Acute Flaccid Paralysis in Children: Clinical Case and QnA

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1. Clinical Case of Acute Flaccid Paralysis in Children

Clinical Case: Acute Flaccid Paralysis

A 4-year-old boy, previously healthy, is brought to the emergency department by his parents with complaints of sudden onset weakness in both legs. The symptoms started 2 days ago and have rapidly progressed.

History:

  • No recent history of trauma or injury
  • Mild fever (38.1°C) and general malaise 5 days prior to onset of weakness
  • No recent vaccinations
  • No recent travel
  • No known tick bites

Physical Examination:

  • Alert and oriented
  • Vital signs: Temperature 37.8°C, Heart rate 110/min, Respiratory rate 24/min, BP 100/60 mmHg
  • Neurological examination:
    • Flaccid paralysis of both lower limbs (power 1/5 bilaterally)
    • Absent deep tendon reflexes in lower limbs
    • Decreased muscle tone in affected limbs
    • Sensory function intact
    • No involvement of upper limbs or cranial nerves
    • No bladder or bowel involvement
  • Other systems: Unremarkable

Investigations:

  • Complete blood count: Mild leukocytosis (12,000/μL)
  • CSF analysis:
    • Protein: 65 mg/dL (slightly elevated)
    • Glucose: 60 mg/dL (normal)
    • WBC: 15 cells/μL (slightly elevated)
  • MRI spine: Enhancement of cauda equina nerve roots
  • Stool culture: Pending

Initial Management:

  • Admission to pediatric intensive care unit for close monitoring
  • Supportive care
  • Initiation of intravenous immunoglobulin (IVIG)
  • Physiotherapy
  • Further investigations to determine etiology (viral studies, neuromuscular studies)

Differential Diagnosis:

  • Guillain-Barré syndrome
  • Acute poliomyelitis
  • Transverse myelitis
  • Acute flaccid myelitis

This case presents a typical scenario of acute flaccid paralysis in a pediatric patient, highlighting the importance of prompt recognition, thorough examination, and appropriate management.

2. Clinical Presentations of Acute Flaccid Paralysis in Children

Clinical Presentations of Acute Flaccid Paralysis in Children

  1. Classic Ascending Paralysis (Guillain-Barré Syndrome)

    • Rapid onset of symmetrical weakness starting in the legs and ascending to arms
    • Absent or diminished deep tendon reflexes
    • Possible facial weakness or ophthalmoplegia
    • Potential respiratory involvement in severe cases
    • Preceded by viral illness or gastrointestinal infection in many cases

  2. Focal Limb Weakness (Acute Flaccid Myelitis)

    • Sudden onset of limb weakness, often asymmetrical
    • Predominantly affects one or more limbs
    • Associated with gray matter lesions on MRI
    • May be accompanied by fever and respiratory symptoms
    • Potential for rapid progression and respiratory failure

  3. Bulbar Paralysis (Poliomyelitis or Brainstem Encephalitis)

    • Weakness of muscles innervated by cranial nerves
    • Difficulties with swallowing, speaking, or facial expressions
    • Possible involvement of respiratory muscles
    • May be accompanied by fever and meningeal signs

  4. Acute Transverse Myelitis

    • Rapid onset of bilateral leg weakness
    • Sensory level deficit
    • Bladder and bowel dysfunction
    • Clear spinal cord lesion on MRI

  5. Todd's Paralysis (Post-ictal)

    • Temporary paralysis following a seizure
    • Typically unilateral and resolves within 24-48 hours
    • History of seizure activity

  6. Tick Paralysis

    • Ascending flaccid paralysis
    • Begins in lower extremities and ascends
    • Associated with attached tick, often found in scalp or axilla
    • Rapid improvement after tick removal

  7. Hypokalemic Periodic Paralysis

    • Episodic weakness, often triggered by rest after exercise or high-carbohydrate meals
    • May affect all four limbs
    • Associated with low serum potassium levels
    • Family history may be present

  8. Botulism

    • Descending flaccid paralysis
    • Begins with cranial nerve involvement (diplopia, ptosis, dysarthria)
    • Progresses to limb and respiratory muscle weakness
    • Associated with constipation and poor feeding in infants

  9. Acute Myositis

    • Sudden onset of muscle pain and weakness, often in calves
    • May follow viral illness
    • Elevated creatine kinase levels
    • Usually self-limiting

  10. Spinal Cord Compression

    • Acute onset of weakness, often with a sensory level
    • May be associated with back pain
    • History of trauma or presence of spinal abnormality
    • MRI shows cord compression

These presentations highlight the diverse etiologies and manifestations of acute flaccid paralysis in children, emphasizing the need for a thorough clinical assessment and targeted investigations to determine the underlying cause and guide appropriate management.

3. Viva Questions and Answers on Acute Flaccid Paralysis in Children

  1. Q: What is the definition of Acute Flaccid Paralysis (AFP)?

    A: Acute Flaccid Paralysis is defined as a sudden onset of weakness and reduced muscle tone in one or more limbs, with decreased or absent reflexes, in a child less than 15 years of age. It can also include weakness of respiratory or bulbar muscles.

  2. Q: What are the main causes of AFP in children?

    A: The main causes include:

    • Guillain-Barré Syndrome (GBS)
    • Acute Flaccid Myelitis (AFM)
    • Poliomyelitis
    • Transverse Myelitis
    • Traumatic Neuritis
    • Botulism
    • Hypokalemic Periodic Paralysis
    • Spinal Cord Compression

  3. Q: Why is AFP surveillance important in global health?

    A: AFP surveillance is crucial for the global polio eradication initiative. It helps in early detection of potential polio cases, monitoring the effectiveness of polio vaccination programs, and identifying other neurological conditions that may mimic polio.

  4. Q: What is the typical presentation of Guillain-Barré Syndrome in children?

    A: GBS typically presents with:

    • Symmetrical ascending weakness starting in the legs
    • Decreased or absent deep tendon reflexes
    • Potential respiratory involvement
    • Often preceded by a viral or gastrointestinal infection

  5. Q: How does Acute Flaccid Myelitis (AFM) differ from GBS?

    A: AFM differs from GBS in several ways:

    • Often asymmetrical limb weakness
    • Primarily affects the spinal cord gray matter
    • Associated with specific viruses (e.g., enteroviruses)
    • MRI shows characteristic spinal cord lesions

  6. Q: What immediate steps should be taken when a child presents with AFP?

    A: Immediate steps include:

    • Assess and stabilize airway, breathing, and circulation
    • Detailed neurological examination
    • Admit for close monitoring, especially respiratory function
    • Initiate appropriate investigations (blood tests, lumbar puncture, imaging)
    • Report to relevant health authorities for AFP surveillance

  7. Q: What investigations are essential in a child with AFP?

    A: Essential investigations include:

    • Complete blood count and electrolytes
    • Cerebrospinal fluid analysis
    • Nerve conduction studies and electromyography
    • MRI of brain and spinal cord
    • Stool samples for poliovirus culture
    • Viral studies (serology and PCR)

  8. Q: How is Guillain-Barré Syndrome typically managed in children?

    A: Management of GBS typically includes:

    • Close monitoring in an ICU setting
    • Respiratory support if needed
    • Intravenous Immunoglobulin (IVIG) or plasmapheresis
    • Pain management
    • Early physiotherapy and rehabilitation
    • Supportive care (nutrition, prevention of complications)

  9. Q: What are the red flags indicating a poor prognosis in AFP?

    A: Red flags include:

    • Rapid progression of weakness
    • Early bulbar involvement
    • Autonomic dysfunction
    • Respiratory muscle involvement requiring ventilation
    • Severe axonal involvement on electrophysiological studies

  10. Q: How does poliomyelitis present, and why is it still a concern globally?

    A: Poliomyelitis presents with:

    • Acute onset flaccid paralysis, often asymmetrical
    • Fever and meningeal signs may precede paralysis
    • Predominantly affects lower limbs

    It remains a concern due to the risk of outbreaks in under-vaccinated populations and the goal of global eradication.

  11. Q: What is the role of lumbar puncture in AFP evaluation?

    A: Lumbar puncture is crucial for:

    • Differentiating between central and peripheral causes
    • Identifying albuminocytological dissociation in GBS
    • Detecting viral or bacterial infections
    • Assessing for malignant cells in cases of compression

  12. Q: How does tick paralysis present, and how is it managed?

    A: Tick paralysis presents with:

    • Ascending flaccid paralysis
    • Normal sensation
    • Absence of fever

    Management involves careful search for and removal of the tick, followed by supportive care. Symptoms typically resolve rapidly after tick removal.

  13. Q: What is the differential diagnosis for acute-onset lower limb weakness in a child?

    A: Differential diagnosis includes:

    • Guillain-Barré Syndrome
    • Transverse Myelitis
    • Acute Flaccid Myelitis
    • Spinal Cord Compression
    • Conversion Disorder
    • Botulism
    • Periodic Paralysis

  14. Q: How does botulism cause AFP, and what are its distinguishing features?

    A: Botulism causes AFP by blocking acetylcholine release at neuromuscular junctions. Distinguishing features include:

    • Descending paralysis starting with cranial nerves
    • Prominent bulbar symptoms (diplopia, dysphagia, dysarthria)
    • Autonomic dysfunction
    • History of exposure (contaminated food in older children, honey ingestion in infants)

  15. Q: What is the significance of stool culture in AFP cases?

    A: Stool culture is significant because:

    • It's essential for ruling out poliovirus infection
    • It's part of the global polio surveillance program
    • It can identify other enteroviruses associated with AFP
    • Two stool samples 24-48 hours apart should be collected within 14 days of paralysis onset

  16. Q: How does hypokalemic periodic paralysis present, and how is it managed?

    A: Hypokalemic periodic paralysis presents with:

    • Episodes of muscle weakness or paralysis
    • Typically triggered by rest after exercise or high-carbohydrate meals
    • Low serum potassium levels during attacks

    Management includes:

    • Acute potassium replacement
    • Avoidance of triggers
    • Consideration of prophylactic medications (e.g., acetazolamide)

  17. Q: What is the role of plasmapheresis in the treatment of Guillain-Barré Syndrome?

    A: Plasmapheresis in GBS:

    • Removes circulating antibodies and inflammatory mediators
    • Can shorten the course of illness and improve outcomes
    • Is typically used as an alternative to IVIG
    • Is usually performed 4-6 times over 1-2 weeks
    • May be preferred in certain subtypes of GBS

  18. Q: How does acute flaccid myelitis (AFM) differ from transverse myelitis?

    A: Key differences include:

    • AFM primarily affects gray matter, while transverse myelitis affects both gray and white matter
    • AFM often presents with asymmetric limb weakness, while transverse myelitis typically causes symmetric weakness
    • AFM is associated with specific viruses (e.g., enteroviruses), while transverse myelitis can have various causes including post-infectious, autoimmune, or idiopathic
    • MRI findings differ: AFM shows anterior horn cell involvement, while transverse myelitis shows diffuse cord involvement

  19. Q: What are the key components of rehabilitation in AFP cases?

    A: Key components of rehabilitation include:

    • Early mobilization and positioning to prevent contractures
    • Physical therapy to maintain joint range of motion and muscle strength
    • Occupational therapy for activities of daily living
    • Respiratory therapy if there's respiratory muscle involvement
    • Psychological support for the child and family
    • Orthotic devices or assistive technology as needed
    • Regular reassessment and goal adjustment

  20. Q: How does the management of AFM differ from that of GBS?

    A: Management differences include:

    • AFM treatment is primarily supportive, while GBS has specific immunomodulatory treatments (IVIG, plasmapheresis)
    • AFM often requires more prolonged respiratory support
    • AFM may benefit from early aggressive physical therapy and nerve transfer surgeries
    • Prognosis and recovery patterns differ, with AFM often having more residual deficits

  21. Q: What are the potential long-term complications of AFP, and how are they managed?

    A: Potential long-term complications and their management include:

    • Residual weakness: Ongoing physiotherapy, occupational therapy, and assistive devices
    • Contractures: Range of motion exercises, splinting, and sometimes surgical correction
    • Chronic pain: Pain management strategies, including medications and non-pharmacological approaches
    • Respiratory insufficiency: Pulmonary rehabilitation, possible need for long-term ventilatory support
    • Psychological issues: Counseling and support for the child and family
    • Growth and development concerns: Regular monitoring and interventions as needed

  22. Q: How does the clinical presentation of AFP differ between infants and older children?

    A: Differences in presentation include:

    • Infants may present with more subtle signs like poor feeding, irritability, or decreased movement
    • Older children can usually verbalize symptoms and localize weakness
    • Respiratory involvement may be more rapidly progressive in infants
    • Autonomic symptoms may be more pronounced or easier to recognize in older children
    • The etiology spectrum may differ, with certain causes (e.g., botulism) being more common in infants

  23. Q: What is the role of electrodiagnostic studies in AFP evaluation?

    A: Electrodiagnostic studies (nerve conduction studies and electromyography) are important for:

    • Differentiating between axonal and demyelinating processes
    • Assessing the severity and distribution of nerve involvement
    • Providing prognostic information
    • Monitoring disease progression or recovery
    • Helping distinguish between various causes of AFP (e.g., GBS subtypes, AFM)

  24. Q: How does the approach to AFP management differ in resource-limited settings?

    A: In resource-limited settings:

    • Focus on clinical diagnosis due to limited access to advanced diagnostic tools
    • Emphasis on supportive care and early recognition of respiratory failure
    • Adaptation of rehabilitation strategies using locally available resources
    • Greater reliance on community-based rehabilitation
    • Increased importance of AFP surveillance for polio eradication efforts
    • Use of alternative treatments when IVIG or plasmapheresis is unavailable (e.g., small volume plasma exchange)

  25. Q: What are the key features of spinal cord compression causing AFP, and how is it managed?

    A: Key features and management of spinal cord compression causing AFP include:

    • Features:
      • Acute onset of weakness with a sensory level
      • Possible associated back pain
      • Bladder and bowel dysfunction
      • History of trauma or spinal abnormality
    • Management:
      • Immediate spinal immobilization
      • Urgent MRI for diagnosis
      • Neurosurgical consultation for potential decompression
      • High-dose corticosteroids in some cases
      • Close monitoring for neurological deterioration
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