ACUTE FLACCID

PARALYSIS
 Acute Flaccid Paralysis

The sudden onset of generalized flaccid

weakness/paralysis 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 and management in a timely
manner.
 Differential Diagnosis of Acute Weakness:
Central: Peripheral nerve:

Bilateral strokes Acute inflammatory

Acute cerebellar ataxia demyelinating
polyneuropathy (GBS),
Anterior horn cells: Toxic neuropathy
Diphtheria
Poliomyelitis Tick paralysis
Myelopathy,
Transverse myelitis Muscle disease:

Neuromuscular junction: Acute myositis,

Acute inflammatory
Botulism myopathies,
Myasthenia Gravis Metabolic myopathies,
Periodic paralysis
Guillain-Barré Syndrome.
Poliomyelitis.
Transverse Myelitis.
 Guillain-Barré Syndrome

⦿ GBS is the most common cause of acute

flaccid paralysis in healthy infants and
children.
⦿ It is an acute demyelinating
polyradiculoneuropathy.
⦿ GBS is an idiopathic monophasic acquired
inflammatory disorder.
 Epidemiology

⦿ Occurs at all ages and in both genders.

⦿ Males are affected approximately 1.5 times more often

than females in all age groups.

⦿ Occurs rarely in children younger than two years of

age, but can occur in infants.
 GBS…Pathophysiology

⦿ 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 etc
 GBS…Pathophysiology

⦿ The main lesions are acute inflammatory

demyelinating polyradiculopathy, with acute
axonal degeneration in some cases,

⦿ A variety of auto-antibodies to gangliosides

have been identified especially with axonal
forms of the disease.
 Clinical Features
Usually 2 - 4 weeks following resp. or GI infection.
⦿ The classic presentation:
Paresthesias in the toes and fingers.
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 as bilateral
facial weakness.
 Physical Examination
⦿ Symmetric limb weakness(Tone and power)
⦿ diminished or absent deep tendon reflexes
⦿ Senses like Vibration and position sensation
are affected in 40% of cases.
⦿ Autonomic dysfunction:
Cardiac dysrhythmias.
Orthostatic hypotension, hypertension
Paralytic ileus
Bladder dysfunction
Constipation
Flashing and/or sweating
 Clinical Course

⦿ The clinical course of GBS in children is shorter than in

adults and recovery is more complete.

⦿ >90% of patients reach the peak of their symptoms

within two to four weeks,

⦿ Return of function occurring slowly over the course of

weeks to months and years in rare cases.

⦿ In children who did not require mechanical ventilation,

the median time to recovery of independent walking
was 6-8 weeks compared to 12-14 weeks in adults.
 Forms of GBS
⦿ Acute inflammatory demyelinating polyneuropathy
(AIDP)
⚫ Most common form in developed countries.

⦿ Acute motor axonal neuropathy(AMAN):

⚫ More common in developing countries.
⚫ More severe with common respiratory involvement.
⚫ Strong association with campylobacter

⦿ Acute motor-sensory axonal neuropathy (AMSAN)

⦿ Miller Fisher syndrome: triad of (AOA)

⚫ Ataxia,
⚫ ophthalmoplegia,
⚫ Areflexia with muscle weakness.
Diagnosis
Cerebrospinal Fluid:
- Early in the course (less than one week), normal
- After the first week of symptoms typically reveals:
normal pressures,
normal cell count and elevated proteins

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.
Management

⦿ Indication for PICU admission:

a. Flaccid quadriparesis
b. Rapidly progressive weakness
c. Reduced vital capacity (≤20 mL/kg)
d. Bulbar palsy
e. Autonomic cardiovascular instability
GBS Management

Predictors for respiratory failure in GBS:

Cranial nerve involvement.
Short time from preceding respiratory illness (<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.
10-20% of children with GBS require mechanical
ventilation for respiratory failure.
Specific 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.
Specific Therapy…cont

⦿ 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.
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 comparatively.

⦿ Randomized trials in severe disease show that IVIG started

within 4 weeks from onset hastens recovery as much as
plasmapheresis.

⦿ But long-term outcome may not be affected.

⦿ IVIG is responsible to neutralize culprit autoantibodies.

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.
Management…cont

⦿ 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.
Prognosis
⦿ In general, the prognosis in affected children is better than
adults.
⦿ Recurrences are not uncommon 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 a year of
onset of the disease.
⦿ Mortality is approximately 3 to 5%, and usually is secondary
to autonomic dysfunction and respiratory failure.
 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.
Poliomyelitis: Etiology

Etiology:
⦿ Caused by a poliovirus (genus Enterovirus).
⦿ 3 serotypes of poliovirus. (Wild Types)
⦿ Type 1 causes paralysis in about 1 in 200
infections; Type 2 was last recorded in
1999; Type 3 is less virulent than type 1, causing
paralysis in about 1 in 1000 cases.
Poliomyelitis

⦿ Multiplies and destroys anterior horn cells.

⦿ In 1% of cases virus invades CNS

⦿ In severe cases, poliovirus may attacks motor neurones

in brainstem, leading to difficulty in swallowing,
speaking and breathing. (Bulbar Polio)
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.
⦿ 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.
Epidemiology

⦿ 3 months-16 years; rarely adults

⦿ 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.
Poliomyelitis: Incidence & Prevalence

Incidence:
Now rare; present in:
(a) Endemic settings.
(b) Small outbreaks in areas where polio eradication has
occurred.
(c) mostly as vaccine-associated paralytic polio (VAPP) cases.

Prevalence:
⦿ Endemic countries: Afghanistan, Pakistan
Polio and Pakistan

⦿ 1988 – WPEP started

⦿ Current year 01 case from KPK has been reported
⦿ Last year 20 cases reported all from KPK
⦿ Last vaccine derived polio infection detected in 2021.
⦿ 8 JUNE 2023 – Karachi's first detection of wild
poliovirus in 2023 has been confirmed in an
environmental sample from Sohrab Goth
⦿ India – eradication 1914
Poliomyelitis: Risk Factors
⦿ Immune deficiency
⦿ Pregnancy
⦿ Poor sanitation and hygiene
⦿ Poverty
⦿ Un-immunized status, especially if <5 years
⦿ Tonsillectomy: a risk factor for bulbar paralysis.
Immunity to Poliovirus Infection

⦿ Exposure to poliovirus initiates a complex process

that eventually results in both humoral (systemic)
and mucosal (local) immunity.
⦿ Poliovirus infection provides lifelong immunity
against the disease, but this protection is limited to
the particular type of poliovirus involved (Type 1, 2,
or 3)
⦿ Thus, infection with one type does not protect an
individual against infection of the other two types.
IgM and IgG antibodies are detected in the serum as
early as 1-3 days following natural infection
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
Clinical Presentation…cont
CNS manifestations: spinal, bulbar, spinobulbar
⦿ 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. Cardiovascular & Resp. symptoms
common

⦿ There may be encephalitis, usually in infancy.

⦿ No, there is no clinical difference between the paralysis caused
by wild poliovirus, OPV, or VDPV.
Outcomes of poliovirus infection in children Outcome
• No symptoms 72%
• Minor illness 24%
• Nonparalytic aseptic
meningitis 1–5%
• Paralytic poliomyelitis 0.1–0.5%

• — Spinal polio
• 79% of paralytic cases
• — Bulbospinal polio
• 19% of paralytic cases
• — Bulbar polio
• 2% of paralytic cases
Physical Exam:

⦿ Significant motor loss on affected side or limb.

⦿ Meningeal signs may be present in minor illness or early
phases of paralytic polio.
⦿ Tone is reduced: asymmetric
⦿ Decreased deep tendon reflexes.
⦿ Muscle atrophy of affected areas.
⦿ The sensory examination is normal.
Diagnosis
⦿ Cerebrospinal Fluid:
Leukocytosis, Increased protein, Normal glucose.

⦿ Virus recovery:
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.
Diagnosis…cont

⦿ Nerve conduction studies reveal

⚫ Sensory nerve studies: normal

⚫ Motor nerve studies: show normal to mildly

slowed conduction velocities and low to normal
amplitudes.
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.
Poliomyelitis: Complications

⦿ Urinary tract infection

⦿ Skin ulcers
⦿ Traumatic injuries to affected limb(s)
⦿ Atelectasis & Pneumonia
⦿ Myocarditis
⦿ Post-poliomyelitis progressive muscular
atrophy.
⦿ Post-poliomyelitis motor neuron disease.
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.
Clinical Evolution
PREVENTION OF POLIOMYELITIS
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.
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.
⦿ One should get it if travel places where polio is still
epidemic.
Transverse Myelitis
⦿ Inflammation of the spinal cord causing acute/or
sub-acute loss of motor, sensory and autonomic
function, often evolves in hours or days.

⦿ Pathophysiology: presumed autoimmune mediated

inflammation and demyelination of the spinal cord.

⦿ Post-infectious etiology largely predominates in

children
 Associated Conditions

⦿ Is most commonly associated with infection varicella,

mumps, measls etc
⦿ 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.
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.
Physical Examination

⦿ Tenderness over the spine may point to trauma or

infection.
⦿ Increased tone, spastic weakness, legs more than arms
⦿ Reflexes are usually brisk, with positive Babinski sign.
⦿ Visual acuity and color vision, funduscopic examination
for optic neuritis.
⦿ Sensory ataxia, a sensory level.
⦿ Sphincter dysfunction.
 Diagnosis:
⦿ MRI and CSF analysis are the two most important
investigations for the diagnosis of ATM.

⦿ If both tests are negative, repeat in 2 to 7 days.

⦿ 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.
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.
 Treatment

⦿ IV methylprednisolone may be useful in ATM

⦿ 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.
 Treatment…cont

⦿ 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.
 Prognosis
⦿ 50% make a full recovery within 3 to 6 months.

⦿ 40% recover incompletely, 10% don’t 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.