With virtual elimination of poliomyelitis, GBS has become the most common cause of acute flaccid paralysis in many parts of the world
The overall prognosis of GBS is quite good,
Children with vital capacity approximately one half the normal value for age or ≤20 mL/kg body weight generally progress to require ventilatory support. In a study of patients with GBS that included some children, serial measurements of pulmonary function tests were most helpful in detecting the risk of developing respiratory failure
Pulmonary testing and measuring vital capacity is difficult in children who cannot cooperate, typically those younger than 6 years of age. These patients should be closely monitored and observed for fatigue and other clinical signs of impending respiratory muscle failure. These signs include the following:
hildren have less metabolic and muscle reserve than adults. They can deteriorate quite rapidly and become apneic or develop alveolar hypoventilation &quot;right under your nose.&quot; Generally, it is wise to have a pediatric pulmonologist involved early in the clinical course. 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.
ACUTE FLACCID PARALYSIS AFP
DEFINITION• sudden onset of weakness or paralysis over a period of 15 days in a patient aged less than 15 years age.
Guillain Barre Syndrome GBS• The most common cause of acute flaccid paralysis (AFP) among infants.• Age : any including newborn• Sex : any ( male > female)
Guillain-Barre’ Syndrome• Post-infectious polyneuropathy; ascending polyneuropathic paralysis• An acute, rapidly progressing and potentially fatal form of polyneuritis
Pathophysiology Autoimmune disorder (T cell sensitization)MYELIN SHEATH 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
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
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
C/P:• 1- IP ( afrebrile ) : 1-3 weeks• 2- CP: motor , sensory , autonomic• 3- Serious Association
Guillain-Barre’ Syndrome• Affects the peripheral nervous system•
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
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
• Symptoms and Signs (Typical GBS)Autonomic : 1- BP: orthostatic hypotension,labile hypertension 2- bradycardia, arrythmia 3- atonic bladder, 4- constipation 5- flashing and/or sweating 6- alteration of temperature
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)
Differentiation from spinal cordsyndrome• Loss of arm reflexes• Absence of sensory level• Lack of spinal tenderness• Normal bowel and bladder function
Investigations Early Nerve Conduction Velocity (NCV) abnormality AFTER 1ST WEEK• Late : CSF study : albuminocytogenic dissociation
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
2- CSF: Albuminocytologicdissociation (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
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)
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
Treatment Hospitalization• 1- General care• 2-- Specific treatment• 3- complication treatment
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 ICUHospitalization is continued until the childs condition has clearly stabilized.
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
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
complication treatment: i- artificial respiration for respiratory failure ii- muscular pain: pain killer as NSAI iii- chronic relapsing: trial of immunosupprive drugs or corticosteroid
Need for intensive care (PICU) - Flaccid quadriparesis - Rapidly progressive weakness - Reduced vital capacity (≤20 mL/kg) - Bulbar palsy - Autonomic cardiovascular instabilityNeed for assisted ventilation — Approximately 20 percent of children with GBS require mechanical ventilation for respiratory failure
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.
• 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.
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
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
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