Neurology Book

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Ataxia in Children

Aka: Ataxia in Children, Pediatric Ataxia, Opsoclonus-myoclonus syndrome, Acute Demyelinating Encephalomyelitis, Acute Disseminated Encephalomyelitis, Acute Cerebellar Ataxia
  1. See Also
    1. Ataxia
  2. Causes
    1. See Ataxia for causes seen in both adults and children
    2. Congenital malformations
    3. Acute Cerebellar Ataxia
      1. Follows febrile illness by 7-10 days, especially in males (see infections below)
      2. Autoimmune mediated Cerebellum demyelination
      3. Maximal findings at onset, most significantly affecting truncal Ataxia
      4. Presents with normal mental status (contrast with Acute Demyelinating Encephalomyelitis)
    4. Acute Demyelinating Encephalomyelitis (ADEM, Acute Disseminated Encephalomyelitis)
      1. Follows infectious illness or Vaccines (or in 26% idiopathic) by days to weeks (see infections below)
      2. More common in males, and in winter and spring
      3. Maximal findings at onset, with a constellation of neurologic symptoms (e.g. Headache, Hemiplegia, Ataxia)
      4. Presents with acute Altered Level of Consciousness or encephalopathy (coma in some cases)
      5. Most severe form is acute hemorrhagic leukoencephalomyelitis
      6. Administer Methylprednisolone 20-30 mg/kg/day (up to 1 gram daily) for 3-5 days, then taper over 4-6 weeks
      7. Full recovery in >60% of cases with aggressive management (previously mortality was >30%)
    5. Opsoclonus-myoclonus syndrome
      1. Paraneoplastic syndrome seen in children under age 3 years old
      2. Neuroblastoma presentation in 50% of cases (post-viral in others)
      3. Associated with rapid chaotic eye movements, myoclonic jerks, encephalopathy, Vomiting, irritability
  3. Causes: Infections and Vaccines
    1. Viral
      1. Varicella (most common)
      2. Influenza
      3. Epstein-Barr Virus (EBV, Mononucleosis)
      4. Cytomegalovirus (CMV)
      5. Coxsachie Virus
      6. Herpes Simplex Virus
      7. Human Immunodeficiency Virus (HIV)
      8. Measles
      9. Mumps
      10. Rubella
      11. Viral Hepatitis
      12. Rocky Mountain Spotted Fever
    2. Bacterial
      1. Streptococcal species
      2. Campylobacter
      3. Legionella
      4. Mycoplasma
      5. Chlamydia
      6. Borrelia
    3. Immunizations
      1. Rabies Vaccine
      2. Diphtheria, Tetanus, Polio Vaccine (Revaxis)
      3. MeaslesVaccine
  4. Evaluation
    1. See Ataxia
  5. References
    1. Streich and Huff (2015) Crit Dec Emerg Med 29(2): 2-9
    2. Salas (2010) Emerg Med J 27(12): 956-7 [PubMed]

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