Acute Flaccid Paralysis

1

• Acute Flaccid Paralysis
• Presented by Dr. Mohd Abu Helwah
• Supervised by Dr. Afaf Al
  Areni

2
Differential Diagnosis of Acute Weakness

• Cerebral Bilateral strokes, Hysteria
• Cerebellar Acute cerebellar ataxia syndromes.
• Spinal Compressive myolopathy, Transverse
• myelitis.
• Peripheral nerve Acute inflammatory
  demyelinating
• neuropathy, Toxic
  neuropathy, Diphtheria,
• Tick paralysis

3
Differential Diagnosis of Acute Weakness

• Neuromuscular junction Botulism, Myasthenia
  Gravis
• Muscle disease Acute myositis
• Acute
  inflammatory myopathies
• Metabolic
  myopathies,
• Periodic
  paralysis

4
Acute Flaccid Paralysis

• The sudden onset of generalized flaccid weakness
  in the absence of symptoms of encephalopathy
  implicates the motor unit.
• AFP is an emergency in which management
  priorities are to support vital functions and
  reach a specific diagnosis in a timely manner
  with a focused history and physical examination.

5

• Guillain-Barré Syndrome.
• Poliomyelitis.
• Transverse Myelitis.

6
Guillain-Barré Syndrome

• It is an acute idiopathic monophasic acquired
  inflammatory demyelinating polyradiculoneuropathy.
  
• GBS is the most common cause of acute flaccid
  paralysis in healthy infants and children.

7
Epidemiology

• It has an annual incidence of 0.6 to 2.4 cases
  per 100,000 population and occurs at all ages and
  in both sexes.
• The incidence is lower in children, 0.38 and 0.91
  cases per 100,000 in two reports.
• Occurs rarely in children younger than two years
  of age, but can occur in infants.
• Males are affected approximately 1.5 times more
  often than females in all age groups.

8
GBSPathophysiology

• Immune mediated disease.
• There is no known genetic factors.
• Two third of cases follow a respiratory or GI
  infection.
• Campylobacter infection is the most common, but
  other organisms include CMV, EBV, HSV,
  Enteroviruses,
• Guillain-Barré syndrome has been reported to
  follow
• vaccinations
• epidural anesthesia
• thrombolytic agents

9
GBSPathophysiology

• The main lesions are acute inflammatory
  demyelinating polyradiculopathy, with acute
  axonal degeneration in some cases, particularly
  those following campylobacter infection.
• Avariety of autoantibodies to gangliosides have
  been identified especially with axonal forms of
  the disease.

10
Clinical Features  

• Usually 2 - 4 weeks following respiratory or GI
  infection.
• The classic presentation
• Fine paresthesias in the toes and
  fingertips.
• Lower extremity weakness
  symmetric ascending.
• Gait unsteadiness.
• Inability to walk.
• Respiratory muscles involvement.
• Neuropathic pain low back pain.
• Cranial Neuropathy
• Facial nerve is most commonly affected,
  resulting in
• bilateral facial weakness.

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Clinical Features cont

• By the peak of the illness, the frequency of
  symptoms was as follows
• - 79 had neuropathic pain
• - 60 could not walk
• - 51 had autonomic dysfunction
• - 46 had cranial nerve involvement
• - 24 could not use their arms
• - 13 required mechanical
  ventilation

12
Physical Examination

• Symmetric limb weakness
• diminished or absent reflexes
• Vibration and position sensation are affected in
  40 of cases.
• Autonomic dysfunction
• Cardiac dysrhythmias.
• Orthostatic hypotension,
  hypertension
• Paralytic ileus
• Bladder dysfunction

13
Clinical Course 

• gt90 of patients reach the nadir of their
  function within two to four weeks, with return of
  function occurring slowly over the course of
  weeks to months.
• The clinical course of GBS in children is shorter
  than in adults and recovery is more complete.
• In patients who did not require mechanical
  ventilation, the median time to recovery of
  independent walking was 43 to 52 days in children
  compared to 85 days in adults.

14
Forms of GBS

•  Acute inflammatory demyelinating polyneuropathy
  (AIDP) the most common form in developed
  countries.
• Acute motor axonal neuropathy more common in
  developing countries. More severe with common
  respiratory involvement. Strong association with
  campylobacter
• Acute motor-sensory axonal neuropathy 
• Miller Fisher syndrome triad of external
  ophthalmoplegia, Ataxia, areflexia with muscle
  weakness.
• Polyneuritis cranialis associated with CMV
  infection

15
Diagnosis

• Cerebrospinal Fluid
• - After the first week of symptoms
  typically reveals
• normal pressures, normal cell count and
  elevated
• proteins (greater than 50 mg/dL)
• - Early in the course (less than one
  week), protein levels
• may not yet be elevated, but only
  rarely do they remain
• persistently normal
• Electrophysiologic studies
• - Most specific and sensitive tests for
  diagnosis
• - Evidence evolving multifocal
  demyelination
• - A normal study after several days of
  symptoms, makes
• the diagnosis of Guillain-Barré
  syndrome unlikely.

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Doubt the Diagnosis of GBS IF

• Marked persistent asymmetry of weakness.
• Persistent bladder or bowel dysfunction.
• Bladder or bowel dysfunction at the onset.
• Mononuclear leukocytosis in the CSF gt 50.
• Sharp sensory level.
• Pupillary abnormalities are not seen in GBS.

17
GBS Management

• Critical care monitoring
• autonomic and respiratory dysfunction.
• Children with the following should be admitted to
  PICU
• a. Flaccid quadriparesis
• b. Rapidly progressive weakness
• c. Reduced vital capacity (20
  mL/kg)
• d. Bulbar palsy
• e. Autonomic cardiovascular
  instability
• N.B Sedation and neuromuscular blockade should
  be avoided in ventilated patients because they
  obscure the course of the illness.

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GBS Management

• 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.
• 20 of children with GBS require mechanical
  ventilation for respiratory failure.

19
Special Therapy

• Immune modulatory therapy
• Intravenous Immunoglobulins
• Plasmapheresis
• Both therapies have been shown to shorten
  recovery time by as much 50.
• Combining plasma exchange and IVIG neither
  improved outcomes nor shortened the duration of
  illness.

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Special Therapycont

• IVIG and plasma exchange are not recommended for
  ambulatory children with GBS who have mild
  disease or for children whose symptoms have
  stabilized.
• IVIG and plasma exchange for children with GBS
  should be reserved for those with
• A. Rapidly progressing weakness.
• B. Worsening respiratory status.
• C. Significant bulbar weakness.
• D. Inability to walk unaided.

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INTRAVENOUS IMMUNE GLOBULIN

• IVIG is preferred to plasma exchange in children
  because of the relative safety and ease of
  administration, although it has not been shown to
  have better results.
• Randomized trials in severe disease show that
  IVIG started within 4 weeks from onset hastens
  recovery as much as plasmapheresis.
• Long-term outcome, however, may not be affected.
• Studies have demonstrated that one effect of the
  IVIG is to neutralize neuromuscular blocking
  antibodies.

22
IVIG Regimens

• Several IVIG regimens have been utilized. One
  regimen includes daily IVIG for 5 days at a dose
  of 0.4 gm/kg/day, which results in an improvement
  within a mean of 2 to 3 days after the start of
  therapy. Other authors use 2 gm/kg of IVIG given
  as a single dose or 1gm/kg/day for 2 days.
• One study compared the outcome of 0.4 gm/kg/day
  given for 3 days versus 6 days. In that study,
  the 6 days of IVIG was superior when time to
  walking was used as an endpoint.
• When comparing treatments of 1gm/kg for 2 days
  versus 0.4gm/kg over 5 days, no significant
  difference in the effectiveness was noted in the
  2 treatment regimens. However, early relapses
  were more frequently observed in the shorter
  treatment group.

23
Plasmapheresis

• Studies in children indicate that plasmapharesis
  may decrease the severity and shorten the
  duration of GBS.
• It is most beneficial when started within 7 days
  of the onset of symptoms but is still beneficial
  in patients treated up to 30 days after disease
  onset.

24
Managementcont

• Corticosteroids are not effective and not
  indicated
• Interferon-ß reported to be beneficial in
  individual cases, but its safety and efficacy
  have not been established in clinical trials.

25
Prognosis

• In general, the prognosis in affected children is
  better than adults.
• Recurrences are uncommon but can occur in
  children. Some may have a chronic progressive
  course, whereas others may show recurrences or
  relapses.
• At long-term follow up, 93 were free of
  symptoms, and the remainder were able to walk
  unaided.
• 50 are ambulatory by 6 mo, 70 walk within ayear
  of onset of the disease.
• Mortality is approximately 3 to 4, and usually
  is secondary to autonomic dysfunction and
  respiratory failure.

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Poliomyelitis

• AKA Infantile paralysis,
• Acute anterior poliomyelitis,
• Acute lateral poliomyelitis.
• polio gray matter
• Myelitis inflammation of the spinal cord.
• Poliomyelitis is caused by a virus that attacks
  the
• nerve cells of the brain spinal cord
  although not
• all infections result in sever injuries and
  paralysis.

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Poliomyelitis Etiology

• Etiology
• Caused by a poliovirus.
• 3 serotypes of poliovirus (genus Enterovirus).
• Type 1 most frequently associated with epidemics.
• Types 2 and 3 usually associated with vaccine-
  associated paralytic polio (VAPP).

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Poliomyelitis

• In 1 of cases virus invades CNS.
• Multiplies and destroys anterior horn cells.
• In severe cases, poliovirus may attacks motor
  neurones in brainstem, leading to difficulty in
  swallowing,
• speaking and breathing.

29
Poliovirus Pathogenesis

• Incubation period of 7 to 14.
• Transmitted by oral-fecal contact.
• Person-to-person spread is the most common means
  of transmission, followed by contaminated water.
• During epidemics, it also may be transmitted by
  pharyngeal spread.
• Poliovirus initially infects the GI tract. It may
  spread to lymph nodes and rarely to CNS.
• The mechanism of spread of poliovirus to the CNS
  is not well understood.

30
Epidemiology

• 3 months-16 years rarely adults
• Predominant sex Male Female
• Improved sanitation led to many less infants
  being exposed to poliovirus.
• When exposure occurred later and the individuals
  were not protected by maternal antibodies, there
  were polio epidemics.

31
Poliomyelitis Incidence Prevalence

• Incidence
• Now rare present in
• (a) Endemic settings.
• (b) Small outbreaks in areas where polio
  eradication has
• occurred.
• (c) Rarely as vaccine-associated paralytic
  polio (VAPP) cases.
• Prevalence
• 1,486 cases in 2005 1,593 cases in 2006.
•  Endemic countries Afghanistan, India, Nigeria
  and Pakistan

32
Poliomyelitis Risk Factors

• Immune deficiency
• Pregnancy
• Poor sanitation and hygiene
•  Poverty
•  Unimmunized status, especially if lt5 years
• Tonsillectomy a risk factor for bulbar
  paralysis.
• Intramuscular injections or truama
• Genetics
• No genetic susceptibility has been identified.

33
Clinical Presentation

• The majority of patients are asymptomatic.
• 5 develop symptoms.
• 10 will show signs and symptoms of a minor GI
  illness, including fever, malaise, nausea, and
  vomiting.
• 0.1 develop the paralytic form of poliomyelitis.
• Symptoms of poliomyelitis always CNS specific.

34
Clinical Presentationcont

• CNS manifestations
• Weakness
• Vary from one muscle or group of muscles,
  to
• quadriplegia.
• Proximal muscles legs more commonly than
  arms.
• Typically worsens over 2 to 3 days but
  sometimes can
• progress for up to a week.
• Bulbar involvement
• 5 35 producing dysphagia, dysarthria,
  and difficulty
• handling secretions.
• There may be encephalitis, usually in infancy.
• Cardiovascular Resp. symptomsbulbar
  poliomyelitis

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• Physical Exam
• Significant motor loss on affected side or limb.
• Meningeal signs may be present in minor illness
  or early phases of paralytic polio.
• Decreased deep tendon reflexes.
• Muscle atrophy of affected areas.
• Tone is reduced asymmetric
• The sensory examination is normal.

36
Poliomyelitis Diagnosis 

• Based on the clinical presentation.
• Cerebrospinal Fluid
• Leukocytosis, Increased protein, Normal
  glucose.
• Virus recovery from stool, throat washing, blood.
• Virus recovery from stool is essential to
  diagnosis.
• Obtain stool, blood and throat samples for viral
  serology, demonstrating a four fold rise in IgG
  is helpful but not always easy.
• Positive IgM is diagnostic.
• Polymerase chain reaction amplification of
  poliovirus RNA from CSF or serologically, by
  comparing viral titers in acute and convalescent
  sera.

37
Diagnosiscont 

• Electrodiagnostic investigations reveal normal
  sensory nerve studies.
• Motor nerve studies
• show normal to mildly slowed conduction
  velocities and low to normal amplitudes.
• MRI may be helpful to evaluate involvement of
  anterior horn of the spinal cord or other
  findings.

38
Treatment

• No definitive treatment.
• Mainly supportive pain relief and physical
  therapy for muscle spasms.
• Patients with bulbar involvement require close
  monitoring of cardiovascular status and autonomic
  dysfunction.
• Mechanical ventilation Respiratory failure.
• Treatment of complications.

39
Poliomyelitis Complications

• Urinary tract infection
• Skin ulcers
• Traumatic injuries to affected limb(s)
• Atelectasis Pneumonia
• Myocarditis
• Postpoliomyelitis progressive muscular atrophy.
• Postpoliomyelitis motor neuron disease.

40
Clinical Course Outcome

• About two-thirds of patients with acute flaccid
  paralysis do not regain full strength.
• The more severe the acute weakness, the greater
  the chance of residual deficits, Bulbar squeals
  are rare.
• The mortality was 5 to 10 in the era of
  epidemics, and approached 50 for those with
  bulbar involvement because of cardiovascular and
  respiratory complications.

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• PREVENTION OF POLIOMYELITIS

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Polio Vaccination 

• Jonas Salk created the inactivated poliovirus
  vaccine (IPV), using killed virus in 1952.
• The Sabin oral poliovirus vaccine (OPV), using
  live attenuated virus, proved successful in 1960.
• In areas of the world where polio is endemic,
  primary immunization is still performed with
  Sabin OPV. But, because it causes polio in one
  out of 2.5 million cases, it has been replaced by
  the Salk IPV in countries without polio,
  including the United States and most of Europe.

43
Polio Vaccination 

• Multiple doses required to achieve high humeral
  conservation rates against all virus types
• Babies are given 4 doses through out their
  infancy.
• Adolescents and adults should get vaccinated as
  well.
• Adolescents younger than 18 should receive the
  routine four doses.
• You should get it if you travel outside places
  where polio is still epidemic.

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What is Post Polio Syndrome ?

• It is the late manifestation of acute paralytic
  polio.
• 25-40 of people who had paralytic polio15-40yr
  previously.
• They show symptoms of muscle and joint pain,
  general fatigue and weakness.
• Three indications of PPS
• A. Previous diagnoses of polio late effect of
  polio to people
• that got it like when they where 10 years
  old.
• B. Long interval following recovery people
  usually live long
• but effect can occur during 30-35 years
  after the diagnoses.
• C. Gradual onset weakness that tends to be
  perceptible until it
• interferes with daily activities.

45
Criteria For Diagnosis of Post Polio Syndrome

• A prior episode of paralytic poliomyelitis.
• EMG evidence of longstanding denervation.
• A period of neurologic recovery and functional
  stability preceding the onset of new problems
  (Usually gt20 years).

46
Criteria for Diagnosis of PPScont

• Gradual or abrupt onset of new non-disuse
  weakness in previously unaffected or affected
  muscles.
• May be asssociated with fatigue, muscle pain,
  joint pain, decreased function, etc.
• Exclusion of other conditions that may cause the
  above features.

47
Main Clinical Features of PPS

• Fatigue (Commonest)
• Weakness
• Muscle pain
• Gait disturbance
• Respiratory problems
• Swallowing problems
• Cold intolerance
• Sleep apnoea

48
Management of Post Polio Syndrome

• Clinical Assessment.
• Exclusion of other causes of disability.
• Introduction to concept of interdisciplinary
  team.
• Follow-up as necessary

49
Transverse myelitis

• Inflammation of the spinal cord causing acute/or
  subacute loss of motor, sensory and autonomic
  function, often evolves in hours or days.
• Pathophysiology presumed autoimmune mediated
  inflammation and demyelination of the spinal
  cord.
• Postinfectious etiology largely predominates in
  children

50
Associated Conditions

• Is most commonly associated with infection.
• May be associated with demyelination of other
  parts of the central nervous system, e.g. MS,
  ADEM
• Connective tissue diseases, e.g. SLE, JRA,
  sarcoidosis, vasculitis
• Rarely seen in association with metabolic causes
  of myelopathy such as vitamin B12 deficiency.
• ATM does not usually occur in association with
  inherited demyelinating diseases, e.g.
  leukodystrophies.

51
Transverse myelitis

• Mean age of onset is 9 years.
• Symptoms progress rapidly, peaking within 2 days.
• Usually level of myelitis is thoracic.
• Asymmetrical leg weakness, sensory level and
  early bladder involvement.
• Recovery usually begins after a week of onset.

52
Physical Examination

• Tenderness over the spine may point to trauma or
  infection.
• Visual acuity and color vision, funduscopic
  examination for optic neuritis.
• Increased tone, spastic weakness, legs more than
  arms
• Reflexes are usually brisk, with positive
  Babinski sign.
• Sensory ataxia, a sensory level.
• Sphincter dysfunction.

53
Diagnosis

• MRI and CSF analysis are the two most important
  tests and are mandatory. If both tests are
  negative, repeat tests in 2 to 7 days is
  recommended.
• MRI of spine usually shows swelling of the cord,
  but at times is normal.
• Gadolinium-enhanced MRI of the brain and the
  orbit and evoked potential studies (e.g. Visual
  evoked potential, Somatosensory evoked potential)
  may identify other sites.

54
Lumbar puncture

• Normal or slightly increased protein.
• Mild pleocytosis with lymphocyte predominance.
• Elevation of IgG index and presence of
  oligoclonal
• bands are indicative of MS or other
  systemic
• inflammatory disease.
• CSF studies for infections all should be
  negative.
• Investigation for underlying systemic
  inflammatory disorder.

55
Treatment

• IV methylprednisolone may be useful in ATM or
  other acute demyelinating diseases based on a few
  observational studies.
• IV immunoglobulin (IVIG) or plasmapheresis may be
  a safe and effective therapeutic alternative in
  patients that do not respond to or intolerant of
  IV methylprednisolone.
• Cyclophosphamide has been reported to be useful
  in myelitis associated with systemic inflammatory
  diseases.

56
Treatmentcont

• Symptomatic management of bowel and bladder
  dysfunction.
• Management of respiratory, cardiovascular
  autonomic dysfunction.
• Physical and occupational therapy (PT/OT) may
  help promote functional recovery and prevent
  contractures.

57
Prognosis

• 50 make a full recovery within 3 to 6 months.
• 40 recover incompletely.
• 10 dont recover.
• Older age, increased deep tendon reflexes, and
  presence of Babinski sign may indicate better
  course.
• Rapid progression, back pain, and spinal shock
  predict poor recovery.
• TM may be the presenting feature of MS,
  especially in patients with partial TM and
  abnormal initial brain MRI, in such cases, follow
  up MRIs should be considered.

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• THANK YOU