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Interictal High Frequency Oscillations Detected with Simultaneous Magnetoencephalography and Electroencephalography as Biomarker of Pediatric Epilepsy
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Myoclonic encephalopathy of infancy: a 10 year review

M D Nissen1, D B Appleton

  • 1Department of Neurology, Royal Children's Hospital, Herston, Queensland, Australia.

Journal of Paediatrics and Child Health
|December 1, 1994
PubMed

Insights

Myoclonic encephalopathy of infancy (MEI) can cause acute ataxia in infants and is a rare neuroblastoma symptom. Early investigation for neural crest tumors is crucial, though a cause remains unidentified in many cases.

Area of Science:

  • Pediatric Neurology
  • Oncology

Background:

  • Myoclonic encephalopathy of infancy (MEI) is an uncommon cause of acute ataxia in infants.
  • MEI can be a rare presenting sign of neuroblastoma.

Observation:

  • A review of five infant cases at a tertiary referral children's hospital over 10 years.
  • Two of the five cases were associated with neuroblastoma.

Findings:

  • All infants received intramuscular adrenocorticotropic hormone injections, resulting in symptomatic improvement.
  • One patient died from an opportunistic infection post-neuroblastoma chemotherapy.
  • Four survivors exhibited mild to moderate clinical and intellectual deficits.

Implications:

  • Routine investigation and ongoing surveillance for occult neural crest tumors are recommended for all MEI cases.
  • The underlying cause of MEI was not identified in 60% of the studied infants.

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