Rosen & Barkin's 5-Minute Emergency Medicine Consult (564 page)

More likely to have a biphasic acute course:

• Viral-type syndrome for 1–2 days
  
• Symptom-free period of 2–5 days
  
• Then an abrupt onset of the major illness
  

• Clinical diagnosis
  
• Differentiate from other causes of acute paralysis.
  
• Notify public health officials when diagnosis suspected.
  

• CBC:
  
  • WBC normal or mildly elevated
    
• Diagnosis confirmed by:
  
  • Comparing acute with convalescent sera for antigen titers
    
  • Isolation of virus from blood, CSF, stool, throat secretions (within week 1 of infection)
    

• Lumbar puncture/CSF analysis:
  
  • Abnormalities typical of aseptic meningitis (increased lymphocytes and elevated protein)
    
  • Poliovirus rarely isolated from the CSF
    
• Electrodiagnostics:
  
  • Normal to slow motor function
    
  • Sensory function intact
    

• Abortive poliomyelitis is similar to many viral illnesses.
  
• Nonparalytic poliomyelitis is indistinguishable from any viral, aseptic meningitis.
  
• Paralytic poliomyelitis:
  
  • Amyotrophic lateral sclerosis
    
  • Guillain–Barré (not febrile, symmetric, not ill appearing)
    
  • Acute transverse myelitis
    
  • Spinal cord compression/infarction
    
  • Multiple sclerosis
    
  • Rhabdomyolysis
    
  • Acute intermittent porphyria
    
  • West Nile virus
    
  • Diphtheria
    
  • Botulism
    
  • Tick paralysis
    
  • Encephalitis
    

TREATMENT

Aggressive pulmonary toilet and early intubation mandated for respiratory insufficiency

• Supportive and symptomatic management
  
• Analgesics for severe muscle pain and spasm
  
• Bed rest to prevent augmentation or extension of paralysis
  
• Paralytic poliomyelitis tends to localize to a limb that has been the site of intramuscular injection or injury within 2–4 wk prior to the onset of infection:
  
  • Avoid any unnecessary tissue damage in suspected cases
    
  • No antiviral agents available
    
  • Prevention
    
• Prevention
  
• IPV:
  
  • Costly
    
  • Painful
    
  • No conferred immunity
    
  • No VAP, which previously accounted for all poliomyelitis cases in US
    
• OPV:
  
  • Accounted for only poliomyelitis seen in US (8–10 cases/yr)
    
  • Incidence of VAP: 1/900,000 (immunocompromised: 1/1,000):
    
    • Most at risk are the underimmunized young and their caretakers.
      
  • Confers immunity to unvaccinated contacts by fecal–oral spread.
    
  • Inexpensive
    
  • No longer available in US
    
  • Still remains the vaccine recommended by WHO Expanded Program on Immunization
    

FOLLOW-UP

All acute-phase paralytic poliomyelitis for strict bed rest and observation for respiratory symptoms:

• Isolate from nonvaccinated personnel.
  

No evidence of nervous system involvement and no danger of contact with nonvaccinated population:

• Deterioration of muscle strength usually ends after 3–5 days
  

Physical therapy:

• Only 1/3 of the people with acute flaccid paralysis regain full strength
  
• Lamotrigine may decrease pain, improve symptoms and quality of life.
  
• IV immunoglobulin (IVIg) may improve muscle strength, has not been proven to decrease pain or improve quality of life.
  

• Most cases are asymptomatic, with symptoms ranging from viral illness to acute flaccid paralysis.
  
• IPV is the only vaccine available in US; however OPV is still the vaccine of choice for global eradication.
  
• Diagnosis is primarily clinical and is confirmed by virus isolation from blood, CSF, stool, or throat secretions.
  
• Treatment is supportive; all acute-phase paralytic poliomyelitis patients should be admitted for observation with close monitoring of the respiratory system.
  
• If the patient survives the acute stage, paralysis of respiration and deglutition usually recovers completely.
  
• Paralytic poliomyelitis may occur decades after initial infection and manifests with neurologic and non-neurologic symptoms.
  

• Alexander L, Birkhead G, Guerra F, et al. Ensuring preparedness for potential poliomyelitis outbreaks: Recommendations for the US poliovirus vaccine stockpile from the National Vaccine Advisory Committee (NVAC) and the Advisory Committee on Immunization Practices (ACIP).
  Arch Pediatr Adolesc Med.
  2004;158:1106–1112.
  
• American Academy of Pediatrics:
  Report of the Committee on Infectious Diseases
  . 29th ed. Elk Grove, Il; 2012.
  
• Bouza C, Muñoz A, Amate JM. Postpolio syndrome: A challenge to the health-care system.
  Health Policy.
  2005;71(1):97–106.
  
• Centers for Disease Control and Prevention (CDC). Imported vaccine-associated paralytic poliomyelitis—United States, 2005.
  MMWR Morb Mortal Wkly Rep.
  2006;55(4):97–99.
  
• Centers for Disease Control and Prevention (CDC). Updated recommendations of the Advisory Committee on Immunization Practices (ACIP) regarding routine poliovirus vaccination.
  MMWR Morb Mortal Wkly Rep
  . 2009;58(30):829–830.
  
• Centers for Disease Control and Prevention (CDC). Tracking progress toward global polio eradication—worldwide, 2009–2010.
  MMWR Morb Mortal Wkly Rep.
  2011;60(14):441–445.
  
• Howard RS. Poliomyelitis and the postpolio syndrome.
  BMJ.
  2005;330(7503):1314–1318.
  
• RopperAH, SamuelsMA. Chapter 33. Viral infections of the nervoussystem, chronic meningitis, and prion diseases. In:Ropper AH,Samuels MA, eds.
  Adams and Victor’s Principles of Neurology
  . 9thed. New York, NY:McGraw-Hill; 2009.
  
• Shahzad A, Köhler G. Inactivated polio vaccine (IPV): A strong candidate vaccine for achieving global polio eradication program.
  Vaccine.
  2009;27(39):5293–5294.
  

See Also (Topic, Algorithm, Electronic Media Element)

• Amyotrophic lateral sclerosis
  
• Botulism
  
• Encephalitis
  
• Guillain–Barré Syndrome
  
• Multiple Sclerosis
  
• Rhabdomyolysis
  
• Spinal Cord Syndromes
  
• Tick Bite
  
• West Nile Virus
  

CODES

• 045.00 Acute paralytic poliomyelitis specified as bulbar, poliovirus, unspecified type
  
• 045.20 Acute nonparalytic poliomyelitis, poliovirus, unspecified type
  
• 045.90 Acute poliomyelitis, unspecified, poliovirus, unspecified type
  

BASICS

• Increase in hemoglobin (Hgb) above the normal range:
  
  • Men: Hgb >17.5 g/dL, hematocrit (Hct) >52%
    
  • Women: Hgb >16 g/dL, Hct >48%
    
• Symptoms are related to blood viscosity, which increases exponentially at Hct >60%.
  

• Relative (apparent) polycythemia:
  
  • Resulting from decrease in plasma volume
    
  • Acute: Dehydration
    
  • Chronic: Gaisbock syndrome (stress polycythemia): Obese, hypertensive, middle-aged smokers
    
• Primary erythrocytosis:
  
  • Polycythemia vera (PV): A stem cell disorder characterized by panhyperplasia of all bone marrow elements leading to increased production of RBCs, WBCs, and platelets. Erythrocytosis is the most prominent feature:
    
    • Mutation in the tyrosine kinase (JAK2), which acts in signaling pathways of the EPO-receptor, rendering those cells hypersensitive to erythropoietin
      
    • Median age 60, 5% <40, peak in 70s
      
    • Higher in Ashkenazi Jews and lower in Asians and African Americans.
      
    • May progress to myelofibrosis or acute leukemia
      
• Secondary polycythemia:
  
  • Central hypoxia increasing erythropoietin:
    
    • Chronic pulmonary disease
      
    • Sleep apnea (5–10% have high Hgb)
      
    • Obesity hypoventilation syndrome (Pickwickian syndrome)
      
    • Congenital heart disease (right-to-left shunt)
      
    • High altitude (chronic)
      
    • Smoker’s erythrocytosis
      
    • Carbon monoxide poisoning (chronic)
      
    • Chronic methemoglobinemia
      
  • Renal-mediated causes of increased erythropoietin production:
    
    • Renal cell carcinoma
      
    • Renal artery atherosclerotic narrowing
      
    • Focal glomerulonephritis
      
    • Postrenal transplant with or without rejection
      
    • Chronic hydronephrosis
      
    • Polycystic kidney disease and renal cysts
      
  • Inappropriate autonomous erythropoietin production:
    
    • Hepatomas
      
    • Cerebellar hemangioblastoma
      
    • Wilms tumor
      
    • Parathyroid carcinoma and adenoma
      
    • Ovarian tumors
      
    • Adrenal adenomas and carcinomas (pheochromocytoma, Cushing)
      
    • Uterine leiomyomata
      
  • Blood doping:
    
    • Recombinant erythropoietin abuse
      
    • Autologous transfusions
      
  • Drug abuse:
    
    • Chronic cocaine abuse
      
    • Androgenic steroids
      
• Genetic disorders with polycythemia:
  
  • High-affinity Hgb variants
    
  • Bisphosphoglycerate deficiency
    
  • von Hippel–Lindau syndrome
    
  • Chuvash polycythemia
    
  • Erythropoietin-receptor mutations
    
  • Congenital methemoglobinemia
    
• Infections:
  
  • Viral hepatitis, AIDS