
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General care
• i- bed sores
ii-bowel care
iii- nutrition care • monitoring of vital signs
–Nursing care–Repeated spirometries–Bowel and bladder care–Tube feeding–Care for bed sores–Ventilatory support if required
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Specific treatment
• i- IV immunoglobin: 2 gm/kg treatment
*at a dose of 0.4 g/kg/day for 5 consecutive days or
* 1gm/kg/day for 2 days• ii- plasmapheresis: 5 exchanges
of 50 ml plasma/ kg on alternate days ( 10 days course).
• iii- both i and ii
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complication treatment:
i- artificial respiration for respiratory failure
ii- muscular pain: pain killer as NSAI
iii- chronic relapsing: trial of immunosupprive drugs or corticosteroid
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Need for intensive care (PICU)
- Flaccid quadriparesis- Rapidly progressive weakness- Reduced vital capacity (≤20 mL/kg)- Bulbar palsy- Autonomic cardiovascular instability
Need for assisted ventilation — Approximately 20 percent of children with GBS require mechanical ventilation for respiratory failure
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Warning signs for RF*
- A sustained increase of pCO2 to ≥50 mmHg (normally 35 to 40 mmHg)
- An increasing respiratory rate- Increasing oxygen requirement and increasing
alveolar to arterial oxygen difference (normally 5 to 10 mmHg)
- An increased use of accessory muscles (eg, sternocleidomastoid use, flaring of the ala nasae, intercostal retractions) and decreased or paradoxical diaphragm movements; these reflect restrictive lung-chest wall movement and low lung volumes
- Sweating about the head and neck, wide pulse pressure, and bounding pulses portend CO2 retention.
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• Children have less metabolic and muscle reserve than adults. They can deteriorate quite rapidly and become apneic or develop alveolar hypoventilation "right under your nose."
• Sedation and neuromuscular blockade should be avoided in ventilated patients because they obscure the course of the illness.
• Providing scrupulous airway care and chest physiotherapy reduce the risk of pneumonia.
• Tracheostomy may need to be performed if prolonged ventilation is required.
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PROGNOSIS
• Mortality 3%• 20% of cases need repiratory ve• Recovery
- 1 to 6 months, may take 12 months
- Delayed recovery may be followed by permanent neurological sequel
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Transverse Myelitis:
• ? of immunological disorder• C/P : of AFP ( acute onset of flaccid
hypotonic weakness ) with the following characters: • LL paralysis : paraplegia with areflexia• with sensory level of loss of sensation • Later : hyperreflexia
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Poliomyelitis :
• due to enterovirus affection (polio virus ) ( non or inadequate OPV )
• C/P: of AFP with the following characters:• first shock stage ( with generalized hypotonia) then
patchy asymmetrical weakness • normal sensation• areflexia of affected muscles
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