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Acute Flaccid Paralysis

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Acute Flaccid Paralysis Presented by: Dr. Moh d Abu Helwah Supervised by: Dr. Afaf Al Areni Differential Diagnosis of Acute Weakness: Cerebral: Bilateral strokes ... – PowerPoint PPT presentation

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Title: 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.

11
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.

16
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.

18
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.

20
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.

21
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.

26
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.

27
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).

28
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

35
  • 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.

41
  • PREVENTION OF POLIOMYELITIS

42
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.

44
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.

58
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