A Pediatric Neurology Case. . . you will get one of these at some point!

by Aditi Jayanth

A 4 month-old boy presents to ED with new “strange body movements.” His grandmother first noticed this behavior 2 weeks ago. His arms and legs would be held in a flexed position, lasting 1 to 2 seconds at a time. He remained alert and responsive during the episodes. His mom notices increasing frequency of the movements this week.

PMH- Cardiorespiratory arrest at 1 month due to RSV Bronchiolitis s/p ECMO.

Physical Exam– Vitals signs normal for age. Smiling and playful. On motor examination, spontaneous movements of all extremities are seen but are slightly decreased on the left compared to the right. Bilateral fisting is noted with increased tone in the left upper and lower extremities. Axial tone is mildly decreased for age.

What is the most likely diagnosis?

  • Infantile Spasm
  • DDx: gastroesophageal reflux, colic, spasticity, and benign myoclonus of infancy

Infantile Spasms are subtle spells that may initially not be recognized by primary care physicians resulting in delay of prompt treatment. Parents will often bring their infant for further medical attention when the jerks become more forceful or the child does not show developmental skills. Parents sometimes report a loss of babbling, verbalizations, or even head and trunk control.

Check out this video showing Infantile Spasms.

What you need to know in the ED

  • Infantile spasms should be suspected in any infant or child with repeated flexor or extensor contractions of the body
  • 90% begin to have spasms within the first year of life
  • It is frequently misdiagnosed (see DDx above)
  • EEG is indicated and hypsarrhythmia is the classic EEG finding
  • It is theorized that the earlier the spasms are controlled, the better the cognitive outcome. Overall prognosis is still fairly poor with development of seizure disorder and developmental delays.

 

References

Osman, F. et al. Visual Diagnosis:6-Month-Old Boy With Strange movements. Pediatrics in Review 2014;35;4

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